Question 1

A 12-year-old boy is brought to the emergency department by his parents due to left arm pain.  He was competing in a basketball tournament and fell on his left arm while his left hand was outstretched and immediately experienced severe pain.  The patient has seasonal allergies for which he takes loratadine daily.  Immunizations are up to date.  Physical examination reveals a tearful and anxious boy in moderate distress.  There is significant swelling over the left elbow, and the patient has limited range of motion at the elbow secondary to pain.  He can fully extend and flex all 5 digits.  Radial pulse is palpable, and there is brisk capillary refill.  There is no tenderness to palpation over the wrist or forearm.  Radiograph of the elbow is shown below and reveals a displaced posterior fat pad in the olecranon fossa.  In addition to administration of pain medication, which of the following is the most appropriate next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_c0cd_4043_8f63_91ee60e13e32_MD0005_00 This patient's presentation is suggestive of an occult supracondylar fracture, a common childhood fracture involving the distal humerus. The typical history is immediate, severe pain after falling on an outstretched hand (FOOSH). Examination findings include elbow pain, swelling, and limited range of motion. Due to proximity of blood vessels (ie, brachial artery) and nerves (eg, median nerve), distal perfusion and neurologic function may be compromised as shown by poor capillary refill or weakness in hand flexion. distal perfusion Radiographic findings may show definitive signs of fracture (eg, fracture line, displacement) or a posterior fat pad, which suggests an occult fracture. As seen in this patient, a fat pad (red arrow) is an abnormal radiolucency posterior to the humerus that represents displaced fat due to a traumatic elbow effusion. Treatment of an occult or nondisplaced supracondylar fracture includes splint placement for immobilization. Displaced fractures require reduction (closed or open) with percutaneous pinning (Choice C). fat pad Displaced fractures (Choice A) Manual reduction by flexion and supination is the treatment for nursemaid's elbow, or radial head subluxation. This injury occurs in young children after axial traction of an extended arm; x-ray is typically normal. (Choice B) Compartment syndrome, a rare complication of a supracondylar fracture, presents with excessive swelling, increasing pain, and poor distal perfusion, none of which is present in this patient. Treatment is emergent fasciotomy to prevent limb ischemia. (Choice E) Conservative measures (eg, rest, application of ice, elevation) are appropriate for sprains or strains of an extremity. An occult fracture should be suspected in this patient with severe pain, a classic FOOSH mechanism, and a posterior fat pad visible on x-ray. Educational objective: Supracondylar fractures usually occur after a fall on an outstretched hand and typically present with elbow pain and swelling as well as limited range of motion. An occult, nondisplaced fracture may be presumed if there is fat-pad displacement on radiograph and should be treated with immobilization. References: Fracture supracondylar humerus: a review. (http://www.ncbi.nlm.nih.gov/pubmed/28208961) Supracondylar humeral fractures in children: current concepts for management and prognosis. (http://www.ncbi.nlm.nih.gov/pubmed/26311512)

Question 2

A 4-year-old boy is brought to the emergency department by his mother due to refusing to walk since this morning. The patient started having right groin pain 2 days ago after T-ball practice, and his mother noticed him "walking funny" yesterday. She says, "I had to carry him here because he says it hurts too much to walk." The mother has witnessed no recent falls or injuries, and the symptoms have not improved with acetaminophen. The patient has had upper respiratory symptoms for the last 2 weeks and was diagnosed with viral pharyngitis a week ago. He has a history of atopic dermatitis that is controlled with low-dose topical corticosteroids. Immunizations are up to date. He takes no medications and has no known allergies. Temperature is 38.6 C (101.5 F). The patient appears tired but nontoxic. The posterior pharynx is clear and without exudates. The lungs are clear to auscultation. On examination of the right hip, the patient cries and resists passive movement. There is decreased range of motion of the right hip. Dry, erythematous patches are noted on the bilateral forearms. Laboratory findings show a white blood cell count of 14,000/mm³ and a C-reactive protein of 4.0 mg/L (normal: <3.0 mg/L). Which of the following is the best next step in management of this patient's condition?

Accepted Answer

Explanation: 11ec2b69_c0c6_6367_8f63_236b567e4566_MD0005_00 Septic arthritis is a joint infection that frequently involves the hip or knee in young children. Most cases occur in healthy children, but patients with immunodeficiencies, prior joint surgery, hemoglobinopathies, juvenile idiopathic arthritis, and diabetes mellitus are at increased risk. Patients with septic arthritis of the hip usually have swelling and decreased range of motion around the joint. Children often keep the hip in a flexed, abducted, and externally rotated position. In children with unilateral hip pain, septic arthritis can be differentiated from other etiologies, such as transient synovitis (post-viral inflammatory reaction of the joint), based on the following criteria (ie, Kocher criteria): Non-weight-bearing Fever ≥38.5 C (101.3 F) Erythrocyte sedimentation rate >40 mm/hr OR C-reactive protein >2.0 mg/dL Leukocytosis Pediatric patients with ≥3 criteria have a high likelihood of septic arthritis (>93%), and immediate joint aspiration is performed to both establish the diagnosis and decompress the joint. Once the diagnosis is confirmed, empiric parenteral antibiotics as well as surgical drainage and debridement of the joint are required. A delay in diagnosis and treatment can result in joint damage, avascular necrosis, femoral head enlargement, and limb length discrepancy. (Choice A) Acute rheumatic fever (ie, positive antistreptococcal antibody titers) typically presents a few weeks after pharyngitis. Although patients often have fever and elevated inflammatory markers, they typically have a migratory polyarthritis that rarely involves the hip. (Choice C) MRI may be used to evaluate the extent of infection prior to surgical debridement. However, ordering an MRI prior to joint aspiration delays treatment as antibiotics are not administered until synovial fluid is obtained. (Choice D) Serum antinuclear antibodies may be positive in juvenile idiopathic arthritis, which presents with an inflammatory arthritis characterized by a chronic, waxing-and-waning course involving multiple joints (rarely the hip). (Choice E) Borrelia burgdorferi serology is positive in Lyme arthritis, which typically presents with erythema migrans after a previous tick bite, and the associated arthritis is more common in the knee than the hip. Patients are generally afebrile and able to bear weight despite the swollen, painful joint. erythema migrans Educational objective: Septic arthritis is a joint infection characterized by pain, fever, swelling, decreased range of motion, and refusal to bear weight. Laboratory evidence of infection (eg, leukocytosis, elevated inflammatory markers) is typically present, and joint aspiration confirms the diagnosis and decompresses the joint. References: Bone and joint infections in children: septic arthritis. (http://www.ncbi.nlm.nih.gov/pubmed/26189923)

Question 3

A 12-year-old boy is brought to the office by his father for an urgent care visit due to right hip pain that has worsened over the past 3 weeks.  The patient initially had "a little bit" of hip pain, but now the pain has spread to his thigh and knee.  He says, "It is so bad that I can barely walk."  The boy has had no recent injuries, fever, or sore throat.  Aside from the pain in the right lower extremity, he feels well.  He has mild eczema that is managed with emollients.  He is allergic to dust and cats.  The patient's only hospitalization was for an appendectomy at age 7.  Temperature is 37 C (98.6 F), blood pressure is 114/74 mm Hg, and pulse is 90/min.  He is at the 95th percentile for weight and the 50th percentile for height.  Physical examination reveals an obese boy in moderate pain, with the right hip in an externally rotated position.  There is no warmth, swelling, or tenderness of the right knee or thigh.  The patient has normal Tanner 3 male external genitalia; the testes are descended bilaterally.  Sensation is intact in the lower extremities, and peripheral pulses are 2+ bilaterally.  Dry, red patches are present on the right knee and left elbow.  The remainder of the physical examination is normal.  X-ray of the hips is shown below.  Which of the following is the most appropriate next step in management of this patient's condition?

Accepted Answer

Explanation: 11ec2b69_c0ca_d03d_8f63_93113222a542_MD0005_00 The child has a slipped capital femoral epiphysis (SCFE) that is progressing from stable (able to bear weight) to unstable (unable to bear weight). SCFE typically presents in an obese adolescent (age 10-16). Widening of the epiphyseal growth plate due to abnormal cartilage maturation results in progressive dull hip (or referred knee/thigh) pain, altered gait, and impaired internal rotation of the hip. (SCFE) SCFE is diagnosed by x-ray (anteroposterior and frog-leg lateral views) of the bilateral hips. Radiographs reveal posterior and inferior displacement of the femoral head along the plane of the physis, giving the appearance of "ice cream slipping off a cone." Up to 20% of those affected have bilateral involvement at presentation; an additional 20%-40% have bilateral involvement within 18 months of the initial SCFE. Radiographs The treatment is immediate surgical pinning. Delay in treatment (>24 hours in unstable SCFE) can lead to avascular necrosis and femoroacetabular impingement, which increase the risk of future degenerative arthritis. (Choice A) Septic arthritis, diagnosed with arthrocentesis, presents with fever and acute-onset hip pain. Imaging shows an effusion. (Choice B) Bone scintigraphy (bone scan) is generally avoided in children due to high radiation exposure. Although it may identify long-term complications, such as poor femoral head perfusion and osteonecrosis, it should not delay treatment once a diagnosis is made on radiograph. (Choice C) External reduction and fixation with a cast is the treatment for a fracture, which generally presents as acute pain following trauma. Reduction correlates with worse outcomes in patients with SCFE. (Choice E) Weight loss helps to prevent SCFE but is not the treatment once it has occurred. Analgesics may provide symptomatic relief but do not address the underlying structural instability. Once a diagnosis of SCFE is made, the patient should be made non-weight bearing and referred immediately for surgery to prevent further slippage. Educational objective: Slipped capital femoral epiphysis is a common cause of progressive hip pain and altered gait in obese adolescents. It is diagnosed on x-ray with a posteriorly displaced femoral head, and immediate surgical pinning is the treatment of choice. References: Slipped capital femoral epiphysis: how to evaluate with a review and update of treatment. (http://www.ncbi.nlm.nih.gov/pubmed/25439015) Long-term outcomes of slipped capital femoral epiphysis treated with in situ pinning. (http://www.ncbi.nlm.nih.gov/pubmed/27438268)

Question 4

A 48-year-old Caucasian woman comes to the physician because of a 1 week history of a lump in her neck. She has muscle pains and body aches. Upon further questioning, she adds that she often experiences a "gritty, burning sensation in both of my eyes, and sometimes they get red and inflamed." She does not wear contact lenses. She has not had fever, chills, weight loss, dysphagia, cough, hemoptysis, muscle weakness, or joint swelling. She has fibromyalgia, depression, and cholelithiasis. She has extensive caries of her teeth and has been visiting a dentist regularly. She does not use tobacco, alcohol, or drugs. Her only medication is fluoxetine. Her temperature is 37.0 C (98.6 F), blood pressure is 122/80 mm Hg, pulse is 76/min, and respirations are 14/min. Her BMI is 27 kg/m2. Examination shows a mobile, non-tender 2x2 cm right-sided submandibular mass. Item 1 of 2 Which of the following is the most appropriate next step in management?

Accepted Answer

Explanation: The most likely diagnosis in this patient is Sjogren's syndrome, a chronic, autoimmune inflammatory condition that primarily affects the lacrimal and salivary glands. The "gritty, burning sensation" and conjunctival irritation described by this patient are typical manifestations of keratoconjunctivitis sicca secondary to abnormal tear production by the lacrimal glands. Keratoconjunctivitis sicca may also present with prominent bulbar blood vessels and stringy discharge from the eyes. Dental caries may be a manifestation of xerostomia (decreased saliva production). Anti-Ro/SSA and anti-La/SSB antibodies are associated with Sjogren's syndrome - 50% of patients with Sjogren's syndrome test positive for one of the above antibodies. Because there is no definitive diagnostic test, these antibodies can be helpful to confirm the clinical diagnosis of Sjogren's syndrome. (Choice B) Tuberculosis can produce necrotic lymph nodes (sometimes referred to as scrofula). However, this patient's overall presentation is more consistent with Sjogren's syndrome. (Choice C) Sjogren's syndrome results in very few symptoms referable to the paranasal sinuses. MRI could be considered for evaluation of the submandibular mass - not for evaluation of the sinuses. (Choice D) Nasopharyngeal endoscopy should be considered if there was concern for nasopharyngeal carcinoma. While a metastatic lymph node is in the differential for the submandibular mass, this is unlikely to be secondary to nasopharyngeal carcinoma in this particular patient. (Choice E) Thyroid abnormalities would not explain this patient's symptoms. Educational objective: The combination of ocular symptoms consistent with keratoconjunctivitis sicca and oral symptoms consistent with xerostomia should lead one to suspect a diagnosis of Sjogren's syndrome. Anti-Ro-SSA and anti-La/SSB antibodies are commonly present and can be helpful to confirm one's clinical suspicion of Sjogren's syndrome, as no true diagnostic test is available. References: Anti-Ro (SS-A) and anti-La (SS-B) in patients with Sjögren's syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/3485431)

Question 5

An 8-year-old boy is brought to the urgent care clinic by his grandmother due to a fever, sore throat, headache, and nausea that began yesterday.  His symptoms have worsened and he is now having difficulty swallowing.  He is up to date on vaccines and has no chronic medical conditions.  The grandmother cares for the child after school while his mother is at work.  The boy's father died of leukemia 3 years ago, and his mother is healthy.  Temperature is 39.4 C (102.9 F), blood pressure is 100/70 mm Hg, pulse is 112/min, and respirations are 14/min.  Pulse oximetry is 95% on room air.  The boy is awake, alert, and in mild discomfort.  Examination of the oropharynx reveals pharyngeal erythema and exudate.  The uvula is deviated to the right and the left soft palate is depressed downward.  The patient has pooled oral secretions but is able to swallow.  There is bilateral anterior cervical lymphadenopathy.  Lung fields are clear to auscultation bilaterally with good air entry.  A small peritonsillar abscess is suspected and the proposed plan, including transfer to the hospital for needle aspiration, is discussed with the grandmother.  When asked if she has written consent to permit treatment, the grandmother replies that she has never been asked such a question before.  Which of the following is the best next step in management of this patient?

Accepted Answer

Explanation: Informed consent from a parent or legal guardian should be obtained prior to nonemergency medical interventions in a minor. Children often present with a caretaker (eg, relative, nanny) who does not have the legal right to consent for treatment (Choice B). For routine care, a written consent by proxy should be signed by the parent or legal guardian specifying who may consent to the child's medical care. Verbal consent is often accepted in cases of urgent intervention, as in this case of a child with a peritonsillar abscess. Because this patient is hemodynamically stable and not in imminent danger, proceeding without consent is inappropriate; the mother should be contacted and legal consent should be obtained (Choice E). (Choice A) Although the child may assent to treatment, he cannot legally consent. Emancipated minors, including those who are married, financially independent, or on active duty, can consent to treatment. (Choice D) If a parent refuses medically necessary treatment, a court order can be obtained to overrule the parental decision. If the boy's mother were to refuse consent for abscess drainage, petitioning the court would be appropriate and in the best interest of the child. Educational objective: In nonemergency situations, informed consent should be obtained from a parent or legal guardian prior to any medical intervention in a minor. References: Informed consent in decision-making in pediatric practice. (http://www.ncbi.nlm.nih.gov/pubmed/27456514) Consent by proxy for nonurgent pediatric care. (http://www.ncbi.nlm.nih.gov/pubmed/28115542)

Question 6

A previously healthy 28-year-old Caucasian woman comes to the physician because of fatigue, arthralgias, and chest pain over the past 6 months.  She has had a 2.3-kg (5-lb) weight loss during this period.  She denies any recent travel or sick contacts.  Her past medical history is insignificant.  She takes no medications and has no known drug allergies.  She is sexually active and uses condoms for contraception.  She has never been pregnant.  She does not use tobacco, alcohol, or drugs.  Her mother has rheumatoid arthritis.  Her temperature is 37.2 C (99 F), blood pressure is 122/77 mm Hg, and pulse is 78/min.  Physical examination shows a faint facial rash.  The lungs are clear to auscultation with mildly decreased breath sounds at the left lung base.  The abdomen is soft and non-tender.  There is no organomegaly.  There is no peripheral edema.  Neurologic examination is unremarkable.   Further evaluation reveals a positive antinuclear antibody (ANA) screen in high titers.  Other lab studies show:  Which of the following antibody titers is most likely to correlate with the disease activity in this patient?

Accepted Answer

Explanation: This patient's high-titer ANA and mild cytopenia further support a diagnosis of systemic lupus erythematosus (SLE). Anti-dsDNA antibodies are helpful to confirm a diagnosis of SLE, but are also useful to follow the course of disease. In some patients, titers of anti-dsDNA antibodies correlate with disease activity. Anti-dsDNA antibody levels have also been associated with the development of lupus nephritis-immune complexes containing these antibodies are actually seen within the glomeruli of patients with lupus nephritis. Rising titer levels of anti-dsDNA antibodies can be helpful in confirming that the underlying lupus is the cause of a clinical problem as opposed to infection, medication toxicity, or unrelated disease. (Choice B) Anti-centromere antibodies are usually seen in scleroderma-not SLE. (Choice C) Anti-mitochondrial antibodies are associated with primary biliary cirrhosis (PBC), and levels are usually stable in a given patient over time. (Choice D) Anti-Smith antibodies do not correlate with disease activity in SLE patients. However, they are highly specific for SLE and may remain positive in patients with clinically-improving SLE when anti-dsDNA antibodies have returned to the normal range. (Choice E) A positive anti-Ro/SSA antibody test has been correlated with a butterfly photosensitivity rash and other cutaneous manifestations of SLE as well as interstitial lung disease and congenital heart block. However, it is not considered as reliable as anti-dsDNA antibodies. Educational objective: Anti-dsDNA antibodies can be used to follow disease activity in SLE patients and is associated with the development of lupus nephritis. References: Predictive value of autoantibodies for activity of systemic lupus erythematosus. (http://www.ncbi.nlm.nih.gov/pubmed/15230281)

Question 7

A 4-year-old boy is brought to the emergency department by his mother due to refusing to walk since this morning. The patient started having right groin pain 2 days ago after T-ball practice, and his mother noticed him "walking funny" yesterday. She says, "I had to carry him here because he says it hurts too much to walk." The mother has witnessed no recent falls or injuries, and the symptoms have not improved with acetaminophen. The patient has had upper respiratory symptoms for the last 2 weeks and was diagnosed with viral pharyngitis a week ago. He has a history of atopic dermatitis that is controlled with low-dose topical corticosteroids. Immunizations are up to date. He takes no medications and has no known allergies. Temperature is 38.6 C (101.5 F). The patient appears tired but nontoxic. The posterior pharynx is clear and without exudates. The lungs are clear to auscultation. On examination of the right hip, the patient cries and resists passive movement. There is decreased range of motion of the right hip. Dry, erythematous patches are noted on the bilateral forearms. Laboratory findings show a white blood cell count of 14,000/mm³ and a C-reactive protein of 4.0 mg/L (normal: <3.0 mg/L). The patient undergoes aspiration of the right hip joint. Synovial fluid analysis shows a white blood cell count of 60,000/mm³ with a neutrophil predominance. Gram stain and culture are pending. Immediate initiation of which of the following therapies is most important to improve the long-term outcome of this patient's condition?

Accepted Answer

Explanation: 11ec2b69_c0c7_26b8_8f63_ab398035bc30_MD0005_00 The white blood cell (WBC) count of synovial fluid is typically >20,000/mm³ in an infected joint, and a WBC count of >50,000/mm³ with >90% neutrophil predominance is highly suggestive of bacterial arthritis. Gram stain and culture can identify the causative organism and guide antibiotic choice. Staphylococcus aureus, followed by Streptococcus pneumoniae and S pyogenes, is the most common pathogen causing septic arthritis in all ages. Empiric coverage for Staphylococcus aureus and streptococci with an agent such as vancomycin is required. Group B Streptococcus and gram-negative bacilli are also common pathogens in infants age <3 months, in whom vancomycin plus cefotaxime is indicated. (Choices A and B) Cefotaxime and ceftriaxone are third-generation cephalosporins that treat streptococci but do not adequately cover S aureus. Third-generation cephalosporins are added if there is suspicion for unusual pathogens, such as Kingella kingae (in children age <3 with indolent arthritis and oral ulcers), Haemophilus influenzae type b (in unimmunized children), and Neisseria gonorrhoeae (in sexually active adolescents). (Choice D) Oral naproxen may be prescribed for pain control in patients with septic arthritis but does not improve long-term outcomes. (Choice E) Oral prednisone treats inflammatory arthropathies, such as juvenile idiopathic arthritis, in which WBC counts of synovial fluid are in the range of 2,000-20,000/mm³. Educational objective: A synovial fluid white blood cell count of >50,000/mm³ with neutrophil predominance indicates a septic joint. Empiric antibiotics (eg, vancomycin) covering the most common organisms, Staphylococcus aureus and streptococci, are required to improve long-term outcomes. References: Pediatric septic arthritis. (http://www.ncbi.nlm.nih.gov/pubmed/28336043) Septic arthritis in children. (http://www.ncbi.nlm.nih.gov/pubmed/25901405)

Question 8

A 65-year-old woman comes to the office with a 6-month history of dry mouth.  She says that she feels dry all day long and has to drink water.  She also has had a sandy sensation in her eyes, especially after watching television for a while.  Medical history is significant for long-term hypertension that is controlled with metoprolol.  She does not smoke or consume alcohol.  Her recent fasting blood glucose level was normal.  Physical examination findings are within normal limits.  Which of the following is the best next step in the management of this patient?

Accepted Answer

Explanation: This patient has dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). These sicca symptoms are extremely common, especially in the elderly (prevalence ~30%), where they may be due to medication usage or to age-related atrophy of tissues that secrete saliva and tears; they are also commonly associated with Sjogren syndrome, an infiltrative autoimmune condition that can affect multiple organ systems (eg, musculoskeletal pain, cutaneous manifestations, interstitial lung disease, hematologic abnormalities). In patients with sicca symptoms, the choice of appropriate diagnostic modalities depends on the patient's age, past medical history, associated findings, and overall pretest probability of Sjogren syndrome. The initial evaluation can include ophthalmologic evaluation, including Schirmer test (slit-lamp examination to assess tear break-up time), as well as autoantibody screening with anti-Ro/SSA, anti-La/SSB, antinuclear antibodies, and rheumatoid factor. Evidence of systemic autoimmunity (eg, positive autoantibodies) can support the diagnosis of Sjogren syndrome. (Choice B) Although labial salivary gland biopsy is the gold standard for diagnosing Sjogren syndrome, it is typically not the first test that is performed. It helps confirm the diagnosis, especially in young patients with aggressive disease but with no evidence of systemic autoimmunity. (Choice C) Low-dose corticosteroids are not typically used to treat sicca symptoms alone. Initial measures for xerostomia include salivary flow stimulation (mechanical, topical) and artificial saliva. (Choice D) Imaging modalities may be helpful in assessing the structure and function of the salivary glands. Ultrasonography and MR imaging are commonly used. Parotid gland sialography requires cannulation of the glands with injection of contrast material; it is associated with a risk of rupture and is not routinely performed. (Choice E) It is necessary to use appropriate diagnostic tools in order to diagnose Sjogren syndrome or to identify other conditions that may present as sicca syndrome (eg, sarcoidosis). Educational objective: A reasonable initial approach to sicca symptoms (eg, dry mouth, dry eyes) is confirming a secretory deficiency (eg, Schirmer test) and obtaining an autoantibody screen to evaluate for Sjogren syndrome.

Question 9

A previously healthy 28-year-old Caucasian woman comes to the physician because of fatigue, arthralgias, and chest pain over the past 6 months. She has had a 2.3-kg (5-lb) weight loss during this period. She denies any recent travel or sick contacts. Her past medical history is insignificant. She takes no medications and has no known drug allergies. She is sexually active and uses condoms for contraception. She has never been pregnant. She does not use tobacco, alcohol, or drugs. Her mother has rheumatoid arthritis. Her temperature is 37.2 C (99 F), blood pressure is 122/77 mm Hg, and pulse is 78/min. Physical examination shows a faint facial rash. The lungs are clear to auscultation with mildly decreased breath sounds at the left lung base. The abdomen is soft and non-tender. There is no organomegaly. There is no peripheral edema. Neurologic examination is unremarkable. Item 1 of 3 Which of the following tests is most likely to be positive in this patient?

Accepted Answer

Explanation: The constellation of this patient's symptoms is most consistent with a diagnosis of systemic lupus erythematosus (SLE). Constitutional symptoms such as fatigue, arthralgias/myalgias, and weight loss are frequently present in SLE. Although not seen in this patient, fever is also a common systemic manifestation in SLE. The most telling clue in this patient is her facial rash, which likely represents the hallmark butterfly rash seen in many SLE patients after sun exposure. The decreased breath sounds at the left lung base are most likely a small pleural effusion secondary to SLE-related inflammation. The pleural effusion may cause pleuritis or chest pain (experienced by this patient). Patients may have a family history of SLE or other connective tissue disorders. Testing for antinuclear antibodies (ANA) is indicated in patients who have suggestive symptoms and is positive in nearly all patients with SLE. However, ANA can also be positive in many patients who are healthy or who have connective tissue diseases other than SLE. Antibodies to double-stranded DNA (dsDNA), a subtype of ANA, are helpful because they are more specific for a diagnosis of SLE, and, with a sensitivity of 66-95%, are also the most sensitive of the listed antibodies to detect SLE. (Choice B) Anti-centromere antibodies are most sensitive for the detection of the CREST variant of scleroderma. (Choice C) Anti-mitochondrial antibodies are sensitive to detect primary biliary cirrhosis (PBC). (Choice D) Anti-Smith antibodies are highly specific for SLE, but have a sensitivity of only 25% (versus anti-dsDNA antibodies, which have a sensitivity of approximately 70%). (Choice E) Anti-Ro/SSA antibodies are seen in some patients with SLE but are most sensitive for Sjogren's syndrome. Educational objective: Although virtually all patients with SLE screen positive for antinuclear antibody (ANA), the test is nonspecific and can also be positive in healthy populations and in patients with connective tissue diseases. Both the anti-dsDNA and anti-Smith antibodies are associated with SLE, although anti-dsDNA antibodies are more sensitive at 66-95%. References: The sensitivity and specificity of autoantibodies to the Sm antigen in the diagnosis of systemic lupus erythematosus. (http://www.ncbi.nlm.nih.gov/pubmed/9588270) Information on diagnosis and management of systemic lupus erythematosus derived from the routine measurement of 8 nuclear autoantibodies. (http://www.ncbi.nlm.nih.gov/pubmed/12913932)

Question 10

A 41-year-old man is brought to the emergency department by ambulance after a motor vehicle accident.  He was driving a car with his 6-year-old daughter in the back seat when they were broadsided by a large truck traveling at high speed.  The patient is conscious during transit to the emergency department.  He appears oriented to date and time, and informs paramedics that he is a Jehovah's Witness and does not want blood transfusions for himself or his daughter.  Soon after, he begins moaning and writhing and is clearly in distress.  He complains of feeling nauseated and thirsty.  A facemask is placed to administer 100% oxygen.  Initial assessment indicates his temperature is 37 C (98.6 F), blood pressure is 100/58 mm Hg, pulse is 132/min and thready, and respirations are 34/min.  There are numerous lacerations on his scalp, trunk, abdomen, and extremities.  The patient's skin is cool and clammy.  Chest auscultation shows tachypnea and tachycardia but is otherwise normal.  His abdomen is diffusely tender to palpation and guarding is present.  Rectal examination is normal.  The patient's blood pressure begins to drop rapidly and approaches 60/15 mm Hg.  The trauma surgeon advises that an immediate exploratory laparotomy is warranted to ascertain the source of the internal bleeding.  Hemoglobin is 5.5 g/dL. The patient's 6-year-old daughter was also severely injured in the accident.  She suffered a significant crush injury to her chest and is now unconscious and bleeding profusely.  At initial assessment, her temperature was 37 C (98.6 F), blood pressure was 72/38 mm Hg, pulse was 154/min, and respirations were 42/min.  While her chest wound is examined externally, her blood pressure declines precipitously.  Two large-bore intravenous catheters are inserted and fluids are administered aggressively, but the child becomes increasingly hemodynamically unstable.  Her hemoglobin is 6 g/dL.  She is intubated and chest tubes are placed.  Which of the following is the most appropriate next step?

Accepted Answer

Explanation: In providing medical care, clinicians must seek to balance the autonomy of the family with the welfare of the child. In urgent situations such as this, clinicians must act to preserve the child's life, even if such actions contradict parental wishes. In general, a competent adult can sacrifice his or her life for religious beliefs, but, as a parent, cannot refuse life-sustaining treatment for a child who has not reached the age of consent and has not chosen to adhere to the religion. Therefore, it is best to proceed with immediate blood transfusion. Obtaining approval from either the child's mother (Choice A) or the court (Choice D) requires the luxury of time. This is an urgent situation, and it is more important to act to save the child immediately. When the child requires intervention within the next few days but is currently stable, it would be appropriate to seek approval from the courts to proceed with medically necessary treatments. (Choice B) The hospital ethics committee should be consulted when a clinician is confronted with an ethically difficult but nonemergency situation. (Choice C) Administering fluids instead of a blood transfusion would be an acceptable choice if the patient were a competent adult. However, because this patient is a child and considered unable to fully understand the consequences of making such a choice, it is standard practice to act to preserve the child's life despite parental wishes. Educational objective: Clinicians must act to preserve the life of a child, even if such actions directly contradict parental wishes. A prime example is the standard practice of administering emergency blood transfusions to the children of Jehovah's Witnesses. References: Jehovah's Witnesses in the emergency department: what are their rights? (http://www.ncbi.nlm.nih.gov/pubmed/16299196)

Question 11

A 22-year-old college student comes to the office due to a 1-week history of right lower leg pain.  She has mild dull pain at rest that becomes sharp and "bothersome" with exercise.  She is an avid runner and has been preparing for an upcoming marathon event for the past 2 months.  Her shoe has recently been bothering her, especially on the lateral side of the right foot.  The patient has no other medical problems or history of recent trauma.  Physical examination shows point tenderness and localized swelling over the right lower tibial border. The diagnostic study performed on the right lower extremity reveals a nondisplaced, distal tibial stress fracture.  Which of the following is the most appropriate next step in the management of this patient?

Accepted Answer

Explanation: Most stress fractures of the lower extremities can be managed with conservative treatment. This includes pneumatic splinting and reduced weightbearing with a graduated exercise program. Crutches are often advised in the first 1-2 weeks to minimize weightbearing. The rehabilitation regimen may be scheduled over 8-12 weeks, but exercise intensity should be reduced and the regimen prolonged if the patient has recurrent symptoms during treatment. (Choice A) Calcium and vitamin D supplementation in postmenopausal women reduces the rate of bone loss, and may decrease the risk of osteoporotic fracture. However, there is currently no evidence that calcium and vitamin D supplementation accelerates healing or reduces the risk of stress fractures in younger patients without osteoporosis. (Choice B) Surgical correction with open reduction and internal fixation is not required in the majority of stress fractures. Adequate rest with a gradual return to activity is usually sufficient in mild cases. (Choice D) Advising on the proper shoe size and fit is important in preventing future injuries to the lower extremities; however, this patient should first be advised to reduce weightbearing with a gradual return to full-intensity exercise. Educational objective: Tibial stress fractures can be managed in most cases with pneumatic splinting, reduced weightbearing, and a graduated exercise program. Most patients may resume full-intensity exercise within 12 weeks, but the program may need to be delayed if the patient develops recurrent pain. References: Stress fractures: diagnosis, treatment, and prevention. (http://www.ncbi.nlm.nih.gov/pubmed/21888126)

Question 12

A 22-year-old college student comes to the office due to a 1-week history of right lower leg pain.  She has mild dull pain at rest that becomes sharp and "bothersome" with exercise.  She is an avid runner and has been preparing for an upcoming marathon event for the past 2 months.  Her shoe has recently been bothering her, especially on the lateral side of the right foot.  The patient has no other medical problems or history of recent trauma.  Physical examination shows point tenderness and localized swelling over the right lower tibial border. 
Which of the following is most likely to be seen on plain x-rays of the right lower extremity?

Accepted Answer

Explanation: This patient, with lower leg pain and tibial tenderness in the setting of a vigorous running regimen, has features suggesting a tibial stress fracture. Stress fractures are a common injury in athletes (up to 15% incidence in runners) and nonathletes who suddenly increase their activity. Besides runners, stress fractures are common in ballet dancers, basketball and soccer players, and military recruits. The risk of stress fractures can be increased by activity-related (eg, excessive training or improper footwear), biomechanical (eg, weak calf muscles or high arched feet), and metabolic (eg, demineralized bone from hormonal or nutritional diseases) factors. Stress fractures occur due to a sudden increase in repeated tension or compression without adequate rest, which eventually breaks the bone. Patients usually initially develop medial tibial stress syndrome ("shin splints") without tibial tenderness; further activity can cause progression to a complete or incomplete fracture, resulting in tibial tenderness on palpation. A stress fracture is diagnosed clinically when there is pain at a specific area that increases with jumping or running and is associated with local swelling and point tenderness on examination. Initial plain x-rays of the tibia have low sensitivity and are negative during the first few weeks in over half of the cases. Possible abnormalities on x-ray may take up to 4 weeks to become apparent and include bone sclerosis, cortical thickening, periosteal elevation, and visible fracture line (Choices A, B, D, and E). If the initial x-ray is negative, patients may be managed empirically based on clinical findings. However, if a definitive diagnosis is needed, MRI is preferred over bone scan or ultrasound as it can show the fracture line that extends through the cortex into the medullary line. MRI can also identify ligament, muscle, and cartilage injuries. However, MRI findings may be persistently abnormal for up to 1 year after the stress fracture has healed. Educational objective: Tibial stress fractures are common in athletes and nonathletes who suddenly increase their physical activity. Clinical features include pain, localized tenderness, and swelling. Plain x-ray is <50% sensitive for stress fractures, especially in the first 2-3 weeks after the onset of symptoms. References: Stress fractures in athletes (http://www.ncbi.nlm.nih.gov/pubmed/17414993)

Question 13

A previously healthy 28-year-old Caucasian woman comes to the physician because of fatigue, arthralgias, and chest pain over the past 6 months.  She has had a 2.3-kg (5-lb) weight loss during this period.  She denies any recent travel or sick contacts.  Her past medical history is insignificant.  She takes no medications and has no known drug allergies.  She is sexually active and uses condoms for contraception.  She has never been pregnant.  She does not use tobacco, alcohol, or drugs.  Her mother has rheumatoid arthritis.  Her temperature is 37.2 C (99 F), blood pressure is 122/77 mm Hg, and pulse is 78/min.  Physical examination shows a faint facial rash.  The lungs are clear to auscultation with mildly decreased breath sounds at the left lung base.  The abdomen is soft and non-tender.  There is no organomegaly.  There is no peripheral edema.  Neurologic examination is unremarkable.  
The patient returns one week after the initial visit.  Her laboratory work-up shows the following additional findings:
 An x-ray film of the chest shows a mild, left-sided pleural effusion.  Which of the following is the most appropriate next step in management?

Accepted Answer

Explanation: This patient's positive anti-dsDNA antibody test confirms the diagnosis of systemic lupus erythematosus (SLE). The left-sided pleural effusion is likely a result of pleurisy secondary to SLE. Hydroxychloroquine is an anti-malarial agent which is particularly effective at improving arthralgias, serositis, and cutaneous symptoms. Since these are the patient's primary symptoms, initiating hydroxychloroquine is indicated. Hydroxychloroquine may also help to prevent future damage to the kidneys and central nervous system. Low dose prednisone at a dose of 5-15 mg/day may be helpful in the short term to improve the patient's symptoms until the hydroxychloroquine takes full effect. In patients with significant disease involving the kidneys or central nervous system, higher dose prednisone may be needed. Long term glucocorticoid use is likely unnecessary in this patient at the current time, since there is no evidence of significant organ involvement. (Choice A) A combination of prednisone and cyclophosphamide are typically reserved for patients with more serious manifestations of SLE (e.g. lupus nephritis, central nervous system involvement, and vasculitis). (Choice C) Methotrexate is generally indicated only for patients with significant organ involvement who have had incomplete response to prednisone alone. (Choice D) Rituximab is an anti-B cell antibody that may have therapeutic value in patients with SLE. However, its use in SLE has been limited due to case reports of associated progressive multifocal leukoencephalopathy (PML). (Choice E) TNF-alpha receptor inhibitors are commonly used in the rheumatoid arthritis but do not currently have a role in the management of SLE. Educational objective: Hydroxychloroquine is an anti-malarial agent that is particularly effective at improving arthralgias, serositis, and cutaneous symptoms in SLE. Low dose, short-term prednisone may be used in patients with acute mild manifestations of SLE, whereas higher dose steroids or other immunosuppressants are typically reserved for patients with more severe, solid-organ manifestations. References: Systemic lupus erythematosus: safe and effective management in primary care. (http://www.ncbi.nlm.nih.gov/pubmed/21050957)

Question 14

A 19-year-old, previously healthy woman comes to the emergency department due to right shoulder pain.  The patient fell while skiing an hour ago and landed with most of her weight on her outstretched right arm.  The pain is sharp and associated with tingling in the shoulder and arm.  Temperature is 37.2 C (99 F), blood pressure is 140/90 mm Hg, pulse is 122/min and regular, and respirations are 14/min.  On examination, the right shoulder is deformed; sensation is diminished over the right lateral aspect of the shoulder but intact throughout the rest of the arm and hand.  Peripheral pulses are symmetric between the upper extremities.  X-ray of the right shoulder is shown in the exhibit.  Which of the following is the best next step in management?

Accepted Answer

Explanation: 11ec2b69_c0c7_9be9_8f63_3385b4c61af9_MD0005_00 This patient with shoulder pain after falling on an outstretched arm has shoulder deformity and an x-ray demonstrating a humeral head that is anterior, medial, and inferior to the glenoid fossa. This is consistent with anterior dislocation of the shoulder. In addition to having a painful and slightly abducted and externally rotated shoulder, some patients (as in this case) experience numbness over the shoulder due to axillary nerve compression. Additional findings include protrusion of the acromion, flattening of the deltoid prominence, and anterior axillary fullness due to the humeral head's displacement. axillary nerve compression Additional findings Diagnosis is confirmed with plain radiograph. Treatment is closed reduction (eg, traction-countertraction, scapular manipulation), which is commonly performed using procedural sedation to minimize the patient's discomfort and increase the likelihood of procedural success because the patient is less likely to resist the manipulation. Successful reduction is accomplished by hearing (and/or feeling) a clunk during the procedure and seeing return of the normal shoulder contour. Postreduction x-ray should be performed to confirm successful reduction and rule out complications that can occur during reduction (eg, glenoid fracture). In addition to improving pain, reduction usually improves the axillary neuropraxia. (Choice B) CT angiography of the upper extremity is helpful to diagnose vascular injury; however, vascular injury is unlikely in this patient who has normal peripheral pulses. (Choice C) Although noncontrast CT scan of the shoulder is sometimes used to confirm diagnosis of shoulder dislocation when x-ray is equivocal, this patient's x-ray is diagnostic of anterior shoulder dislocation. Therefore, reduction is the best next step to minimize pain and address the associated axillary neuropathy. (Choice D) Emergent orthopedic surgical repair is indicated when closed reduction is unsuccessful or when the dislocation is associated with major fracture (eg, humeral neck fracture). This patient has no contraindications to closed reduction (eg, associated fracture, neurovascular compromise), which should be attempted first. (Choice E) Sling immobilization and outpatient orthopedic referral is often appropriate for simple fractures without dislocation (eg, isolated humeral head fracture). However, a shoulder dislocation should be reduced without delay to control pain and prevent ligamentous or neurologic damage. Educational objective: Anterior shoulder dislocations result in anterior, medial, and inferior displacement of the humeral head, which can cause axillary nerve compression. Diagnosis is confirmed with plain radiograph, and treatment is prompt closed reduction with procedural sedation. References: A systematic and technical guide on how to reduce a shoulder dislocation. (http://www.ncbi.nlm.nih.gov/pubmed/27995208) Management of primary anterior shoulder dislocations: a narrative review. (http://www.ncbi.nlm.nih.gov/pubmed/31297678)

Question 15

A 46-year-old woman comes to the office for follow-up of rheumatoid arthritis.  The patient was diagnosed 2 years ago and started on treatment with prednisone and methotrexate; she had a good initial response to treatment, and the oral glucocorticoid was gradually tapered.  However, her symptoms have progressively worsened over the last several months, despite increases in the methotrexate dose.  The patient continues to have hand stiffness and pain lasting 2-3 hours every morning and increasing knee and ankle pain.  She takes frequent doses of acetaminophen and ibuprofen but her symptoms still significantly restrict her daily activities.  Medical history is otherwise unremarkable.  The patient does not use tobacco, alcohol, or illicit drugs.  Physical examination shows symmetric swelling and tenderness of the proximal small hand joints, wrists, and knees.  Blood cell count shows mild normochromic, normocytic anemia with normal leukocyte and platelet counts.  Erythrocyte sedimentation rate is 55 mm/hr.  Hand x-ray reveals periarticular osteopenia and mild joint erosions. The new treatment is initiated and the patient's symptoms gradually improve.  Six months later, she returns to the office with 12 hours of right knee pain.  The patient has had no trauma and says, "This pain is more severe than prior arthritis flare-ups."  Temperature is 38.3 C (101 F), blood pressure is 130/80 mm Hg, pulse is 82/min, and respirations are 14/min.  Right knee examination demonstrates a moderate effusion, warmth, and tenderness.  Range of motion is decreased due to pain.  Which of the following is the best next step in management of this patient's condition?

Accepted Answer

Explanation: Septic arthritis should be considered in patients with rheumatoid arthritis who develop acute monoarthritis, especially those on immunosuppressive therapy or when systemic signs of infection (eg, fever) are also present. Expedited joint aspiration is recommended and fluid should be sent for cell count, Gram stain, and culture. Blood cultures are positive in approximately 50% of patients and should be obtained along with synovial fluid studies prior to antibiotic therapy. Treatment of septic arthritis requires prompt administration of intravenous antibiotics to lessen the likelihood of joint destruction (Choice D). (Choice A) Short courses of glucocorticoids are appropriate for flare-ups of rheumatoid arthritis symptoms. Such flares are typically polyarticular. This patient's acute, severe monoarthritis is more consistent with septic arthritis. (Choice B) MRI for evaluation of septic arthritis is primarily used in joints that are not easily accessible with arthrocentesis (eg, lumbar facet joints, sternoclavicular joints). However, routine use can delay proper diagnosis, and this patient should proceed directly to joint aspiration. (Choice E) Joint replacement is primarily considered for chronic management of late-stage arthritis that limits patient activity. This patient's acute symptoms require immediate action to control infection. Educational objective: Septic arthritis should be considered in patients with rheumatoid arthritis who develop acute monoarthritis, especially in patients on immunosuppressive therapy or when it is accompanied by systemic signs of infection (eg, fever). Expedited joint aspiration is recommended, and fluid should be sent for cell count, Gram stain, and culture.

Question 16

A 12-year-old boy with Down syndrome is brought to the emergency department by ambulance after being in a motor vehicle collision.  The patient was a restrained, rear seat passenger who required extrication from the vehicle.  Temperature is 36.1 C (97 F), blood pressure is 89/60 mm Hg, pulse is 128/min, and respirations are 24/min.  The patient is unconscious and has a cervical neck collar in place.  Multiple lacerations to his face, arms, and abdomen are noted.  A large hematoma is noted across the upper pelvis.  His 17-year-old sister, who was driving the boy to music class, is present.  The sister has several contusions from the airbag but is otherwise alert and oriented.  She is distressed about her brother and says that he has severe impairment in intellectual functioning and requires constant supervision.  The sister states that their parents are on a flight, but they are due to land in the next 30 minutes and can then be contacted by phone.  The grandparents, who also live with them, do not have mobile phones but are expected to arrive in the emergency department within the next 5-10 minutes.  Which of the following is the best next step in management of this patient?

Accepted Answer

Explanation: 11ec2b69_c0ce_0394_8f63_e9c38e58cddc_MD0005_00 A parent or legal guardian of a minor typically must provide informed consent for routine medical care. If immediate medical care is necessary to prevent serious harm or death, consent is implied. This patient experienced life-threatening, traumatic injuries: He is unconscious and tachycardic with a pelvic hematoma concerning for internal hemorrhage. Withholding treatment until parental consent is obtained may lead to death (Choice A). Therefore, treatment should be provided for this child without consent. (Choices B and C) Although some states allow adult siblings or grandparents to provide consent if a parent or legal guardian is unavailable, this child's sister is a minor and the grandparents are not readily available. Treatment of life-threatening injuries should not be withheld for even 5 or 10 minutes to obtain consent from the grandparents. (Choice D) A court order is typically obtained when parents refuse medically necessary, nonemergent treatment for a child, not when they are unavailable to provide consent, as in this case. In an emergency, care should not be delayed by petitioning the court. Educational objective: A parent or legal guardian is typically required to consent for medical care of a minor. However, if immediate medical care is necessary to prevent serious harm or death, treatment may be provided without seeking consent. References: Consent for emergency medical services for children and adolescents. (http://www.ncbi.nlm.nih.gov/pubmed/21788221)

Question 17

A 36-year-old white female with systemic lupus erythematosus was seen in your clinic.  She has been on prednisone for lupus nephritis for the past year.  Decreasing the dose of prednisone below 10 mg per day appears to be associated with an increase in proteinuria.  She is currently on no other medications except for birth control pills.  She denies any symptoms.  She has normal menstrual cycles and is sexually active with one partner.  She has no children.  Because of a fear of weight gain, she has been restricting dairy products in her diet.  She avoids sunlight due to a fear of skin cancer.  She is concerned about bone loss secondary to prednisone use.  Which of the following should be given to prevent bone loss in this patient?

Accepted Answer

Explanation: Glucocorticoid use may lead to a low bone mass due to multiple mechanisms. Glucocorticoids decrease calcium absorption from the gut, cause renal calcium wasting, and have a direct anti-anabolic effect on the bone. Furthermore, they suppress the release of gonadotropin-releasing hormone from the hypothalamus, leading to central hypogonadism, which can aggravate bone loss. This patient is on pharmacological doses of glucocorticoids, has a suboptimal intake of calcium and vitamin D because of poor dietary habits, and has limited sunlight exposure. She is at an increased risk for bone loss. Supplementation of calcium and vitamin D is the most reasonable choice to prevent bone loss. Her current use of birth control pills is also likely to protect her from bone loss. (Choice A and B)Alendronate or risedronate is not indicated at this time. Both of these agents are approved by the FDA for treating glucocorticoid-induced osteoporosis. Treatment could be warranted if bone mineral density studies reveal a T score of <-1.5 that continues to rapidly deteriorate. (Choice C)Fluoride causes an increase in the bone mineral density of the vertebral column; however, an abnormal quality of bone and increased fracture rates have been shown when fluoride is used to treat osteoporosis. (Choice D)Calcitonin is a weak anti-resorptive agent that is used as a nasal spray with a dosage of 200 international units per day; other dosages do not seem to work. It is not approved for use in premenopausal patients. Educational Objective: Achieving optimum calcium and vitamin D levels is the best initial step to prevent bone loss due to prolonged glucocorticoid therapy.

Question 18

A 36-year-old African American woman comes to the emergency department and complains of a severe, throbbing headache that began several hours ago.  She also has acute onset of blurred vision and nausea.  She took acetaminophen, which did not provide any relief.  She was diagnosed with Raynaud phenomena a few years ago.  She recently started having heartburn, for which she takes over-the-counter ranitidine.  Her temperature is 36.7C (98.0F), blood pressure is 200/110 mm Hg, and pulse is 94/min.  Pulse oximetry showed 94% in room air.  There is no abdominal bruit.  Ophthalmologic examination demonstrates bilateral papilledema.  The laboratory results are as follows: CBC
 Serum
 
All her routine laboratory values were completely normal six months ago.  Her blood pressure during her previous visits was always <140/80 mm Hg.  What is the most likely diagnosis of this patient?

Accepted Answer

Explanation: This patient's elevated blood pressure is clearly an acute change based on her prior blood pressure measurements. The most likely diagnosis in this case is scleroderma renal crisis. Underlying scleroderma should be considered in this patient given her history of Raynaud phenomenon and GERD. Scleroderma (systemic sclerosis) is characterized by the abnormal deposition of collagen in multiple organ systems. The majority of the patients have underlying renal involvement, but overt symptoms of scleroderma renal disease are seen less commonly. Thickening of the vessel wall and narrowing of the vascular lumen in the renal arterioles result in ischemia, which activates the renin-angiotensin system. This results in hypertension, which is often as severe as malignant hypertension, as seen in this patient with CNS symptoms and papilledema. It is, therefore, important to monitor the blood pressure in scleroderma patients, and the presence of hypertension should alert the physician to the possibility of scleroderma renal crisis. Acute renal failure is also typical of scleroderma renal crisis and is likely secondary to a combination of severe hypertension superimposed on the abnormal renal vasculature. (Choice A) Another possible cause for a sudden onset of renal crisis is renal artery stenosis secondary to atherosclerosis. However, atherosclerosis is unlikely in a young patient, such as this one, without atherosclerotic risk factors. A bruit is typically present in patients with atherosclerotic disease involving the renal arteries, but a bruit is not seen with scleroderma involvement of the kidneys because only the small arterioles are affected. (Choice B) Fibromuscular dysplasia can be a cause of renal artery stenosis in young patients. However, the presence of Raynaud phenomenon and GERD make scleroderma the more likely diagnosis. (Choice C) Sudden-onset renal failure can be seen in hemolytic uremic syndrome (HUS); however, it is predominantly manifested by hematological abnormalities, (i.e., thrombocytopenia and microangiopathic hemolytic anemia), and there is usually an accompanying history of prior diarrhea. HUS is commonly seen in children. (Choice D) Pheochromocytoma is usually associated with episodes of tachycardia, palpitations, headache, and hypertension. Educational objective: Scleroderma renal crisis presents with severe hypertension and renal failure in a patient with evidence of underlying scleroderma, such as Raynaud phenomenon and GERD. References: Scleroderma renal crisis: a rare but severe complication of systemic sclerosis. (http://www.ncbi.nlm.nih.gov/pubmed/20012923)

Question 19

A 36-year-old African American woman comes to the emergency department and complains of a severe, throbbing headache that began several hours ago.  She also has acute onset of blurred vision and nausea.  She took acetaminophen, which did not provide any relief.  She was diagnosed with Raynaud phenomena a few years ago.  She recently started having heartburn, for which she takes over-the-counter ranitidine.  Her temperature is 36.7C (98.0F), blood pressure is 200/110 mm Hg, and pulse is 94/min.  Pulse oximetry showed 94% in room air.  There is no abdominal bruit.  Ophthalmologic examination demonstrates bilateral papilledema.  The laboratory results are as follows: CBC
 Serum
 Which of the following is the most appropriate pharmacotherapy?

Accepted Answer

Explanation: ACE inhibitors are the agents of choice in the treatment of scleroderma renal crisis since these reverse the angiotensin-induced vasoconstriction. Most patients respond favorably if these drugs are used promptly after the diagnosis, and the goal should be to reduce blood pressure to baseline over 72 hours. The ACE inhibitor of choice in these patients is captopril, given that it has a relatively short time to onset and is the agent with which there is the most clinical experience for scleroderma renal crisis. ACE inhibitors also alleviate renal failure in the majority of patients in the long term, although they may temporarily worsen renal failure in the short term. Since ACE inhibitors result in efferent arteriole vasodilation, there may be a mild drop in GFR and subsequent worsening of creatinine, which is already elevated in this patient as is commonly seen in scleroderma renal crisis. Creatinine should be closely monitored in these patients, but generally any increase in creatinine is mild and does not warrant cessation of the ACE inhibitor. Although ACE inhibitors are generally avoided in most patients with acute renal failure, scleroderma renal crisis is an exceptionto this general rule as long as renal function is closely monitored. In addition, in patients such as this one with CNS manifestations (eg, malignant hypertension), intravenous nitroprusside is added to acutely lower the blood pressure. This should be done cautiously as a sudden reduction of blood pressure is not favorable because it can further compromise renal perfusion and result in acute tubular necrosis; in addition, nitroprusside use should be very carefully monitored in the setting of renal injury (due to the risk of cyanide poisoning). Nitroprusside should be used for a short duration but is generally necessary in patients with scleroderma renal crisis who have CNS manifestations in order to gain control of the blood pressure acutely as the captopril takes effect. (Choice A) Calcium channel blockers may be used as a second-line agent in addition to captopril in the management of chronic hypertension in patients with scleroderma. Calcium channel blockers may also be of benefit for treatment of Raynaud phenomenon in these patients. (Choice B) Lasix would not be helpful for this patient as volume overload is not the underlying problem. (Choice D) Intravenous nitroprusside is frequently utilized in the management of malignant hypertension. However, starting captopril should be a priority in these patients as this is the agent of choice for treating scleroderma renal crisis. (Choice E) Clonidine patch will take longer to start its effect. Educational objective: ACE inhibitors, particularly captopril, are the drugs of choice for the treatment of hypertension in scleroderma renal crisis. The addition of intravenous medications such as nitroprusside is needed if CNS manifestations or papilledema is present. References: Long-term outcomes of scleroderma renal crisis. (http://www.ncbi.nlm.nih.gov/pubmed/11033587)

Question 20

A Schirmer test confirms inadequate tear production in this patient and anti-Ro/SSA antibodies are positive.  Which of the following is the most likely explanation for this patient's neck mass?

Accepted Answer

Explanation: The Schirmer test is used to confirm the diagnosis of keratoconjunctivitis sicca. Filter paper is placed along the lower eyelid and wetting of the paper is measured after a defined period of time. Too little wetting of the paper is consistent with abnormal tear production. The presence of anti-Ro/SSA antibodies also helps to confirm the diagnosis of Sjogren's syndrome. The existence of a focal submandibular mass should raise concern for a B-cell non-Hodgkin's lymphoma in patients with Sjogren's syndrome. Sjogren's disease results in polyclonal B cell activation and infiltration of the salivary glands. This increased lymphocyte activation can go awry and result in the development of B-cell lymphoma in some patients. The lifetime risk of B-cell lymphoma is approximately 5% for Sjogren's syndrome patients - much higher than the risk for the general population. (Choice A) Adenocarcinoma from anywhere in the body can metastasize to neck lymph nodes, but this patient has no risk factors for adenocarcinoma, and her Sjogren's syndrome makes lymphoma much more likely. (Choice C) The chronic inflammation in Sjogren's syndrome is not associated with T cell activation, and therefore there is no increased risk of T cell lymphoma. (Choice D) Sjogren's syndrome is not associated with Hodgkin's lymphoma. Patients with Hodgkin's disease may have B symptoms consisting of fever, fatigue, and pruritus. (Choice E) Metastatic squamous cell carcinoma could present as a pathologic lymph node in the submandibular region. However, this is unlikely in this patient given the absence of alcohol or tobacco use. Educational objective: B lymphocyte activation occurs as a component of the chronic inflammation in patients with Sjogren's syndrome. This activation results in an increased risk of B cell lymphoma. Metastatic squamous cell carcinoma frequently spreads to lymph nodes in the head and neck but is usually associated with a history of tobacco or alcohol use. References: Hematologic manifestations and predictors of lymphoma development in primary Sjögren syndrome: clinical and pathophysiologic aspects. (http://www.ncbi.nlm.nih.gov/pubmed/19745687)

Exam 1: Advanced Clinical Medicine

A Schirmer test confirms inadequate tear production in this patient and anti-Ro/SSA antibodies are positive. Which of the following is the most likely explanation for this patient's neck mass?