A 35-year-old immigrant from Eastern Europe comes to the office due to dry cough, malaise, and fatigue. He has had joint aches and involuntary weight loss over the past 3 months. His other medical problems include severe allergies that manifest as nasal stuffiness, headache, and ear pain. He takes over-the-counter decongestants. He is a construction worker, smokes a pack of cigarettes a day, and drinks 6 cans of beer once a week. His mother suffers from severe rheumatoid arthritis.
His temperature is 37.2 C (99 F), blood pressure is 122/70 mm Hg, and pulse is 89/min. He has tenderness on palpation of bilateral maxillary sinuses. Tympanic membranes are full and bulging bilaterally. Pulmonary auscultation reveals occasional rhonchi. There are no heart murmurs. The tip of the spleen is not palpable. There are no skin rashes or joint swelling.
Laboratory results are as follows:

Liver panel is unremarkable. Urinalysis is positive for blood and protein, and urine sediment analysis reveals multiple red blood cells casts. Chest x-ray is shown below.

Which of the following is most likely to establish this patient's diagnosis?

Question

A 35-year-old immigrant from Eastern Europe comes to the office due to dry cough, malaise, and fatigue.  He has had joint aches and involuntary weight loss over the past 3 months.  His other medical problems include severe allergies that manifest as nasal stuffiness, headache, and ear pain.  He takes over-the-counter decongestants.  He is a construction worker, smokes a pack of cigarettes a day, and drinks 6 cans of beer once a week.  His mother suffers from severe rheumatoid arthritis.
His temperature is 37.2 C (99 F), blood pressure is 122/70 mm Hg, and pulse is 89/min.  He has tenderness on palpation of bilateral maxillary sinuses.  Tympanic membranes are full and bulging bilaterally.  Pulmonary auscultation reveals occasional rhonchi.  There are no heart murmurs.  The tip of the spleen is not palpable.  There are no skin rashes or joint swelling.
Laboratory results are as follows:

Liver panel is unremarkable.  Urinalysis is positive for blood and protein, and urine sediment analysis reveals multiple red blood cells casts.  Chest x-ray is shown below.

Which of the following is most likely to establish this patient's diagnosis?

Quizplus · Accepted Answer

11ec193a_9925_033d_853d_277ad64cdca5_MD0002_00 This patient's clinical presentation is consistent with a systemic vasculitis. The history of weight loss, arthralgias, anemia of chronic disease, ear/sinus symptoms, pulmonary nodules, and renal insufficiency with glomerulonephritis suggests granulomatosis with polyangiitis (GPA), formerly Wegener granulomatosis. It is an anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, with nearly 90% of patients testing ANCA positive. However, the absence of ANCA does not exclude GPA. Immunofluorescence test results are reported as c-ANCA (cytoplasmic pattern) or p-ANCA (perinuclear pattern) titers. The c-ANCA pattern is caused by anti-proteinase 3 (PR3) antibodies, and the p-ANCA pattern is caused by anti-myeloperoxidase (MPO) antibodies. The c-ANCA pattern and anti-PR3 antibodies are most commonly associated with GPA; p-ANCA pattern and MPO are associated with microscopic polyangiitis (Choice B). However, patients with typical clinical presentation and tissue biopsy confirming vasculitis can be diagnosed with GPA, even with positive p-ANCA or negative ANCA. (Choice A) Anti-double-stranded DNA antibodies are more specific for the diagnosis of systemic lupus erythematosus. However, this patient does not have other clinical criteria (eg, malar rash, photosensitivity, or pleurisy) for this disease. In addition, the pulmonary nodules and sinus/ear disease are more common with GPA. (Choice D) Bone marrow biopsy would be indicated to evaluate for thrombocytosis due to myeloproliferative disorders. However, this patient likely has reactive thrombocytosis from vasculitis and does not have significant white blood cell or red blood cell abnormalities to warrant a biopsy. (Choice E) Tuberculin skin testing may be indicated for this patient's pulmonary symptoms combined with weight loss. However, the renal involvement with active urinary sediment makes tuberculosis less likely. Educational objective: Granulomatosis with polyangiitis (GPA) presents classically with sinus disease, pulmonary disease, renal disease, and systemic symptoms. The c-ANCA pattern and anti-PR3 antibodies are most commonly associated with GPA. Diagnosis is confirmed by tissue biopsy showing vasculitis. __________ References: Antineutrophil cytoplasmic antibodies. (http://www.ncbi.nlm.nih.gov/pubmed/16876669) Wegener's granulomatosis: an update on diagnosis and therapy. (http://www.ncbi.nlm.nih.gov/pubmed/20477576) Clinical manifestations and treatment of Wegener's granulomatosis. (http://www.ncbi.nlm.nih.gov/pubmed/20688247)

A 35-Year-Old Immigrant from Eastern Europe Comes to the Office