A 43-year-old woman comes to the office due to progressive fatigue and intermittent pain in the hands, feet, and knee joints. The pain caused the patient to stop her daily yoga routine 2 months ago, and she has had difficulty performing daily household activities. She has had no fever, rash, or oral ulcers but frequently drinks water or chews gum due to her mouth feeling dry. The patient has no prior medical conditions and takes no medications or herbal supplements. She does not use tobacco, alcohol, or illicit drugs. Vital signs are within normal limits. Physical examination shows no muscle tenderness or joint swelling. The thyroid gland is normal to palpation. There is nontender diffuse swelling of the parotid glands. Laboratory evaluation reveals mild normocytic normochromic anemia and an elevated erythrocyte sedimentation rate. A salivary gland biopsy image representative of this patient's disease process is shown in the exhibit. This patient is at greatest risk for which of the following due to her current condition?

Question

A 43-year-old woman comes to the office due to progressive fatigue and intermittent pain in the hands, feet, and knee joints.  The pain caused the patient to stop her daily yoga routine 2 months ago, and she has had difficulty performing daily household activities.  She has had no fever, rash, or oral ulcers but frequently drinks water or chews gum due to her mouth feeling dry.  The patient has no prior medical conditions and takes no medications or herbal supplements.  She does not use tobacco, alcohol, or illicit drugs.  Vital signs are within normal limits.  Physical examination shows no muscle tenderness or joint swelling.  The thyroid gland is normal to palpation.  There is nontender diffuse swelling of the parotid glands.  Laboratory evaluation reveals mild normocytic normochromic anemia and an elevated erythrocyte sedimentation rate.  A salivary gland biopsy image representative of this patient's disease process is shown in the exhibit.  This patient is at greatest risk for which of the following due to her current condition?

Quizplus · Accepted Answer

11ec160b_3a6a_ecdf_9249_e564ff146af4_MD0001_00 This patient with chronic dry mouth and palpable salivary gland enlargement has extensive lymphocytic infiltrate with a germinal center on salivary gland biopsy, consistent with Sjögren syndrome (SS). SS is an autoimmune disorder characterized by a lymphocytic inflammatory infiltrate in the exocrine glands. It presents with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Extraglandular manifestations such as arthralgias and cutaneous vasculitis can also occur due to deposition of circulating immune complexes. Complications of SS can include corneal ulcerations (due to diminished lacrimal gland function) and an increase in dental caries (due to diminished salivary gland function). The chronic B-lymphocyte stimulation in SS also predisposes patients to malignant transformation, causing non-Hodgkin lymphoma (eg, marginal zone lymphoma, diffuse large B-cell lymphoma). (Choice A) Systemic lupus erythematosus is associated with increased risk for arterial and venous thrombosis due to autoantibody production (antiphospholipid antibodies). Patients with this disease also often have arthralgias and anemia, but dry mouth and parotid enlargement in association with the characteristic biopsy findings are more characteristic of SS. (Choice B) Atlantoaxial subluxation (ie, displacement of C1 on C2 vertebrae) is caused by ligamentous deterioration in the cervical spine. It can be seen in a variety of inflammatory disorders (eg, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis) but is not common in SS. (Choice D) Renal artery stenosis (occlusion) is most commonly seen in the context of advanced atherosclerosis or fibromuscular dysplasia and often causes treatment resistant hypertension. (Choice E) Restrictive cardiomyopathy is often idiopathic or can be associated with infiltrative disorders (eg, amyloidosis, sarcoidosis, hemochromatosis). Cardiac manifestations of SS are rare and do not include restrictive disease. Educational objective: Sjögren syndrome (SS) is an autoimmune disorder characterized by lymphocytic inflammation in exocrine glands (eg, lacrimal, salivary glands). It presents with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Chronic B-cell proliferation in patients with SS increases the risk of developing non-Hodgkin lymphoma. __________ References: Marginal zone lymphoma: associated autoimmunity and auto-immune disorders. (http://www.ncbi.nlm.nih.gov/pubmed/28288719) Sjögren syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/27383445)

A 43-Year-Old Woman Comes to the Office Due to Progressive