Wednesday, December 22, 2010

Clinical Case: Woman With Fevers and Fatigue ........

Clinical Vignette: A 32-year-old African American woman complains of mild fevers and fatigue for the past month. She has no significant past medical history. Her temperature is 38.I C (100.6 F), blood pressure is 115/70 mm Hg, pulse is 75/min, and respirations are 18/min. Nontender, mobile, cervical and axillary lymph nodes are noted. Auscultation of the lungs reveals fine crackles bilaterally. A chest x-ray film shows hilar lymphadenopathy and diffuse interstitial infiltrates. Lymph node biopsy shows noncaseating granulomas. Which of the following is the most appropriate therapy?

(A) Allopurinol
(B) Angiotensin converting enzyme (ACE) inhibitor
(C) Cyclosporine
(D) Gluoocorliooids
(E) Isoniazid

The correct answer is D. This clinical vignette will be asked on the USMLE one way or the other... 
This patient has pulmonary sarcoidosis. The peak age group for sarcoidosis is 20-40 years, and the disease seems to be more common in blacks. Noncaseating granulomas can occur in the lungs, heart, kidneys, skin, liver, or other organs. Most characteristically, the patients are asymptomatic, and the disease is detected by an abnormal chest x-ray film. which usually shows bilateral symmetric hilar adenopathy often associated with paratracheal adenopathy and/or parenchymal infiltrates. Patients may have uveitis, peripheral arthritis, skin involvement with granulomas, or erythema nodosum. The lungs are the most frequently involved organs; pulmonary symptoms, when present, include dyspnea on exertion, nonproductive cough, and wheezing. Radiologic abnormalities are graded 0-3. Grade 0 is associated with a normal x-ray. Grade I is associated with lymph node enlargement without pulmonary parenchymal abnormalities. Grade 2A is a combination of lymph node and diffuse pulmonary parenchymal disease. Grade 2B is a diffuse parenchymal disease without lymph node enlargement,
Grade 3 is associated with radiographic changes indicating more chronic disease with pulmonary fibrosis (“honey-combing”). Many patients show spontaneous total remission of disease for a period up to 3 years.
Prednisone is usually the drug of choice for treatment, with a starting dose of 30-40 mg/day. Neither allopurinol (choice A) nor cyclosporine (choice C), an immune modulator, has been proven to
be of benefit in sarcoidosis.

Levels of ACE may be elevated in patients with sarcoidosis but are also elevated in many other diseases. This enzyme elevation is thought to be related to induction by the granulomas. There is no evidence that ACE inhibitors (choice B) have any therapeutic value in treatment of sarcoidosis.
At one point in history, some theorized that sarcoidosis was caused by a type of mycobacterium, related to tuberculosis. However, this has not been definitively proven. Furthermore, isoniazid (choice E) has not been shown to be beneficial.