A 30-year-old man comes to the office due to shortness of breath, nonproductive cough, and fatigue for the past 3-4 weeks. He also has been urinating more than usual. The patient has no other medical issues and currently takes no medications. He has smoked a pack of cigarettes daily for 8 years. Vital signs are normal. Cardiopulmonary examination shows scattered crackles but is otherwise unremarkable. The abdomen is soft and nontender. Laboratory results are as follows: Chest x-ray reveals bilateral hilar fullness and interstitial infiltrates. Results of HIV testing and purified protein derivative skin testing are negative. Which of the following is the best therapy for this patient?

Question

A 30-year-old man comes to the office due to shortness of breath, nonproductive cough, and fatigue for the past 3-4 weeks.  He also has been urinating more than usual.  The patient has no other medical issues and currently takes no medications.  He has smoked a pack of cigarettes daily for 8 years.  Vital signs are normal.  Cardiopulmonary examination shows scattered crackles but is otherwise unremarkable.  The abdomen is soft and nontender.  Laboratory results are as follows:  Chest x-ray reveals bilateral hilar fullness and interstitial infiltrates.  Results of HIV testing and purified protein derivative skin testing are negative.  Which of the following is the best therapy for this patient?

Quizplus · Accepted Answer

11ec25c8_d523_e8ee_bc81_e7b92c677610_MD0003_00 This patient's presentation with 3-4 weeks of dyspnea, fatigue, and nonproductive cough with bilateral hilar fullness and interstitial infiltrates on chest x-ray is consistent with sarcoidosis. Diagnosis is made by a combination of compatible clinical presentation and imaging findings, exclusion of other diagnoses, and noncaseating granulomas on biopsy. Although neither sensitive nor specific, hypercalcemia (present in this patient) occurs in up to 20% of patients and can be an additional clue for diagnosis. Hypercalcemia can cause frequent urination, possibly due to calcium deposition leading to tubulointerstitial dysfunction and impaired renal tubule concentrating ability. The mechanism responsible for hypercalcemia is granulomatous activity of 1-alpha hydroxylase causing increased production of 1,25-dihydroxyvitamin D and increased intestinal calcium absorption. Hypercalciuria (occurring in up to 50% of patients) is present more often than hypercalcemia and can result in nephrolithiasis. Patients with sarcoidosis who are asymptomatic are often followed without treatment due to a high rate of spontaneous remission. However, patients with symptomatic disease (eg, cough, dyspnea, fatigue, hypercalcemia) are treated with systemic glucocorticoids. It is important to rule out infectious granulomatous diseases with similar presentations, such as tuberculosis (TB) and histoplasmosis, prior to initiation of glucocorticoids. (Choice A) Antifungal therapy (eg, itraconazole) would be indicated for treatment of infection with endemic fungi such as histoplasmosis. Sarcoidosis and histoplasmosis may present with similar symptoms and chest x-ray findings, but histoplasmosis infection is rare in an immunocompetent patient and leukocytosis would be expected. (Choice B) TB can present similarly to sarcoidosis, but the negative purified protein derivative skin test makes TB unlikely in this patient with no evidence of immunosuppression. (Choice C) Bisphosphonates are indicated in the management of severe, symptomatic hypercalcemia, usually due to malignancy. This patient's hypercalcemia is likely due to sarcoidosis and should respond to treatment with glucocorticoids. (Choice D) Chemotherapy is indicated in the treatment of Hodgkin lymphoma, which, like sarcoidosis, often presents with mediastinal adenopathy and fatigue. Hypercalcemia can occur but is rare, and interstitial infiltrates are not typically seen on chest x-ray. (Choice F) Parathyroidectomy is indicated in advanced manifestations of primary hyperparathyroidism. This patient's hypercalcemia is more likely due to sarcoidosis than primary hyperparathyroidism. Educational objective: Sarcoidosis is diagnosed in the setting of a compatible history, chest x-ray findings of hilar adenopathy with or without reticulonodular infiltrates, and noncaseating granulomas on biopsy. Hypercalcemia can be present due to granulomatous production of 1,25-dihydroxyvitamin D. Symptomatic disease is treated with systemic glucocorticoids. References: Calcium and vitamin D metabolism in sarcoidosis. (http://www.ncbi.nlm.nih.gov/pubmed/24071882)

A 30-Year-Old Man Comes to the Office Due to Shortness