A 56-year-old man comes to the physician with fatigue, bilateral knee and ankle pain, and left-sided chest pain. He has awakened in an uncomfortable sweat several nights during the last 4 weeks. He experienced exertional shortness of breath and was diagnosed with non-ischemic cardiomyopathy a year ago. During his initial evaluation, his left ventricular ejection fraction was 20%, and cardiac catheterization showed no significant coronary disease. The patient's medications include carvedilol, hydralazine, isosorbide dinitrate, furosemide, and spironolactone. He received an automatic implantable cardioverter-defibrillator for primary prevention of cardiac death 6 months ago. He is allergic to angiotensin-converting enzyme inhibitors, which caused facial angioedema. His blood pressure is 142/90 mm Hg and his heart rate is 65/min and regular. There is no jugular venous distension. Breath sounds are decreased at the left base due to splinting. There is no splenomegaly. There is a small effusion in the right knee with mild tenderness. There are no skin rashes. Chest x-ray shows cardiomegaly and a small left pleural effusion. Which of the following is the best next step in management of this patient?

Question

A 56-year-old man comes to the physician with fatigue, bilateral knee and ankle pain, and left-sided chest pain.  He has awakened in an uncomfortable sweat several nights during the last 4 weeks.  He experienced exertional shortness of breath and was diagnosed with non-ischemic cardiomyopathy a year ago.  During his initial evaluation, his left ventricular ejection fraction was 20%, and cardiac catheterization showed no significant coronary disease.  The patient's medications include carvedilol, hydralazine, isosorbide dinitrate, furosemide, and spironolactone.  He received an automatic implantable cardioverter-defibrillator for primary prevention of cardiac death 6 months ago.  He is allergic to angiotensin-converting enzyme inhibitors, which caused facial angioedema.  His blood pressure is 142/90 mm Hg and his heart rate is 65/min and regular.  There is no jugular venous distension.  Breath sounds are decreased at the left base due to splinting.  There is no splenomegaly.  There is a small effusion in the right knee with mild tenderness.  There are no skin rashes.  Chest x-ray shows cardiomegaly and a small left pleural effusion.  Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

Explanation: This patient's presentation - fatigue, night sweats, arthralgia/arthritis, and pleuritis/pleural effusion - is suggestive of drug-induced lupus due to hydralazine. It is seen in approximately 5%-10% of patients receiving hydralazine and occurs due to development of autoantibodies that trigger an autoimmune response and a clinical syndrome similar to systemic lupus erythematosus. Other autoantibodies, with the exception of anti-histone antibodies, are uncommon in this disorder. Drug-induced lupus causes multisystem involvement, and clinical findings can include fever, malaise, myalgia, arthralgia/arthritis, serositis (pleuritis, pericarditis), and/or hepatomegaly/splenomegaly. The diagnosis is made by a history of one or more offending drugs and a positive test for antinuclear antibodies (ANAs). Some common drugs associated with risk of lupus include procainamide, hydralazine, minocycline, and anti-tumor necrosis factor (TNF) alpha therapy (etanercept, infliximab). Treatment is generally symptomatic, and discontinuation of the offending drug typically leads to resolution of symptoms within weeks to months. (Choice B) The patient is tolerating diuretics quite well without side effects and has compensated nonischemic cardiomyopathy. Discontinuation of diuretics can lead to worsening congestive heart failure. (Choice C) The patient had no significant coronary disease on cardiac catheterization and has no symptoms of progressive coronary artery disease. Exercise echocardiography is not indicated at this point. (Choice D) Oral doxycycline is used for the treatment of Lyme disease. Lyme disease is a tick-borne illness that typically presents with a characteristic skin rash and erythema migrans at the site of the bite in the early-localized phase. The next phase, early-disseminated disease, is characterized by multiple erythema migrans lesions. In addition, neurologic (eg, peripheral neuropathy, cranial nerve palsies, lymphocytic meningitis) and/or cardiac involvement (eg, myopericarditis, atrioventricular block) occurs weeks to months after the initial infection. Late disease (months to years) is associated with arthritis of the large joints and mild neurological manifestations (encephalopathy or polyneuropathy). This patient's clinical presentation is not consistent with Lyme disease. (Choice E) Troponin I levels are typically elevated in patients with acute or recent myocardial infarction. Measurement of brain natriuretic peptide (BNP) levels is useful in the evaluation of patients with symptomatic left ventricular dysfunction and heart failure. This patient's presentation is suggestive of drug-induced lupus, and measurement of troponin or BNP levels is not useful. Educational objective: Drug-induced lupus causes multisystem involvement; clinical findings include fever, malaise, myalgia, arthralgia/arthritis, serositis (pleuritis, pericarditis), and/or hepatomegaly/splenomegaly. The diagnosis is made by typical symptoms, history of taking an offending drug, and a positive test for antinuclear and anti-histone antibodies. References: Drug-induced lupus erythematosus. (http://www.ncbi.nlm.nih.gov/pubmed/16373256) Drug-induced arthritic and connective tissue disorders. (http://www.ncbi.nlm.nih.gov/pubmed/18166218)

A 56-Year-Old Man Comes to the Physician with Fatigue, Bilateral