Diffuse pulmonary amyloidosis that mimics interstitial lung disease in a patient with familial Mediterranean fever

Abstract

Familial Mediterranean fever (FMF) is an autosomal-recessive disorder characterized by recurrent attacks of fever, serositis, and arthritis. Amyloidosis, which mostly affects the kidneys, represents the most serious complication of the disease. The lungs, particularly after the onset of renal failure, may be asymptomatically involved in some of the patients with AA amyloidosis secondary to FMF. However, clinically detectable pulmonary amyloidosis is quite rare, and only 2 cases of pulmonary amyloidosis secondary to FMF have been reported so far. We describe a patient with pulmonary amyloidosis who had pulmonary hypertension and presented with clinical and radiologic features highly suggestive of interstitial lung disease. Amyloidosis was diagnosed only after lung biopsy. FMF was confirmed by molecular analysis.