Diffuse pulmonary amyloidosis that mimics interstitial lung disease in a patient with familial Mediterranean fever.
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.
Department of Medicine, Division of Rheumatology, Gülhane Military School of Medicine, Etlik/Ankara, 06018, Turkey. herdem67@gata.edu.tr
Hakan
H
Erdem
Ismail
I
Simşek
Salih
S
Pay
Ayhan
A
Dinc
Omer
O
Deniz
Ayhan
A
Ozcan
D000686:Amyloidosis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D001706:Biopsy; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D005260:Female; D006801:Humans; D007677:Kidney Function Tests; D008171:Lung Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000473:pathology; D008875:Middle Aged; D014057:Tomography, X-Ray Computed