Pleural Amyloidosis

Abstract

Pleural involvement in primary (AL) amyloidosis was reported. Pulmonary involvement in patients with primary amyloidosis is not so uncommon, but the pleura is regarded as an unusual site for amyloid deposition. A woman, born in 1942, was referred for evaluation of a four-month history of edema of eye lids and lower legs in 1987. Nephrotic syndrome with Bence Jones (lambda-type) proteinuria was demonstrated. She was diagnosed of having renal amyloidosis. In 1990, X-ray films incidentally demonstrated a small amount of right pleural effusion. A needle biopsy of the pleura was performed. Congo red stain demonstrated amyloid deposition in the pleura. The following clinical features indicating systemic amyloidosis occurred in succession: orthostatic hypotension, diarrhea or constipation, carpal tunnel syndrome, and weight loss. In 1993, sudden death due to cardiac arrest occurred. The patient survived 6 years after the diagnosis of renal amyloidosis. A combined use of melphalan, prednisolone, colchicine, and dimethyl sulfoxide (DMSO) might have contributed to the long survival of the patient. At postmortem examination, systemic deposition of type AL amyloid was confirmed. In patients with pleural effusion and multiorgan involvement or monoclonal gammopathy, a closed pleural biopsy should be performed, especially if the effusion is transudative.