A Tale of Three Rarities: Secondary Amyloid A (AA) Amyloidosis Caused by Recurrent Sialadenitis and Complicated by Pulmonary Hypertension and Adrenal Insufficiency

A 48-year-old lady presented with a parotid mass found to be secondary to recurrent sialadenitis. She was also found to have microcytic anemia, renal dysfunction, an elevated gamma gap, and an isolated alkaline phosphatase elevation. Later, she developed altered mental status and shock, and was found to have adrenal insufficiency, pulmonary hypertension, and pulmonary nodules. A liver biopsy was consistent with amyloid deposition. The constellation of findings was consistent with systemic amyloid A (AA) amyloidosis secondary to recurrent sialadenitis with hepatic, renal, pulmonary, and adrenal involvement. The patient later passed away due to acute hypoxic respiratory failure. This case demonstrates rare sequelae of systemic AA amyloidosis of pulmonary hypertension and adrenal insufficiency.

Keywords: adrenal insufficiency; hematology; pulmonary hypertension; secondary amyloidosis.