AA-amyloidosis in Autosomal Dominant Polycystic Kidney Disease Caused by Chronic Cyst Infections Lasting for 30 years

Abstract

We herein report the case of a 66-year-old Japanese woman who was admitted to our hospital due to diarrhea and malaise. She had been diagnosed with autosomal dominant polycystic kidney disease (ADPKD) at 35 years of age and had suffered from recurrent cyst infections since that time. Antibiotic therapy combined with hepatic cyst drainage and cyst sclerosing therapy led to transient improvements each time. At 66 years of age, watery diarrhea occurred. The patient's serum albumin level declined to 1.8 g/dL, and her C-reactive protein level was 4.5 mg/dL. An endoscopic biopsy of the descending colon revealed amorphous deposits in the small arteries and tissues of the submucosal layer. The deposits were positive for Congo Red staining and amyloid A staining. Therefore, AA-amyloidosis was diagnosed. An endoscopic biopsy of the stomach and duodenum also showed AA-amyloid deposits. If an ADPKD patient with a long history of cyst infection develops diarrhea and malaise, AA-amyloidosis should be considered as a possible complication.

Chronic renal failure due to amyloid nephropathy caused by chronic infection after total hip replacement

“…A previous report showed that a patient with filariasis-a condition associated with chronic inflammation-receiving antifilarial therapy suffered from reversible acquired AA-type renal amyloidosis [15]. In a patient with autosomal dominant polycystic kidney disease, recurrent infections from renal or hepatic cysts lasting 30 years induced AA amyloidosis in gastrointestinal tract [16]. In our case, a localized, yet prolonged infection, around the region of the replaced hip, could have led to secondary amyloidosis.…”

De novo amyloidosis in a renal transplant patient

“…Chronic infections including tuberculosis, bronchiectasis, osteomyelitis are well‐known causes of AA amyloidosis. Even chronic cyst infections in autosomal dominant polycytic kidney disease reported to be responsible for the development of AA amyloidosis . These cases suggested that, although very unusual, chronic infections including febrile UTIs might cause de novo amyloidosis in a renal transplant patient.…”

Chronic brucellosis with hepatic brucelloma and AA amyloidosis in a patient with autosomal dominant polycystic kidney disease

“…Case reports of association of ADPKD with secondary systemic amyloidosis are on record in the literature (Table 1). [8][9][10][11] The inciting event for such amyloidosis in the reported cases is primarily attributed to chronic renal cyst infection in 2 cases, chronic hepatic cyst infection and pulmonary tuberculosis in one case each. AA amyloidosis is well known to be associated with chronic infections and chronic inflammatory conditions.…”

“…A total of four case reports have documented histological evidence of AA amyloidosis in patients with ADPKD. [8][9][10][11] The etiology of amyloidosis was ascribed to chronic renal cyst infections in two cases, chronic hepatic cyst infections in one case and pulmonary tuberculosis in one case. [8][9][10][11] AA amyloidosis is well known to be associated with chronic infections and chronic inflammatory states.…”

“…[8][9][10][11] The etiology of amyloidosis was ascribed to chronic renal cyst infections in two cases, chronic hepatic cyst infections in one case and pulmonary tuberculosis in one case. [8][9][10][11] AA amyloidosis is well known to be associated with chronic infections and chronic inflammatory states. 12 In the present study, the patient had chronic brucellosis, which may be ascribed to be the cause of AA amyloidosis.…”