The Clinical Features and Outcomes of Renal Amyloidosis in Tunisia

Abstract

Systemic amyloidosis is a rare disease characterized by clinical polymorphism. Indeed, the kidney is the most common organ involved and represents a real turning point in the disease. We aimed to determine the clinicopathological prognostic factors of renal amyloidosis (RA). We conducted a retrospective study including 40 cases with biopsy-proven RA collected in our department over a period of 10 years. Biochemical, demographic, and clinicopathological findings at diagnosis, as well as the follow-up data, were evaluated for each patient. The prevalence of amyloidosis was 2.7 per 100 nontransplant renal biopsies. The mean age at presentation was 55.5 ± 15.6 years with a male-to-female ratio of 1.85. The diagnosis of RA was confirmed by a renal biopsy in 85% of cases. Amyloid A (AA) amyloidosis was the most common type of amyloidosis (65%), and chronic infections ranked first in the panel of etiologies (41%). Amyloid light chain amyloidosis was mainly associated with multiple myeloma (57%). The median patient survival was 59 months versus 12 months for kidney survival. Age and extrarenal localization were independent predictors of mortality, whereas renal failure at presentation significantly influenced renal survival. The results of our study emphasize the rarity but also the severity of RA. AA amyloidosis was the most common type identified, which was mainly caused by chronic infections. Prevention remains the best solution until we can achieve therapeutic advances in inflammatory diseases.