POS-472 EPIDEMIOLOGY AND CLINICAL PROFILE OF RENAL AMYLOIDOSIS: SINGLE-CENTER EXPERIENCE

Abstract

The kidney is the most common organ involved in systemic amyloidosis. Indeed, Kidney disease is a serious manifestation and a major cause of morbidity and mortality. We aimed to study etiology and clinicopathological profile of renal amyloidosis in our center. This was a retrospective record review of cases of amyloidosis diagnosed in the nephrology department in Monastir between January 2010 and November 2020. 41 cases of amyloidosis were identified over the study period. The calculated biopsy prevalence was 4.1 per 100 non-transplant renal biopsies. Amyloid A (AA) amyloidosis was the most common type of amyloidosis observed in 61% of cases. Amyloid light chain (AL) amyloidosis accounted for 19.5% of cases, and the rest remained undetermined. The mean age at presentation was 55± 16 years with a male-to-female ratio of 1.73. AA amyloidosis had widespread age distribution but relatively younger, while AL amyloidosis was confined to those >40 years The main clinical presentation was proteinuria (97%) with nephrotic range among 66 patients (Mean 24 h proteinuria was 7,8 g). Renal failure was the second most common manifestation seen in 58,5% of patients, of whom 12,5% required emergent hemodialysis and 83% evolved to chronic renal failure. Bronchietasis and chronic infection disease accounted for 26% and 29% cases of AA amyloidosis respectively. AL amyloidosis was mainly associated with multiple myeloma (37,5%) while primitive amyloidosis was seen in 5 cases. Cardiac involvement was seen in ten patients mostly stage II Mayoclinic. Median survival after diagnosis was 46 months. Amyloidosis is a rare cause of kidney disease and typically presents with nephrotic syndrome. AA amyloidosis was the most common type of renal amyloidosis in this cohort. While chronic obstructive pulmonary disease remains the major underlying disease in this type, Tuberculosis was unexpectedly infrequent. Prevalence of AL amyloidosis noted in our study was higher than previous Tunisian studies.