engleski

Fibrillary glomerulonephritis: a report of 11 cases from a single center

INTRODUCTION: Fibrillary glomerulonephritis (FGN) is a rare glomerular disease found in less than 1% of native kidney biopsies. FGN is characterized by glomerular presence of randomly arranged fibriles measuring 12 to 25 nm by electron microscopy. FGN is usually idiopathic (primary), however, it has been associated with underlying infective, malignant or sistemic autoimmune disease in some patients as well. Therapeutic strategies in primary FGN, particularly the use of immunosuppressive drugs, are not clearly defined. We aimed to investigate clinical characteristics and outcomes of patients diagnosed with FGN in our center. METHODS: We identified patients with FGN by retrospective review of all renal biopsies in the Department of Pathology, University Hospital Dubrava, from 2009 until 2018. Clinical and histologic features of patients and kidney disease outcomes were analyzed. For the purpose of outcome analysis, following definitions were used: complete remission (CR) was defined as reduction of proteinuria to <0.5 g/d with normal kidney function, partial remission (PR) was defined as reduction in proteinuria by >50% and to <2 g/d with stable kidney function (no more than a 20% increase in serum creatinine), end-stage renal disease (ESRD) was defined as decrease in eGFR (based on MDRD formula) under 15 ml/min/1.73m² or beginning of renal replacement therapy and persistent renal dysfunction (PRD) was defined as failure to meet criteria for CR and PR but not reaching ESRD. RESULTS: There were 11 patients with FGN, whose mean age at biopsy was 60 years and 9 of them were females. Presentation at the time of biopsy included proteinuria (in 9 patients), hematuria (8), reduced eGFR < 60 ml/min/1.73m² (7), hypertension (9), pretibial oedema (4) and anemia (4). Primary FGN was found in 8 patients, 2 had autoimmune diseases (SLE and primary antiphospolipide syndrom) and one had monoclonal gamapathy. Three patients were treated with renin-angiotensive-aldosterone system (RAAS) blockade alone, six (including 2 with autoimmune diseases) with combination of steroids and RAAS blockade and one patient was treated only with supportive therapy without progression of disease. In patients treated with combination of RAAS blockade and steroids, cyclophosphamide was added in three and rituximab in two cases with favorable effect. Two patients were lost to follow-up (one with primary FGN and one with secondary). Of the remaining patients with primary FGN two reached ESRD, two entered sustained remission (one had CR and one had PR) and two patients had PRD. Of the patients with secondary FGN one entered CR and one had PRD. CONCLUSIONS: According to clinical presentation and outcomes, FGN is a very heterogenous glomerular disease. The therapeutic approach in FGN remains challenging. The use of immunosuppressive therapy needs to be assessed in larger studies.

fibrillary glomerulonephritis, outcome, clinical features

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