Nephrol Dial Transplant (1996) 11: 837-842
© 1996 European Renal Association-European Dialysis and Transplant Association
research-article
Clinical features, predictors of disease progression and results of renal transplantation in fibrillary/immunotactoid glomerulopathy
1Renal Division, Department of Medicine Boston, USA 2Department of Pathology, Brigham & Women's Hospital Boston, USA 3Renal Division, New England Deaconess Hospital/Joslin Diabetes Center, Harvard Medical School Boston, USA 4Department of Medicine, Mater Miseracordiae Hospital, University College Dublin Ireland
Correspondence and offprint requests to: Correspondence and offprint requests to: Hugh R. Brady MD, PhD, FRCPI, Department of Medicine, Mater Miseracordiae Hospital, University College Dublin, 41 Eccles Street, Dublin 7, Ireland
BACKGROUND: The clinical manifestations of fibrillary-immunotactoid glomerulopathy are still being appreciated. It is unclear whether fibrillary-immunotactoid glomerulopathy represents two distinct clinicopathol-ogical entities, fibrillary glomerulopathy (FG) and immunotactoid glomerulopathy (ITG), or a single disease with different ultrastructural variants.
METHODS: To address these issues, we analysed the clinical features of 186 patients with fibrillary-immunotactoid glomerulopathy referred to our institutions (25 patients) or reported in the literature (161 patients). In separate analyses, patients were sub-classified as having either fibrillary glomerulopathy (FG) or immunotactoid glomerulopathy (ITG) according to fibril diameter (FG
30 nm, ITG>30 nm) or arrangement (FG, random; ITG, focally organized).
RESULTS: Proteinuria (FG 
100%, ITG 100%), nephrotic syndrome (FG 71%, ITG 82%), haemat-uria (FG 71%, ITG 64%), hypertension (FG 67%, ITG 45%), and renal insufficiency (FG 54%, ITG 42%) were frequent clinical correlates of both FG and ITG, irrespective of the ultrastructural criteria for diagnosis. Twenty-five patients presenting to our institutions (24 FG, 1 ITG) were divided into three groups based on rate of decline of GFR (mean slope of 1/serum creatinine versus time: group 1 –0.103±0.238; group 2 0.121±0.040; group 3 0.466±0.318) in an attempt to identify clinical predictors of progression at presentation. Rapid progressors (Group 3) had an increased incidence of nephrotic syndrome and tended to have higher blood pressure than patients with milder disease, but did not differ from other groups in age, prevalence of haematuria or degree of renal insufficiency. The number of patients requiring dialysis was 0/10 in group 1, 2/6 in group 2, and 2/4 in group 3 over a follow-up period of 47±46, 55±32, and 19±19 months respectively; two predialysis deaths being recorded in group 3. Four patients received five renal allografts (one patient being transplanted twice) and were followed for 4–11 years. Whereas recurrence of FG was documented in three allografts undergoing post-transplant biopsy, the rate of deterioration of GFR was invariably slower in allografts than native kidneys (mean slope of 1/Cr versus time: 0.036±0.01 versus 0.301±0.18 respectively). The strength of association between FG-ITG and lymphoproliferative malignancy varied depending on whether patients with monoclonal-gammopathy-associated fibrillary deposits were included or excluded from the analysis.
CONCLUSIONS: We contend that patients presenting with Congo-red-negative fibrillary deposits on renal biopsy should be evaluated carefully for monoclonal gammopathy and cryoglobulins, but that there is insufficient published data, as yet, to justify subclassification of FG and ITG as distinct clinicopathological entities. Indeed, we argue that it remains to be determined if FG-ITG represents a unique condition or a forme fruste of cryoglobulin- or monoclonal-gammopathy-associated renal disease. Although the optimal treatment for FG-ITG has not been determined, renal transplantation appears an attractive option in patients with end-stage renal failure.
Keywords: amyloid; cryoglobulinaemia fibrils; fibrillary glomerulonephritis; fibrillary glomerulopathy; glomerulonephritis; immunotactoid glomerulopathy; kidney diseases; lymphoma; leukaemia; multiple myeloma; transplantation
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