A description of clinical course of 4 patients diagnosed and treated in our department with fibrillary GN is given. Fibrillary GN and immunotactoid glomerulopathy are entities, characterized by fibrillar and microtubular deposits in mesangium and the glomerular capillary loops. At our nephrologic division 4 cases of patients with fibrillary GN were diagnozed by the end of the year 2001. Renal biopsy was indicated for proteinuria, hematuria and decrease of renal function. Histologic features observed in light microscopy and immunofluorescency were non–diagnostic; fibrillary character of deposits compatible with the diagnosis of fibrillary GN was proven by electron microscopy. During several months follow–up proteinuria and/or renal insufficiency progressed, despite immunosuppressive treatment. On conclusion: though fibrillary GN/ immunotactoid GN are relatively rare disorders, they represent entities, which should not be omitted in the differential diagnosis of nephrotic syndrome/renal insufficiency, while prognosis remain rather unfavourable and optimal therapy is controversial.

1st Internal Dept 1st Medical School Charles University Prague