title: Fibrillary glomerulonephritis associated with Behçet's syndrome.
abstract: Fibrillary glomerulonephritis (FGN) is a rare cause of progressive renal dysfunction resulting in fibrillary deposits in the mesangium and/or glomerular basement membrane (GBM). Some case reports have shown FGN in patients with rheumatoid arthritis and other autoimmune diseases. This is the first case report of FGN in a patient with Behçet's syndrome. The most common renal histological finding in Behçet's syndrome is secondary amyloidosis. A 46-year-old woman with a 4-year history of Behçet's syndrome was referred to the nephrology clinic with foamy urine with non-selective proteinuria (urine protein-to-creatinine ratio was 1400 mg protein/g creatinine) and microscopic hematuria. Serum and urine protein electrophoresis showed no evidence of monoclonal gammopathy. A renal biopsy was performed. Light microscopy showed mesangial widening and nodular expansion with hyaline deposits. Immunofluorescence microscopy revealed immunoglobulin M deposits in the mesangium. Congo red staining was negative. Electron microscopy showed fibrillary deposits on the GBM. Pathological findings were consistent with FGN. She had been taking 50 mg azathioprine and 3000 mg mesalazine per day for 4 years due to Behçet's syndrome, so we did not add any other immunosuppressive agents or corticosteroids. Treatment of this glomerulopathy is not promising. It has been noted that none of the various approaches, including corticosteroid, plasmapheresis, and cytotoxic therapy, improves prognosis.
authors.affiliation: Department of Nephrology, College of Medicine, Pusan Paik Hospital, Inje University, Busan, Republic of Korea.
authors.forename: Hyun Ju
authors.initials: HJ
authors.lastname: Kim
authors.forename: Sun Woo
authors.initials: SW
authors.lastname: Kang
authors.forename: Seok Ju
authors.initials: SJ
authors.lastname: Park
authors.forename: Tae Hee
authors.initials: TH
authors.lastname: Kim
authors.forename: Mi Seon
authors.initials: MS
authors.lastname: Kang
authors.forename: Yeong Hoon
authors.initials: YH
authors.lastname: Kim
mesh_terms: D001528:Behcet Syndrome / Q000150:complications*; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D050533:Glomerular Basement Membrane / Q000648:ultrastructure; D005921:Glomerulonephritis / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008854:Microscopy, Electron; D008875:Middle Aged