Fibrillary glomerulonephritis in a patient with systemic lupus erythematosus with no evidence of lupus nephritis

被引:0
作者
Whelband, Matthew Carl [1 ]
Willingham, Tom [2 ]
Thirunavukkarasu, Sathiamalar [2 ]
Patrick, Jean [3 ]
机构
[1] James Paget Univ Hosp NHS Fdn Trust, Acute Med, Great Yarmouth, England
[2] Cambridge Univ Hosp NHS Fdn Trust, Dept Histopathol, Cambridge, England
[3] James Paget Univ Hosp NHS Fdn Trust, Nephrol, Great Yarmouth, England
关键词
Biological agents; Pathology; Renal medicine; Systemic lupus erythematosus; CLINICAL-FEATURES;
D O I
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中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Fibrillary glomerulonephritis (FGN) is a rare proliferative form of glomerular disease characterised by randomly oriented fibrillary deposits with a mean diameter of 20 nm. It has a rare association with systemic lupus erythematosus (SLE). We report the case of a female in her mid-50's with a 20 year history of SLE, who developed proteinuria due to FGN and had no histological evidence of lupus nephritis. She was maintained on azathioprine and prednisolone. A renal biopsy revealed randomly arranged fibrillary deposits that positively stained for DNAJB9, consistent with a diagnosis of FGN. Azathioprine was switched to mycophenolate mofetil, and the patient showed significant improvement in proteinuria. This case-based review describes the diagnosis, management and clinical outcome of FGN in association with SLE in the absence of lupus nephritis.
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页数:4
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