Immunoglobulin-Negative DNAJB9-Associated Fibrillary Glomerulonephritis: A Report of 9 Cases
| Autor: | Mary E. Fidler, Jason D. Theis, Christopher P. Larsen, Anthony M. Valeri, Ellen D. McPhail, Virginie Royal, Samar M. Said, Saied Safabakhsh, Julie A. Vrana, Alejandro Best Rocha, Lynn D. Cornell, Chadwick Barnes, Lalitha Bandi, Nelson Leung, Mariam P. Alexander, Samih H. Nasr |
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| Rok vydání: | 2021 |
| Předmět: |
Pathology
medicine.medical_specialty Proteinuria medicine.diagnostic_test biology business.industry Fibrillary Glomerulonephritis 030232 urology & nephrology Mesangial hypercellularity Immunofluorescence Stain Pathogenesis 03 medical and health sciences 0302 clinical medicine Elevated serum creatinine Nephrology medicine biology.protein 030212 general & internal medicine medicine.symptom Antibody business |
| Zdroj: | American Journal of Kidney Diseases. 77:454-458 |
| ISSN: | 0272-6386 |
| DOI: | 10.1053/j.ajkd.2020.04.015 |
| Popis: | Fibrillary glomerulonephritis (FGN) was previously defined by glomerular deposition of haphazardly oriented fibrils that stain with antisera to immunoglobulins but do not stain with Congo red. We report what is to our knowledge the first series of immunoglobulin-negative FGN, consisting of 9 adults (7 women and 2 men) with a mean age at diagnosis of 66 years. Patients presented with proteinuria (100%; mean protein excretion, 3g/d), hematuria (100%), and elevated serum creatinine level (100%). Comorbid conditions included carcinoma in 3 and hepatitis C virus infection in 2; no patient had hypocomplementemia or monoclonal gammopathy. Histologically, glomeruli were positive for DNAJB9, showed mostly mild mesangial hypercellularity and/or sclerosis, and were negative for immunoglobulins by immunofluorescence on frozen and paraffin tissue. Ultrastructurally, randomly oriented fibrils measuring 13 to 20nm in diameter were seen intermingling with mesangial matrix in all and infiltrating glomerular basement membranes in 5. On follow-up (mean duration, 21 months), 2 had disease remission, 4 had persistently elevated serum creatinine levels and proteinuria, and 3 required kidney replacement therapy. Thus, rare cases of FGN are not associated with glomerular immunoglobulin deposition, and the diagnosis of FGN in these cases can be confirmed by DNAJB9 immunostaining. Pathogenesis remains to be elucidated. |
| Databáze: | OpenAIRE |
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