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      Treatment of fibrillary glomerulonephritis with use of repository corticotropin injections

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          ABSTRACT

          Fibrillary glomerulonephritis (FGN) is a rare idiopathic condition linked to malignancy, autoimmune disorders, monoclonal gammopathies and hepatitis C virus. It usually has a poor prognosis, resulting in progression to end-stage renal disease within a few years, given the lack of standardized treatment. Repository corticotrophin (RC) injections are approved for use in a variety of nephrotic syndromes, but are not routinely considered for treatment of FGN. We present a case in which a patient with FGN began treatment with RC 3 months after diagnosis. The patient has attained partial remission with complete resolution of nephrotic syndrome and stabilization of renal function.

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          Fibrillary glomerulonephritis: a report of 66 cases from a single institution.

          Sanjeev Sethi, Samih Nasr, Fernando Fervenza … (2011)
          Fibrillary glomerulonephritis (FGN) is a rare primary glomerular disease. Most previously reported cases were idiopathic. To better define the clinical-pathologic spectrum and prognosis, we report the largest single-center series with the longest follow-up. The characteristics of 66 FGN patients who were seen at Mayo Clinic, Rochester, between 1993 and 2010 are provided. The mean age at diagnosis was 53 years. Ninety-five percent of patients were white, and the female:male ratio was 1.2:1. Underlying malignancy (most commonly carcinoma), dysproteinemia, or autoimmune disease (most commonly Crohn's disease, SLE, Graves' disease, and idiopathic thrombocytopenic purpura), were present in 23, 17, and 15% of patients, respectively. Presentation included proteinuria (100%), nephrotic syndrome (38%), renal insufficiency (66%), hematuria (52%), and hypertension (71%). The most common histologic pattern was mesangial proliferative/sclerosing GN followed by membranoproliferative GN. During an average of 52.3 months of follow-up for 61 patients with available data, 13% had complete or partial remission, 43% had persistent renal dysfunction, and 44% progressed to ESRD. The disease recurred in 36% of 14 patients who received a kidney transplant. Independent predictors of ESRD by multivariate analysis were older age, higher creatinine and proteinuria at biopsy, and higher percentage of global glomerulosclerosis. Underlying malignancy, dysproteinemia, or autoimmune diseases are not uncommon in patients with FGN. Prognosis is poor, although remission may occur in a minority of patients without immunosuppressive therapy. Age, degree of renal impairment at diagnosis, and degree of glomerular scarring are predictors of renal survival. © 2011 by the American Society of Nephrology
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            Morphologic and clinical features of fibrillary glomerulonephritis versus immunotactoid glomerulopathy.

            Marion Horn, A Fogo, Nadia Qureshi (1993)
            Renal diseases characterized by Congo red-negative extracellular fibrillary deposits, either organized arrays of larger, microtubular fibrils (immunotactoid glomerulopathy [IT]) or smaller, randomly organized fibrils (fibrillary glomerulonephritis), have been recognized recently. The clinical significance, if any, of the distinction of these patterns has not been determined. On review of all renal biopsy specimens evaluated in a private referral renal pathology laboratory over the last 11 years, 26 cases with fibrillary glomerulonephritis pattern were identified and compared with our six most recent cases with the IT pattern. The fibrillary glomerulonephritis patients, 17 women and nine men, had an average age of 50 +/- 2 years and contributed 1% of the renal biopsy specimens examined. All patients had marked proteinuria and 16 had microscopic hematuria. Follow-up at 23 +/- 5 months in 25 of these patients revealed end-stage renal disease in 11 patients (44%) and one death due to renal failure. End-stage renal disease developed an average of 10 +/- 5 months after biopsy. One patient developed multiple myeloma. Twenty-four renal biopsy specimens showed proliferation, with crescents in seven. Immunofluorescence showed moderate to intense staining for immunoglobulin G and weaker staining for C3, in a predominantly mesangial pattern, with weaker glomerular basement membrane (GBM) staining, corresponding to electron microscopic deposit localization. In four cases, linear GBM staining by immunofluorescence corresponded to extensive subendothelial or transmembranous deposits. The average fibril diameter was 14.0 +/- 0.5 nm (range, 10.4 to 18.4 nm). Immunotactoid glomerulopathy patients (three women and three men) were significantly older, 62 +/- 2 years (P < 0.025). All had marked proteinuria, with microscopic hematuria in two patients. Associated hematopoietic diseases were present in four patients, with monoclonal proteins and/or abnormal plasma cell proliferation in three. One patient died of nonrenal causes. The remaining five patients have stable renal function at 20 +/- 5 months. Biopsy specimens showed proliferative (n = 3) or membranous-like (n = 3) patterns. Immunofluorescence showed immunoglobulin G and weaker C3 staining in a granular GBM pattern, with lesser mesangial staining. The microtubular fibril diameter was on average 43.2 +/- 10.3 nm (range, 16.8 to 90.0 nm). Thus, fibrillary glomerulonephritis and IT can be separated based on ultrastructurally distinct features. Patients with fibrillary glomerulonephritis are less likely than those with IT to have associated hematopoietic disease and also have poorer renal survival. We propose that classification based on these morphologic differences appears to have clinical significance.
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              Long-term kidney disease outcomes in fibrillary glomerulonephritis: a case series of 27 patients.

              Frank Bridoux, Guy Touchard, Pierre Aucouturier … (2013)
              Fibrillary glomerulonephritis (GN) is a rare disorder with poor renal prognosis. Therapeutic strategies, particularly the use of immunosuppressive drugs, are debated.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Clin Kidney J
                Journal ID (iso-abbrev): Clin Kidney J
                Journal ID (publisher-id): ckj
                Title: Clinical Kidney Journal
                Publisher: Oxford University Press
                ISSN (Print): 2048-8505
                ISSN (Electronic): 2048-8513
                Publication date (Print): December 2018
                Publication date (Electronic): 20 March 2018
                Publication date PMC-release: 20 March 2018
                Volume: 11
                Issue: 6
                Pages: 788-790
                Affiliations
                [1 ]Kettering Medical Center, OH, USA
                [2 ]Renal Physicians Inc
                [3 ]Wright State University, OH, USA
                [4 ]Department of Pathology, The Ohio State University Wexner Medical Center, OH, USA
                Author notes
                Correspondence and offprint requests to: Natallia Maroz; E-mail: natallia.maroz@ 123456wright.edu
                Article
                Publisher ID: sfy009
                DOI: 10.1093/ckj/sfy009
                PMC ID: 6275454
                PubMed ID: 30524713
                SO-VID: a8e99400-23d3-4124-b15a-e003b9ce9518
                Copyright © © The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                Date received : 05 November 2017
                Date accepted : 19 January 2018
                Page count
                Pages: 3
                Categories
                Subject: Rare Diseases

                ScienceOpen disciplines: Nephrology
                Keywords: fibrillary glomerulonephritis,immunotherapy,nephrotic-range proteinuria,partial remission,repository corticotropin
                Data availability:
                ScienceOpen disciplines: Nephrology
                Keywords: fibrillary glomerulonephritis, immunotherapy, nephrotic-range proteinuria, partial remission, repository corticotropin

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