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      Collapsing Focal Segmental Glomerulosclerosis in a Patient with Systemic Lupus Erythematosus

      case-report
      Author(s): * , ,
      Publication date (Electronic):
      Journal: Case Reports in Medicine
      Publisher: Hindawi Publishing Corporation

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          Abstract

          We present a case of a 36-year-old female from Ghana who presented with atypical chest pain and shortness of breath and was found to have bilateral transudative pleural effusion and trivial pericardial effusion. Further work-up revealed serological markers consistent with active lupus and negative HIV. She developed rapid deterioration of her renal function requiring dialysis. Her renal biopsy showed collapsing focal segmental glomerulosclerosis with diffuse mesangial proliferative glomerulonephritis, consistent with lupus nephritis class II along with tubular degenerative changes. She was started on high dose steroids and later on mycophenolate mofetil. Her renal function slowly recovered to baseline.

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          Apolipoprotein L1 risk variants associate with systemic lupus erythematosus-associated collapsing glomerulopathy.

          Q Walker, Marjorie Beggs, Christian P. Larsen … (2013)
          Collapsing glomerulopathy is a devastating renal disease that primarily affects African Americans and associates with numerous etiologies, such as HIV and autoimmune disease. The presence of APOL1 risk alleles associates with HIV-associated collapsing glomerulopathy, but it is unknown whether these risk alleles also associate with systemic lupus erythematosus (SLE) -associated collapsing glomerulopathy. Here, re-examination of 546 renal biopsies from African-American patients with SLE identified 26 cases of collapsing glomerulopathy, which we genotyped for APOL1 risk alleles using DNA extracted from archived biopsy tissue. APOL1 strongly associated with SLE-associated collapsing glomerulopathy (P<0.001). In a recessive model, two APOL1 risk alleles conferred 5.4-fold (95% CI=2.4 to 12.1) higher odds of developing SLE-associated collapsing glomerulopathy (P<0.001). In conclusion, APOL1 genotyping of African-American patients with SLE might help identify patients at risk for collapsing glomerulopathy, an entity with a poor prognosis that is often resistant to treatment.
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            A proposed taxonomy for the podocytopathies: a reassessment of the primary nephrotic diseases.

            Laura Barisoni, H. William Schnaper, Jeffrey B Kopp (2007)
            A spectrum of proteinuric glomerular diseases results from podocyte abnormalities. The understanding of these podocytopathies has greatly expanded in recent years, particularly with the discovery of more than a dozen genetic mutations that are associated with loss of podocyte functional integrity. It is apparent that classification of the podocytopathies on the basis of morphology alone is inadequate to capture fully the complexity of these disorders. Herein is proposed a taxonomy for the podocytopathies that classifies along two dimensions: Histopathology, including podocyte phenotype and glomerular morphology (minimal-change nephropathy, focal segmental glomerulosclerosis, diffuse mesangial sclerosis, and collapsing glomerulopathy), and etiology (idiopathic, genetic, and reactive forms). A more complete understanding of the similarities and differences among podocyte diseases will help the renal pathologist and the nephrologist communicate more effectively about the diagnosis; this in turn will help the nephrologist provide more accurate prognostic information and select the optimal therapy for these often problematic diseases. It is proposed that final diagnosis of the podocytopathies should result from close collaboration between renal pathologists and nephrologists and should whenever possible include three elements: Morphologic entity, etiologic form, and specific pathogenic mechanism or association.
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              Focal segmental glomerulosclerosis in nephrotic adults: presentation, prognosis, and response to therapy of the histologic variants.

              Rebecca Schwartz, Stephen M. Korbet, Seung Chun … (2004)
              The histopathologic diagnosis of primary focal segmental glomerulosclerosis (FSGS) has come to include a number of histologic lesions (variants), but the prognostic significance of these discrete lesions is controversial because published information regarding the presentation, course, and response to treatment is limited. A retrospective analysis was conducted of 87 nephrotic adult patients with biopsy-proven primary FSGS. Patients were categorized on the basis of histologic criteria into those with a classic scar (36 patients), the cellular or collapsing lesion (40 patients), or the tip lesion (11 patients) of FSGS to evaluate differences in presentation, response to therapy, and clinical outcomes. The clinical features at biopsy were similar among the three groups with the exception that patients with the tip lesion were older and patients with the collapsing lesion had more severe proteinuria. Over the course of follow-up, 63% of patients treated attained remission and the response to steroid therapy was similar among the groups (classic scar 53% versus collapsing lesion 64% versus tip lesion 78%; P = 0.45). The overall renal survival was significantly better for patients who entered remission compared with patients who did not enter remission (92% versus 33% at 10 yr; P < 0.0001). The renal survival at 10 yr for patients who entered remission was similar among the three groups (classic scar 100% versus tip lesion 100% versus collapsing lesion 80%; P = 0.61). In patients who did not enter remission, the renal survival at 10 yr was significantly worse for patients with collapsing lesion and tip lesion (classic scar 49% versus tip lesion 25% versus collapsing lesion 21%; P = 0.002). In conclusion, the prognosis for nephrotic FSGS patients who enter remission is excellent regardless of the histologic lesion. Because the remission rate after treatment is similar among patients with the histologic variants, response to therapy cannot be predicted on the basis of histology alone. Thus, nephrotic patients with primary FSGS should receive a trial of therapy irrespective of the histologic lesion when not contraindicated.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Case Rep Med
                Journal ID (iso-abbrev): Case Rep Med
                Journal ID (publisher-id): CRIM
                Title: Case Reports in Medicine
                Publisher: Hindawi Publishing Corporation
                ISSN (Print): 1687-9627
                ISSN (Electronic): 1687-9635
                Publication date (Print): 2014
                Publication date (Electronic): 11 August 2014
                Volume: 2014
                Electronic Location Identifier: 732192
                Affiliations
                Bronx Lebanon Hospital Center, Department of Medicine, 1650 Selwyn Avenue, Suit 10C, Bronx, NY 10457, USA
                Author notes

                Academic Editor: W. Zidek

                Author information
                http://orcid.org/0000-0002-9178-0342
                http://orcid.org/0000-0001-8010-4317
                Article
                DOI: 10.1155/2014/732192
                PMC ID: 4144085
                PubMed ID: 25180039
                SO-VID: 1ce98c06-231d-4696-b423-15a899549d72
                Copyright © Copyright © 2014 Hassan Tariq et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 31 May 2014
                Date revision received : 15 July 2014
                Date accepted : 29 July 2014
                Categories
                Subject: Case Report

                Data availability:

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