What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report

Abstract Introduction Collapsing glomerulopathy (CG) is a rare form of glomerular injury. Although commonly associated with human immunodeficiency virus (HIV) infection, it can occur in association with systemic lupus erythematosus (SLE). Case Report We present the case of a 50-year-old man, with ch...

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Veröffentlicht in:Transplantation proceedings 2017-05, Vol.49 (4), p.913-915
Hauptverfasser: Pinto, H, Leal, R, Rodrigues, L, Santos, L, Romãozinho, C, Macário, F, Alves, R, Pratas, J, Sousa, V, Marinho, C, Prado e Castro, L, Costa, F, Campos, M, Mota, A, Figueiredo, A
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container_issue 4
container_start_page 913
container_title Transplantation proceedings
container_volume 49
creator Pinto, H
Leal, R
Rodrigues, L
Santos, L
Romãozinho, C
Macário, F
Alves, R
Pratas, J
Sousa, V
Marinho, C
Prado e Castro, L
Costa, F
Campos, M
Mota, A
Figueiredo, A
description Abstract Introduction Collapsing glomerulopathy (CG) is a rare form of glomerular injury. Although commonly associated with human immunodeficiency virus (HIV) infection, it can occur in association with systemic lupus erythematosus (SLE). Case Report We present the case of a 50-year-old man, with chronic kidney disease secondary to focal and segmental glomerulosclerosis, who received a cadaveric kidney transplant in 2007. There were no relevant intercurrences until May 2015, when he presented with nephrotic range proteinuria (± 4 g/d). A graft biopsy was performed and it did not show any significant pathological changes. In September, he developed a full nephrotic syndrome (proteinuria 19 g/d) and a graft biopsy was repeated. CG features were evident with a rich immunofluorescence. Antinuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) antibodies were positive; the remaining immunologic study was normal. Viral markers for HIV, hepatitis C virus (HCV), and hepatitis B virus (HBV) were negative. The patient was treated with corticosteroid pulses and plasmapheresis (seven treatments). A rapid deterioration of kidney function was seen and he became dialysis dependent. He was discharged with a low-dose immunosuppressive treatment. In October, he was hospitalized with diffuse alveolar hemorrhage (DAH). The auto-immune study was repeated, revealing complement consumption and positive titers of ANA and Anti-dsDNA antibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) and antiglomerular basement membrane antibody (anti-GBM) were negative. Treatment with intravenous corticosteroids, plasmapheresis, and human immunoglobulin was ineffective and the outcome was fatal. Conclusion This case report highlights the possible association of CG and SLE. To our knowledge, it is the first case of SLE presenting with CG and DAH, with the singularity of occurring in a kidney transplant recipient receiving immunosuppression.
doi_str_mv 10.1016/j.transproceed.2017.03.007
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Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report</title><source>MEDLINE</source><source>Elsevier ScienceDirect Journals Complete</source><creator>Pinto, H ; Leal, R ; Rodrigues, L ; Santos, L ; Romãozinho, C ; Macário, F ; Alves, R ; Pratas, J ; Sousa, V ; Marinho, C ; Prado e Castro, L ; Costa, F ; Campos, M ; Mota, A ; Figueiredo, A</creator><creatorcontrib>Pinto, H ; Leal, R ; Rodrigues, L ; Santos, L ; Romãozinho, C ; Macário, F ; Alves, R ; Pratas, J ; Sousa, V ; Marinho, C ; Prado e Castro, L ; Costa, F ; Campos, M ; Mota, A ; Figueiredo, A</creatorcontrib><description>Abstract Introduction Collapsing glomerulopathy (CG) is a rare form of glomerular injury. Although commonly associated with human immunodeficiency virus (HIV) infection, it can occur in association with systemic lupus erythematosus (SLE). Case Report We present the case of a 50-year-old man, with chronic kidney disease secondary to focal and segmental glomerulosclerosis, who received a cadaveric kidney transplant in 2007. There were no relevant intercurrences until May 2015, when he presented with nephrotic range proteinuria (± 4 g/d). A graft biopsy was performed and it did not show any significant pathological changes. In September, he developed a full nephrotic syndrome (proteinuria 19 g/d) and a graft biopsy was repeated. CG features were evident with a rich immunofluorescence. Antinuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) antibodies were positive; the remaining immunologic study was normal. Viral markers for HIV, hepatitis C virus (HCV), and hepatitis B virus (HBV) were negative. The patient was treated with corticosteroid pulses and plasmapheresis (seven treatments). A rapid deterioration of kidney function was seen and he became dialysis dependent. He was discharged with a low-dose immunosuppressive treatment. In October, he was hospitalized with diffuse alveolar hemorrhage (DAH). The auto-immune study was repeated, revealing complement consumption and positive titers of ANA and Anti-dsDNA antibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) and antiglomerular basement membrane antibody (anti-GBM) were negative. Treatment with intravenous corticosteroids, plasmapheresis, and human immunoglobulin was ineffective and the outcome was fatal. Conclusion This case report highlights the possible association of CG and SLE. To our knowledge, it is the first case of SLE presenting with CG and DAH, with the singularity of occurring in a kidney transplant recipient receiving immunosuppression.</description><identifier>ISSN: 0041-1345</identifier><identifier>EISSN: 1873-2623</identifier><identifier>DOI: 10.1016/j.transproceed.2017.03.007</identifier><identifier>PMID: 28457424</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Antibodies, Antinuclear - blood ; Biopsy ; Fatal Outcome ; Glomerulosclerosis, Focal Segmental - complications ; Glomerulosclerosis, Focal Segmental - immunology ; Glomerulosclerosis, Focal Segmental - surgery ; Hemorrhage - immunology ; Hemorrhage - pathology ; Humans ; Immunosuppressive Agents - therapeutic use ; Kidney Glomerulus - immunology ; Kidney Transplantation - adverse effects ; Lupus Erythematosus, Systemic - complications ; Lupus Erythematosus, Systemic - immunology ; Lupus Nephritis - immunology ; Lupus Nephritis - pathology ; Male ; Middle Aged ; Nephrotic Syndrome - blood ; Nephrotic Syndrome - etiology ; Nephrotic Syndrome - immunology ; Nephrotic Syndrome - pathology ; Postoperative Complications - immunology ; Postoperative Complications - pathology ; Proteinuria - immunology ; Proteinuria - pathology ; Pulmonary Alveoli ; Surgery</subject><ispartof>Transplantation proceedings, 2017-05, Vol.49 (4), p.913-915</ispartof><rights>Elsevier Inc.</rights><rights>2017 Elsevier Inc.</rights><rights>Copyright © 2017 Elsevier Inc. 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Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report</title><title>Transplantation proceedings</title><addtitle>Transplant Proc</addtitle><description>Abstract Introduction Collapsing glomerulopathy (CG) is a rare form of glomerular injury. Although commonly associated with human immunodeficiency virus (HIV) infection, it can occur in association with systemic lupus erythematosus (SLE). Case Report We present the case of a 50-year-old man, with chronic kidney disease secondary to focal and segmental glomerulosclerosis, who received a cadaveric kidney transplant in 2007. There were no relevant intercurrences until May 2015, when he presented with nephrotic range proteinuria (± 4 g/d). A graft biopsy was performed and it did not show any significant pathological changes. In September, he developed a full nephrotic syndrome (proteinuria 19 g/d) and a graft biopsy was repeated. CG features were evident with a rich immunofluorescence. Antinuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) antibodies were positive; the remaining immunologic study was normal. Viral markers for HIV, hepatitis C virus (HCV), and hepatitis B virus (HBV) were negative. The patient was treated with corticosteroid pulses and plasmapheresis (seven treatments). A rapid deterioration of kidney function was seen and he became dialysis dependent. He was discharged with a low-dose immunosuppressive treatment. In October, he was hospitalized with diffuse alveolar hemorrhage (DAH). The auto-immune study was repeated, revealing complement consumption and positive titers of ANA and Anti-dsDNA antibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) and antiglomerular basement membrane antibody (anti-GBM) were negative. Treatment with intravenous corticosteroids, plasmapheresis, and human immunoglobulin was ineffective and the outcome was fatal. Conclusion This case report highlights the possible association of CG and SLE. 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Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report</title><author>Pinto, H ; Leal, R ; Rodrigues, L ; Santos, L ; Romãozinho, C ; Macário, F ; Alves, R ; Pratas, J ; Sousa, V ; Marinho, C ; Prado e Castro, L ; Costa, F ; Campos, M ; Mota, A ; Figueiredo, A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c514t-66c880606a66e5abe74e9f34e2a3eeaabf0770d368d71df10b9038e2c61314173</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2017</creationdate><topic>Antibodies, Antinuclear - blood</topic><topic>Biopsy</topic><topic>Fatal Outcome</topic><topic>Glomerulosclerosis, Focal Segmental - complications</topic><topic>Glomerulosclerosis, Focal Segmental - immunology</topic><topic>Glomerulosclerosis, Focal Segmental - surgery</topic><topic>Hemorrhage - immunology</topic><topic>Hemorrhage - pathology</topic><topic>Humans</topic><topic>Immunosuppressive Agents - therapeutic use</topic><topic>Kidney Glomerulus - immunology</topic><topic>Kidney Transplantation - adverse effects</topic><topic>Lupus Erythematosus, Systemic - complications</topic><topic>Lupus Erythematosus, Systemic - immunology</topic><topic>Lupus Nephritis - immunology</topic><topic>Lupus Nephritis - pathology</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Nephrotic Syndrome - blood</topic><topic>Nephrotic Syndrome - etiology</topic><topic>Nephrotic Syndrome - immunology</topic><topic>Nephrotic Syndrome - pathology</topic><topic>Postoperative Complications - immunology</topic><topic>Postoperative Complications - pathology</topic><topic>Proteinuria - immunology</topic><topic>Proteinuria - pathology</topic><topic>Pulmonary Alveoli</topic><topic>Surgery</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Pinto, H</creatorcontrib><creatorcontrib>Leal, R</creatorcontrib><creatorcontrib>Rodrigues, L</creatorcontrib><creatorcontrib>Santos, L</creatorcontrib><creatorcontrib>Romãozinho, C</creatorcontrib><creatorcontrib>Macário, F</creatorcontrib><creatorcontrib>Alves, R</creatorcontrib><creatorcontrib>Pratas, J</creatorcontrib><creatorcontrib>Sousa, V</creatorcontrib><creatorcontrib>Marinho, C</creatorcontrib><creatorcontrib>Prado e Castro, L</creatorcontrib><creatorcontrib>Costa, F</creatorcontrib><creatorcontrib>Campos, M</creatorcontrib><creatorcontrib>Mota, A</creatorcontrib><creatorcontrib>Figueiredo, A</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Transplantation proceedings</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Pinto, H</au><au>Leal, R</au><au>Rodrigues, L</au><au>Santos, L</au><au>Romãozinho, C</au><au>Macário, F</au><au>Alves, R</au><au>Pratas, J</au><au>Sousa, V</au><au>Marinho, C</au><au>Prado e Castro, L</au><au>Costa, F</au><au>Campos, M</au><au>Mota, A</au><au>Figueiredo, A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report</atitle><jtitle>Transplantation proceedings</jtitle><addtitle>Transplant Proc</addtitle><date>2017-05-01</date><risdate>2017</risdate><volume>49</volume><issue>4</issue><spage>913</spage><epage>915</epage><pages>913-915</pages><issn>0041-1345</issn><eissn>1873-2623</eissn><abstract>Abstract Introduction Collapsing glomerulopathy (CG) is a rare form of glomerular injury. Although commonly associated with human immunodeficiency virus (HIV) infection, it can occur in association with systemic lupus erythematosus (SLE). Case Report We present the case of a 50-year-old man, with chronic kidney disease secondary to focal and segmental glomerulosclerosis, who received a cadaveric kidney transplant in 2007. There were no relevant intercurrences until May 2015, when he presented with nephrotic range proteinuria (± 4 g/d). A graft biopsy was performed and it did not show any significant pathological changes. In September, he developed a full nephrotic syndrome (proteinuria 19 g/d) and a graft biopsy was repeated. CG features were evident with a rich immunofluorescence. Antinuclear antibodies (ANA) and anti-double-stranded DNA (anti-dsDNA) antibodies were positive; the remaining immunologic study was normal. Viral markers for HIV, hepatitis C virus (HCV), and hepatitis B virus (HBV) were negative. The patient was treated with corticosteroid pulses and plasmapheresis (seven treatments). A rapid deterioration of kidney function was seen and he became dialysis dependent. He was discharged with a low-dose immunosuppressive treatment. In October, he was hospitalized with diffuse alveolar hemorrhage (DAH). The auto-immune study was repeated, revealing complement consumption and positive titers of ANA and Anti-dsDNA antibodies. Anti-neutrophil cytoplasmic antibodies (ANCAs) and antiglomerular basement membrane antibody (anti-GBM) were negative. Treatment with intravenous corticosteroids, plasmapheresis, and human immunoglobulin was ineffective and the outcome was fatal. Conclusion This case report highlights the possible association of CG and SLE. To our knowledge, it is the first case of SLE presenting with CG and DAH, with the singularity of occurring in a kidney transplant recipient receiving immunosuppression.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>28457424</pmid><doi>10.1016/j.transproceed.2017.03.007</doi><tpages>3</tpages><oa>free_for_read</oa></addata></record>
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subjects Antibodies, Antinuclear - blood
Biopsy
Fatal Outcome
Glomerulosclerosis, Focal Segmental - complications
Glomerulosclerosis, Focal Segmental - immunology
Glomerulosclerosis, Focal Segmental - surgery
Hemorrhage - immunology
Hemorrhage - pathology
Humans
Immunosuppressive Agents - therapeutic use
Kidney Glomerulus - immunology
Kidney Transplantation - adverse effects
Lupus Erythematosus, Systemic - complications
Lupus Erythematosus, Systemic - immunology
Lupus Nephritis - immunology
Lupus Nephritis - pathology
Male
Middle Aged
Nephrotic Syndrome - blood
Nephrotic Syndrome - etiology
Nephrotic Syndrome - immunology
Nephrotic Syndrome - pathology
Postoperative Complications - immunology
Postoperative Complications - pathology
Proteinuria - immunology
Proteinuria - pathology
Pulmonary Alveoli
Surgery
title What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report
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