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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 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.

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  • Authors+Show Affiliations

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal. Electronic address: helenasofiapinto@gmail.com.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Pathology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Pathology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Pathology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

    ,

    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Nephrology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

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    Urology and Renal Transplantation Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

    Urology and Renal Transplantation Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

    Source

    Transplantation proceedings 49:4 2017 May pg 913-915

    MeSH

    Antibodies, Antinuclear
    Biopsy
    Fatal Outcome
    Glomerulosclerosis, Focal Segmental
    Hemorrhage
    Humans
    Immunosuppressive Agents
    Kidney Glomerulus
    Kidney Transplantation
    Lupus Erythematosus, Systemic
    Lupus Nephritis
    Male
    Middle Aged
    Nephrotic Syndrome
    Postoperative Complications
    Proteinuria
    Pulmonary Alveoli

    Pub Type(s)

    Case Reports
    Journal Article

    Language

    eng

    PubMed ID

    28457424

    Citation

    Pinto, H, et al. "What Can We Do when All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report." Transplantation Proceedings, vol. 49, no. 4, 2017, pp. 913-915.
    Pinto H, Leal R, Rodrigues L, et al. What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report. Transplant Proc. 2017;49(4):913-915.
    Pinto, H., Leal, R., Rodrigues, L., Santos, L., Romãozinho, C., Macário, F., ... Figueiredo, A. (2017). What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report. Transplantation Proceedings, 49(4), pp. 913-915. doi:10.1016/j.transproceed.2017.03.007.
    Pinto H, et al. What Can We Do when All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report. Transplant Proc. 2017;49(4):913-915. PubMed PMID: 28457424.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report. AU - Pinto,H, AU - Leal,R, AU - Rodrigues,L, AU - Santos,L, AU - Romãozinho,C, AU - Macário,F, AU - Alves,R, AU - Pratas,J, AU - Sousa,V, AU - Marinho,C, AU - Prado E Castro,L, AU - Costa,F, AU - Campos,M, AU - Mota,A, AU - Figueiredo,A, PY - 2017/5/2/entrez PY - 2017/5/2/pubmed PY - 2018/1/3/medline SP - 913 EP - 915 JF - Transplantation proceedings JO - Transplant. Proc. VL - 49 IS - 4 N2 - 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. SN - 1873-2623 UR - https://www.unboundmedicine.com/medline/citation/28457424/What_Can_We_Do_When_All_Collapses_Fatal_Outcome_of_Collapsing_Glomerulopathy_and_Systemic_Lupus_Erythematosus_With_Diffuse_Alveolar_Hemorrhage:_Case_Report_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0041-1345(17)30184-7 DB - PRIME DP - Unbound Medicine ER -