What Can We Do When All Collapses? Fatal Outcome of Collapsing Glomerulopathy and Systemic Lupus Erythematosus With Diffuse Alveolar Hemorrhage: Case Report
| Autor: | Luís Rodrigues, Amintas F. S. Figueiredo, Rui Alves, Catarina Romãozinho, L. Prado e Castro, Helena Pinto, Alfredo Mota, Jorge Pratas, Mário Campos, Fátima Costa, Lídia Santos, Vitor Sousa, Carol Marinho, Fernando Macário, Rita Leal |
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| Rok vydání: | 2017 |
| Předmět: |
Male
Pathology medicine.medical_specialty Nephrotic Syndrome Anti-nuclear antibody medicine.medical_treatment Biopsy Kidney Glomerulus Hemorrhage Fatal Outcome Postoperative Complications Hemorragia medicine Humans Lupus Erythematosus Systemic skin and connective tissue diseases Dialysis Transplantation Lupus erythematosus business.industry Glomerulosclerosis Focal Segmental Lupus Eritematoso Sistémico Glomerulosclerosis Nefrite Lúpica Immunosuppression Middle Aged medicine.disease Kidney Transplantation Lupus Nephritis Pulmonary Alveoli Proteinuria Antibodies Antinuclear Surgery Plasmapheresis business Nephrotic syndrome Complicações Pós-operatórias Immunosuppressive Agents Kidney disease |
| Zdroj: | Transplantation proceedings. 49(4) |
| ISSN: | 1873-2623 |
| Popis: | NTRODUCTION: 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. info:eu-repo/semantics/publishedVersion |
| Databáze: | OpenAIRE |
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