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Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure
A 75-year-old male with no prior history of vasculitis or renal deficiency presented with a history of 36 hours of anterior epistaxis, one month of worsening shortness of breath, severe anemia requiring transfusion, thrombocytopenia, coagulopathy with INR 4.9, and renal failure requiring hemodialysi...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4780691/ https://www.ncbi.nlm.nih.gov/pubmed/27004158 http://dx.doi.org/10.7759/cureus.482 |
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author | Chaudhuri, Aadel A Davis, Jason T |
author_facet | Chaudhuri, Aadel A Davis, Jason T |
author_sort | Chaudhuri, Aadel A |
collection | PubMed |
description | A 75-year-old male with no prior history of vasculitis or renal deficiency presented with a history of 36 hours of anterior epistaxis, one month of worsening shortness of breath, severe anemia requiring transfusion, thrombocytopenia, coagulopathy with INR 4.9, and renal failure requiring hemodialysis. A peripheral blood smear revealed no evidence of microangiopathic hemolytic anemia. We performed an autoimmune workup, which revealed high levels of serum serine protease 3 antibody (C-ANCA), elevated rheumatoid factor, low serum C3, and normal levels of serum C4. We performed a renal biopsy and then performed light microscopy, immunofluorescence, and electron microscopy on the resulting samples. This revealed that approximately half of the sampled glomeruli were globally sclerotic, consistent with severe renal disease. Among the non-sclerotic glomeruli, several demonstrated diffuse granular mesangial staining for C3, while other glomeruli had small crescents, consistent with a mixed picture of C3 glomerulonephritis (C3GN) and crescentic glomerulonephritis. The patient responded well to treatment with cyclophosphamide and prednisone, with a resolution of his acute issues, significant improvement in kidney function, and was eventually weaned from routine hemodialysis. In summary, this is a unique case of a patient presenting with features of both granulomatosis with polyangiitis (GPA) and C3GN. |
format | Online Article Text |
id | pubmed-4780691 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-47806912016-03-21 Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure Chaudhuri, Aadel A Davis, Jason T Cureus Rheumatology A 75-year-old male with no prior history of vasculitis or renal deficiency presented with a history of 36 hours of anterior epistaxis, one month of worsening shortness of breath, severe anemia requiring transfusion, thrombocytopenia, coagulopathy with INR 4.9, and renal failure requiring hemodialysis. A peripheral blood smear revealed no evidence of microangiopathic hemolytic anemia. We performed an autoimmune workup, which revealed high levels of serum serine protease 3 antibody (C-ANCA), elevated rheumatoid factor, low serum C3, and normal levels of serum C4. We performed a renal biopsy and then performed light microscopy, immunofluorescence, and electron microscopy on the resulting samples. This revealed that approximately half of the sampled glomeruli were globally sclerotic, consistent with severe renal disease. Among the non-sclerotic glomeruli, several demonstrated diffuse granular mesangial staining for C3, while other glomeruli had small crescents, consistent with a mixed picture of C3 glomerulonephritis (C3GN) and crescentic glomerulonephritis. The patient responded well to treatment with cyclophosphamide and prednisone, with a resolution of his acute issues, significant improvement in kidney function, and was eventually weaned from routine hemodialysis. In summary, this is a unique case of a patient presenting with features of both granulomatosis with polyangiitis (GPA) and C3GN. Cureus 2016-02-05 /pmc/articles/PMC4780691/ /pubmed/27004158 http://dx.doi.org/10.7759/cureus.482 Text en Copyright © 2016, Chaudhuri et al. http://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Rheumatology Chaudhuri, Aadel A Davis, Jason T Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure |
title | Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure |
title_full | Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure |
title_fullStr | Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure |
title_full_unstemmed | Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure |
title_short | Concomitant Granulomatosis with Polyangiitis and C3 Glomerulonephritis Causing Renal Failure |
title_sort | concomitant granulomatosis with polyangiitis and c3 glomerulonephritis causing renal failure |
topic | Rheumatology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4780691/ https://www.ncbi.nlm.nih.gov/pubmed/27004158 http://dx.doi.org/10.7759/cureus.482 |
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