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ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma

Systemic sclerosis (SSc) is a rare autoimmune disorder that is typically divided into limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis. Scleroderma renal crisis (SRC) is a severe complication of SSc and typically presents with new-onset hypertension and a reduction in re...

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Autores principales: Cheta, Jordana, Binder, Michael, Kowalewska, Jolanta, Magoon, Sandeep
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6062775/
https://www.ncbi.nlm.nih.gov/pubmed/30083557
http://dx.doi.org/10.1177/2324709618785188
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author Cheta, Jordana
Binder, Michael
Kowalewska, Jolanta
Magoon, Sandeep
author_facet Cheta, Jordana
Binder, Michael
Kowalewska, Jolanta
Magoon, Sandeep
author_sort Cheta, Jordana
collection PubMed
description Systemic sclerosis (SSc) is a rare autoimmune disorder that is typically divided into limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis. Scleroderma renal crisis (SRC) is a severe complication of SSc and typically presents with new-onset hypertension and a reduction in renal functioning. In patients presenting with typical features of SRC, treatment with an angiotensin-converting enzyme inhibitor along with dialysis as needed is typically initiated empirically. Renal biopsy is not recommended in patients with SSc presenting with typical features of SRC. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare co-occurrence with SSc, in around 2.5% to 9% of patients. AAV is an inflammatory condition that can result in renal failure due to mononuclear cell infiltration and destruction of blood vessels. Treatment of AAV is drastically different from SRC and typically consists of immunosuppressants and dialysis if needed. SRC and AAV can only reliably be distinguished by renal biopsy. We present a rare case of a 70-year-old female with limited cutaneous systemic sclerosis who presented to the emergency department with new-onset renal failure. Her serology was found to be positive for antinuclear antibodies and myeloperoxidase antibodies, resulting in a renal biopsy, which revealed an acute necrotizing vasculitis consistent with AAV. We suggest consideration of a renal biopsy in patients with SSc who present with new-onset renal failure, especially with nonresponse to SRC treatment or positive serology.
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spelling pubmed-60627752018-08-06 ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma Cheta, Jordana Binder, Michael Kowalewska, Jolanta Magoon, Sandeep J Investig Med High Impact Case Rep Case Report Systemic sclerosis (SSc) is a rare autoimmune disorder that is typically divided into limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis. Scleroderma renal crisis (SRC) is a severe complication of SSc and typically presents with new-onset hypertension and a reduction in renal functioning. In patients presenting with typical features of SRC, treatment with an angiotensin-converting enzyme inhibitor along with dialysis as needed is typically initiated empirically. Renal biopsy is not recommended in patients with SSc presenting with typical features of SRC. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare co-occurrence with SSc, in around 2.5% to 9% of patients. AAV is an inflammatory condition that can result in renal failure due to mononuclear cell infiltration and destruction of blood vessels. Treatment of AAV is drastically different from SRC and typically consists of immunosuppressants and dialysis if needed. SRC and AAV can only reliably be distinguished by renal biopsy. We present a rare case of a 70-year-old female with limited cutaneous systemic sclerosis who presented to the emergency department with new-onset renal failure. Her serology was found to be positive for antinuclear antibodies and myeloperoxidase antibodies, resulting in a renal biopsy, which revealed an acute necrotizing vasculitis consistent with AAV. We suggest consideration of a renal biopsy in patients with SSc who present with new-onset renal failure, especially with nonresponse to SRC treatment or positive serology. SAGE Publications 2018-06-28 /pmc/articles/PMC6062775/ /pubmed/30083557 http://dx.doi.org/10.1177/2324709618785188 Text en © 2018 American Federation for Medical Research http://creativecommons.org/licenses/by/4.0/ This article is distributed under the terms of the Creative Commons Attribution 4.0 License (http://www.creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Cheta, Jordana
Binder, Michael
Kowalewska, Jolanta
Magoon, Sandeep
ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma
title ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma
title_full ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma
title_fullStr ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma
title_full_unstemmed ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma
title_short ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma
title_sort anca-associated vasculitis co-occurrence with systemic sclerosis: a case report of a rare diagnostic dilemma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6062775/
https://www.ncbi.nlm.nih.gov/pubmed/30083557
http://dx.doi.org/10.1177/2324709618785188
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