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Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity

Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the re...

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Autores principales: Cheta, Jordana, Rijhwani, Suresh, Rust, Harlan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5637966/
https://www.ncbi.nlm.nih.gov/pubmed/29051891
http://dx.doi.org/10.1177/2324709617734012
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author Cheta, Jordana
Rijhwani, Suresh
Rust, Harlan
author_facet Cheta, Jordana
Rijhwani, Suresh
Rust, Harlan
author_sort Cheta, Jordana
collection PubMed
description Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity. In this article, we report a rare case of scleroderma renal crisis in a patient with MCTD who we treated with plasma exchange for clinical suspicion for an underlying thrombotic thrombocytopenia and mycophenolate mofetil for MCTD. The patient had multiple poor prognostic indicators yet made a full renal recovery in less than 3 months.
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spelling pubmed-56379662017-10-19 Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity Cheta, Jordana Rijhwani, Suresh Rust, Harlan J Investig Med High Impact Case Rep Case Report Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity. In this article, we report a rare case of scleroderma renal crisis in a patient with MCTD who we treated with plasma exchange for clinical suspicion for an underlying thrombotic thrombocytopenia and mycophenolate mofetil for MCTD. The patient had multiple poor prognostic indicators yet made a full renal recovery in less than 3 months. SAGE Publications 2017-10-10 /pmc/articles/PMC5637966/ /pubmed/29051891 http://dx.doi.org/10.1177/2324709617734012 Text en © 2017 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
Rijhwani, Suresh
Rust, Harlan
Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity
title Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity
title_full Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity
title_fullStr Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity
title_full_unstemmed Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity
title_short Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity
title_sort scleroderma renal crisis in mixed connective tissue disease with full renal recovery within 3 months: a case report with expanding treatment modalities to treat each clinical sign as an independent entity
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5637966/
https://www.ncbi.nlm.nih.gov/pubmed/29051891
http://dx.doi.org/10.1177/2324709617734012
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