A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma
Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations wi...
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| Format: | Article |
| Language: | English |
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Wiley
2016-01-01
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| Series: | Case Reports in Rheumatology |
| Online Access: | http://dx.doi.org/10.1155/2016/8536341 |
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| author | Cody M. Lee Diana Girnita Arundhati Sharma Surabhi Khanna Jean M. Elwing |
| author_facet | Cody M. Lee Diana Girnita Arundhati Sharma Surabhi Khanna Jean M. Elwing |
| author_sort | Cody M. Lee |
| collection | DOAJ |
| description | Systemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia. However, anti-RNAP III has not been associated with systemic sclerosis sine scleroderma. We describe a patient with an atypical presentation of anti-RNAP III positive systemic sclerosis sine scleroderma who presented without the typical features of anti-RNAP III disease. Instead, she presented with critical digital ischemia, pulmonary arterial hypertension, gastroesophageal reflux disease, interstitial lung disease, and no clinically detectable sclerodactyly. |
| format | Article |
| id | doaj-art-b5382d38b6084d3aa960e82cfeefca70 |
| institution | Kabale University |
| issn | 2090-6889 2090-6897 |
| language | English |
| publishDate | 2016-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Rheumatology |
| spelling | doaj-art-b5382d38b6084d3aa960e82cfeefca702025-08-20T03:39:28ZengWileyCase Reports in Rheumatology2090-68892090-68972016-01-01201610.1155/2016/85363418536341A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine SclerodermaCody M. Lee0Diana Girnita1Arundhati Sharma2Surabhi Khanna3Jean M. Elwing4Department of Internal Medicine, University of Cincinnati, Cincinnati, OH 45267, USADivision of Immunology, Allergy and Rheumatology, University of Cincinnati, Cincinnati, OH 45267, USADepartment of Internal Medicine, The Christ Hospital, Cincinnati, OH 45219, USADivision of Immunology, Allergy and Rheumatology, University of Cincinnati, Cincinnati, OH 45267, USADivision of Pulmonary, Critical Care, and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, OH 45267, USASystemic sclerosis is a rare autoimmune disorder with a wide spectrum of clinical manifestations and a multitude of autoantibodies that are associated with it. In the past several years, advances in serologic testing have led to research indicating important prognostic and phenotypic associations with certain subsets of autoantibodies. In particular, anti-RNA polymerase III (anti-RNAP III) has been associated with diffuse cutaneous disease, scleroderma renal crisis, a temporal relationship with malignancy, myositis, synovitis, joint contractures, and gastric antral vascular ectasia. However, anti-RNAP III has not been associated with systemic sclerosis sine scleroderma. We describe a patient with an atypical presentation of anti-RNAP III positive systemic sclerosis sine scleroderma who presented without the typical features of anti-RNAP III disease. Instead, she presented with critical digital ischemia, pulmonary arterial hypertension, gastroesophageal reflux disease, interstitial lung disease, and no clinically detectable sclerodactyly.http://dx.doi.org/10.1155/2016/8536341 |
| spellingShingle | Cody M. Lee Diana Girnita Arundhati Sharma Surabhi Khanna Jean M. Elwing A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma Case Reports in Rheumatology |
| title | A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma |
| title_full | A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma |
| title_fullStr | A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma |
| title_full_unstemmed | A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma |
| title_short | A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma |
| title_sort | unique presentation of anti rna polymerase iii positive systemic sclerosis sine scleroderma |
| url | http://dx.doi.org/10.1155/2016/8536341 |
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