title: A Unique Presentation of Anti-RNA Polymerase III Positive Systemic Sclerosis Sine Scleroderma.
abstract: 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.
authors: Department of Internal Medicine, University of Cincinnati, Cincinnati, OH 45267, USA.
authors: Cody M
authors: 0000-0002-8521-271X
authors: CM
authors: Lee
authors: Division of Immunology, Allergy and Rheumatology, University of Cincinnati, Cincinnati, OH 45267, USA.
authors: Diana
authors: 0000-0001-8976-176X
authors: D
authors: Girnita
authors: Department of Internal Medicine, The Christ Hospital, Cincinnati, OH 45219, USA.
authors: Arundhati
authors: 0000-0002-7129-9965
authors: A
authors: Sharma
authors: Division of Immunology, Allergy and Rheumatology, University of Cincinnati, Cincinnati, OH 45267, USA.
authors: Surabhi
authors: 0000-0002-1980-5648
authors: S
authors: Khanna
authors: Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, OH 45267, USA.
authors: Jean M
authors: 0000-0001-6199-1707
authors: JM
authors: Elwing