Autoimmun Rev 2017 Oct 1;16(10):1029-1035. Epub 2017 Aug 1.
Rheumatology Division, Department of Medicine, Georgetown University, United States.
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Autoimmun Rev 2016 Jul 10;15(7):690-4. Epub 2016 Mar 10.
Department of Rheumatology, University of Lübeck, Lübeck, Germany. Electronic address:
Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology characterized by the presence of multiple autoantibodies, including those against angiotensin and endothelin receptors. Patients with SSc can develop heterogeneous clinical manifestations including microvascular damage, the dysregulation of innate and adaptive immunity, and generalized fibrosis of multiple organs. Autoantibodies against angiotensin II type I receptor (AT1R) and endothelin-1 type A receptor (ETAR) play important roles in the pathogenesis of SSc. Read More
Am J Respir Crit Care Med 2014 Oct;190(7):808-17
1 Department of Rheumatology and Clinical Immunology.
Rationale: Systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) portends worse outcome than other forms of PAH. Vasoconstrictive and vascular remodeling actions of endothelin (ET) 1 and angiotensin (Ang) II via endothelin receptor type A (ETAR) and Ang receptor type-1 (AT1R) activation are implicated in PAH pathogenesis.
Objectives: We hypothesized that stimulating autoantibodies (Abs) targeting and activating AT1R and ETAR may contribute to SSc-PAH pathogenesis, and tested their functional and biomarker relevance. Read More
Curr Rheumatol Rep 2015 May;17(5):34
Department of Rheumatology and Clinical Immunology, University Hospital Charité, Luisenstraße 13, 10117, Berlin, Germany,
Circulating antinuclear autoantibodies contribute to the diagnosis of systemic sclerosis (SSc) and correlate with disease-specific organ manifestations. Recent findings show the induction of interstitial lung disease and obliterative vasculopathy by transfer of IgG from SSc patients in healthy mice indicating a contribution of antibodies to SSc pathogenesis. Several functional or agonistic autoantibodies have been described in SSc, thus putting autoimmunity into a new spotlight. Read More
Arthritis Res Ther 2014 Jan 28;16(1):R29. Epub 2014 Jan 28.
Introduction: Vasculopathy, inflammatory fibrosis and functional autoantibodies (Abs) are major manifestations of systemic sclerosis (SSc). Abs directed against the angiotensin II type 1 receptor (AT₁R) and endothelin-1 type A receptor (ETAR) are associated with characteristic disease features including vascular, inflammatory, and fibrotic complications indicating their role in SSc pathogenesis. Therefore, the impact of anti-AT₁R and anti-ETAR Abs on initiation of inflammation and fibrosis was analyzed. Read More