Autoantibodies in Patients with Early Systemic Sclerosis in the Collaborative National Quality and Efficacy Registry

Alyssa Bosso¹, Shervin Assassi², Tracy Frech³, Jessica Gordon⁴, Elana Bernstein⁵, Carrie Richardson⁶, Nora Sandorfi⁷, Laura Hummers⁸, Ami Shah⁹, Dinesh Khanna¹⁰, Lorinda Chung¹¹, Flavia Castelino¹², Faye Hant¹³, Victoria Shanmugam¹⁴, John VanBuren¹⁵, Angela Larkin¹⁶, Luke Evnin¹⁷ and Virginia Steen¹⁸, ¹Medstar Georgetown University Hospital, Washington, DC, ²McGovern Medical School, University of Texas, Houston, TX, ³Vanderbilt University Medical Center, Nashville, TN, ⁴Hospital for Special Surgery, New York, NY, ⁵Columbia University, New York, NY, ⁶Northwestern University, Riverside, IL, ⁷University of Pennsylvania, Philadelphia, PA, ⁸Johns Hopkins Univerisity, Baltimore, MD, ⁹Johns Hopkins Rheumatology, Baltimore, MD, ¹⁰Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹¹Stanford University, Stanford, CA, ¹²Massachusetts General Hospital, Boston, MA, ¹³Medical University of South Carolina, Charleston, SC, ¹⁴George Washington University, Great Falls, VA, ¹⁵University of Utah, Salt Lake City, UT, ¹⁶University of Utah Health, Salt Lake City, UT, ¹⁷Scleroderma Research Foundation, Brisbane, CA, ¹⁸Georgetown University School of Medicine, Washington, DC

Meeting: ACR Convergence 2022

Keywords: Autoantibody(ies), Systemic sclerosis

Session Information

Date: Monday, November 14, 2022

Title: Systemic Sclerosis and Related Disorders – Clinical Poster III

Session Type: Poster Session D

Session Time: 1:00PM-3:00PM

Background/Purpose: We sought to describe the autoantibody profile of SSc patients with early disease and examine the clinical, laboratory and prognostic features associated with these antibody subsets.

Methods: Study participants were from the Collaborative National Quality and Efficacy Registry (CONQUER), a multicenter US-based registry of SSc patients within 5 years (median 2.2-2.8 years) of their first non-Raynaud’s symptom. As of April 25, 2022, 533 patients were enrolled and had autoantibody testing as part of their clinical care. All subjects met 2013 ACR/ European League Against Rheumatism criteria for SSc. Baseline and most abnormal characteristics during follow-up were compared by antibody status.

Results: Of the 533 patients evaluated, the vast majority had a positive ANA screen; only 3.7% were ANA negative. 87 patients (16.3%) had ACA, 134 (25.1%) anti-RNA polymerase III (RNA pol III), 143 (26.8%) Scl-70, and 47 (8.8%) had an isolated nucleolar antibody pattern without other subserology. There were 5% of patients who had other SSc-associated subserologies, including 14 with anti-Pm/Scl, 3 anti-U3-RNP (also nucleolar ANA patterns) and 10 with U1-RNP. Almost 10% were ANA positive but did not have any SSc subserology, while 4.3%% had 2 or more subserologies. ACA patients were likely to be female Caucasians with limited cutaneous disease. They had the lowest modified Rodnan Skin Scores (mRSS) and highest SSc-GI tract (GIT) scores across groups. RNA pol III was strongly associated with diffuse cutaneous disease, having the most severe skin involvement of the groups by mRSS score and frequency of joint contractures and tendon friction rubs. Patients with RNA pol III had the highest rate of scleroderma renal crisis. Scl-70 patients were youngest at symptom onset; additionally, they had the most severe lung disease on pulmonary function tests (PFTs) and high-resolution CT imaging. Patients with an isolated nucleolar antibody were demographically and clinically most similar to Scl-70 patients; these groups had high proportions of African-American and male patients as well as more severe PFTs than other antibody groups. In the 1.6 years of follow up, the lowest forced vital capacity (FVC) measurements were seen in Scl-70 and isolated nucleolar antibody patients with median FVCs of 79% and 77% predicted, respectively (p< 0.001) (Table 2).

Conclusion: Even in early disease, ACA, RNA pol III and Scl-70 have the typical clinical associations that have been established with these antibodies. Patients with an isolated nucleolar antibody pattern without other SSc subserology should be followed closely for interstitial lung disease.

Table 1. Baseline characteristics by autoantibody

Table 2. Most abnormal measurements during follow up

Disclosures: A. Bosso, None; S. Assassi, Boehringer-Ingelheim, Janssen, Novartis, AstraZeneca, CSL Behring, AbbVie/Abbott; T. Frech, None; J. Gordon, None; E. Bernstein, Boehringer-Ingelheim, Kadmon, Pfizer; C. Richardson, None; N. Sandorfi, None; L. Hummers, Boehringer-Ingelheim, Corbus Pharmaceuticals, Cumberland Pharmaceuticals, Kadmon Corporation, Medpace, CSL Behring, Mitsubishi Tanabe, Horizon Pharmaceuticals; A. Shah, Arena Pharmaceuticals, Medpace/Eicos, Kadmon Corporation; D. Khanna, Boehringer Ingelheim, Genentech, Prometheus, Horizon, Chemomab, Talaris, Gesynta, Amgen, Acceleron, Actelion, Bayer, CSL Behring, Paracrine Cell Therapy, Mitsubishi Tanabe, Theraly, Eicos Sciences; L. Chung, Kyverna, Mitsubishi Tanabe, Eicos, Boehringer-Ingelheim, Jasper, Genentech; F. Castelino, Boehringer-Ingelheim; F. Hant, None; V. Shanmugam, None; J. VanBuren, None; A. Larkin, None; L. Evnin, None; V. Steen, None.

To cite this abstract in AMA style:

Bosso A, Assassi S, Frech T, Gordon J, Bernstein E, Richardson C, Sandorfi N, Hummers L, Shah A, Khanna D, Chung L, Castelino F, Hant F, Shanmugam V, VanBuren J, Larkin A, Evnin L, Steen V. Autoantibodies in Patients with Early Systemic Sclerosis in the Collaborative National Quality and Efficacy Registry [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/autoantibodies-in-patients-with-early-systemic-sclerosis-in-the-collaborative-national-quality-and-efficacy-registry/. Accessed .

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