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Abstract Number: 2443

The Clinical Phenotype of Anti-Th/To+ Patients in Systemic Sclerosis: A Case-control Study Within the European Scleroderma Trials and Research (EUSTAR) Cohort

Liala Moschetti1, Eleonora Pedretti2, Francesco Bonomi3, María Martín López4, Fabio Cacciapaglia5, cristiana sieiro santos6, Gianluca Moroncini7, Yannick Allanore8, Joana Caetano9, Brigitte Granel10, Laura Groseanu11, Maria De Santis12, Masataka Kuwana13, Veronica Codullo14, Mariana Pereira Silva15, Pietro Bearzi16, Laura Belloli17, Alida Taberner-Cortés18, Cristina Maglio19, Francesco Del Galdo20, Corrado Campochiaro21, Marie-Elise Truchetet22, Giovanna Cuomo23, Magda Parvu24, Florenzo Iannone25, Patricia Carreira26, Serena Guiducci27, Franco Franceschini1, Paolo Airò1 and Maria-Grazia Lazzaroni1, 1Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, 2Scleroderma Unit, Rheumatology Unit, ERN ReCONNET, ASST Spedali Civili and University of Brescia; Italy, Brescia, Italy, 3Division of Rheumatology, Scleroderma Unit, University of Florence, AOU Careggi Firenze; Italy, Firenze, Italy, 4General University Hospital of Ciudad Real, Ciudad de México, Spain, 5Rheumatology Unit � DiMePRe-J, University and AOU Policlinico of Bari, Italy, Bari, Italy, 6Rheumatology Department, Complejo Asistencial Universitario de León, León, Spain, Leon, Spain, 7Marche Polytechnic University, Ancona, Italy, 8Rheumatology department, Université Paris Cité, Cochin Hospital of Paris; France, Paris, France, 9Systemic Autoimmune Diseases Unit, Fernando Fonseca Hospital, Amadora; Portugal, Amadora, Portugal, 10Service de Médecine Interne Hôpital Nord de Marseille; France, Marseille, France, 11Spitalul Sfanta Maria, Bucharest, Romania, 12Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital and Biomedical Sciences, Hu-manitas University, Milan; Italy, Milan, Italy, 13Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, 14Unit of Rheumatology, San Matteo Hospital, Pavia, Italy, 15Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa; Portugal, Lisboa, Portugal, 16Immunorheumatology Unit, Università Campus Bio-Medico University of Roma; Italy, Roma, Italy, 17Niguarda Hospital, Milan, Milan, Italy, 18Hospital Universitario Doctor Peset, Valencia, Spain, 19University of Gothenburg, Gothenburg, Sweden, 20University of Leeds, Leeds, United Kingdom, 21IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milan, Milan, Italy, 22Bordeaux University Hospital, Bordeaux, France, 23Università degli studi della Campania Luigi Vanvitelli, Napoli, Italy, 24Colentina Clinical Hospital, Rheumatology Department, Bucharest; Romania, Bucharest, Romania, 25Rheumatology Unit- University of Bari "Aldo Moro", IT, Bari, Italy, 26Hospital Universitario 12 de Octubre, Madrid, Madrid, Spain, 27Division of Rheumatology, Scleroderma Unit, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

Meeting: ACR Convergence 2024

Keywords: Autoantibody(ies), Damage Index, interstitial lung disease, Mortality, Systemic sclerosis

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Session Information

Date: Monday, November 18, 2024

Title: Systemic Sclerosis & Related Disorders – Clinical Poster III

Session Type: Poster Session C

Session Time: 10:30AM-12:30PM

Background/Purpose: To evaluate clinical associations of anti-Th/To antibodies in SSc patients in a multicentre international cohort, focusing on interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), synchronous malignancies, organ damage, mortality.

Methods: A case-control study of prospectively collected data from 23 EUSTAR centres was performed (CP144). For every anti-Th/To+ SSc case, centres provided 2 anti-Th/To- SSc controls matched by sex, age at onset (±5 years) and disease duration (±24 months). ILD functional progression was defined as absolute decline in %pFVC≥5% and/or %pDLCO >10% over 12 months1. Synchronous malignancies were defined as diagnosed within ±2 years from SSc onset2. Organ damage was evaluated through the Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI)3.

Results: 89 anti-Th/To+ SSc patients, as compared to 178 anti-Th/To- (Table 1), had higher rate of concomitant anti-Ro52 positivity and lower frequency of diffuse cutaneous involvement and digital ulcers/pitting.

ILD was detected in 40.5% anti-Th/To+ patients (vs 55.2% in anti-Th/To-, p:0.032), with non-specific interstitial pneumonia (NSIP) pattern in 71.9%. Functional progression was detected in 46.9% during the first 5 years of disease, similarly to the control group. Only 1 (3.1%) anti-Th/To+ patient developed group 3 PH. A higher prevalence of usual interstitial pneumonia (UIP) pattern was found in cases.

PAH was confirmed at right heart catheterization in 3 (3.6%) anti-Th/To+ cases, slightly less frequently than in controls.

Malignancies rate was similar in the two groups. In anti-Th/To+ cases, the most common synchronous cancer type was breast cancer in 5 cases (2 infiltrating ductal carcinomas, 1 infiltrating lobular carcinoma, 2 not specified), followed by 1 pancreatic, 1 thyroid 1 cecum cancer.

Anti-Th/To+ patients accrued less organ damage as compared to controls, exhibiting lower DI scores after 2, 3 and 5 years from SSc onset.

6 (6.7%) anti-Th/To+ patients died at 79.5 (72.5-84.3) years of age and 9.5 (9.0-17.5) years of disease duration with no deaths attributable to SSc-ILD or other SSc complications. Survival curves are depicted in Figure 1: anti-Th/To+ patients had a survival rate of 100.0% at 1 year, 98.8% at 5 years, and 89.9% at both 10 and 15 years of disease duration, slightly higher than controls (98.8% at 1, 94.5% at 5, 90.3% at 10 and 76.7% at 15 years of disease duration).

Conclusion: Through the analysis of this multicentric series of anti-Th/To+ SSc patients, well known clinical associations were confirmed in particular limited cutaneous involvement in most cases and ILD in half of the cases. PAH seems to be part of anti-Th/To spectrum only in a small percentage of patients while the relationship with synchronous malignancies needs to be better explained in future analyses. Globally, it seems that anti-Th/To+ patients have favorable outcome characterized by mild organ damage and good survival.

References: 1Raghu G. 2022;2Lazzaroni MG. 2017;3Ferdowsi N. 2019

Supporting image 1

Supporting image 2


Disclosures: L. Moschetti: None; E. Pedretti: None; F. Bonomi: None; M. Martín López: None; F. Cacciapaglia: AbbVie/Abbott, 2, 6, Boehringer-Ingelheim, 2, 6, Eli Lilly, 6, Galapagos, 6, Janssen, 2, 6, Pfizer, 6; c. sieiro santos: None; G. Moroncini: None; Y. Allanore: None; J. Caetano: None; B. Granel: None; L. Groseanu: None; M. De Santis: None; M. Kuwana: Asahi Kasei Pharma, 6, AstraZeneca, 2, Boehringer Ingelheim, 2, 6, Chugai, 2, 6, GSK, 2, MBL, 9, Ono Pharmaceuticals, 6; V. Codullo: None; M. Pereira Silva: None; P. Bearzi: None; L. Belloli: None; A. Taberner-Cortés: None; C. Maglio: None; F. Del Galdo: AbbVie, 2, 5, Argenx, 2, Arxx, 2, 5, AstraZeneca, 2, 5, Boehringer Ingelheim, 2, 5, Chemomab, 5, Deepcure, 2, GlaxoSmithKline (GSK), 2, Janssen, 2, Mitsubishi Tanabe, 5, Novartis, 2, Ventus, 2; C. Campochiaro: Boehringer-Ingelheim, 1, 6, Janssen, 6, Novartis, 1, 6; M. Truchetet: None; G. Cuomo: None; M. Parvu: None; F. Iannone: AstraZeneca, 2, GSK, 2, Pfizer, 2, UCB, 2; P. Carreira: None; S. Guiducci: None; F. Franceschini: None; P. Airò: None; M. Lazzaroni: None.

To cite this abstract in AMA style:

Moschetti L, Pedretti E, Bonomi F, Martín López M, Cacciapaglia F, sieiro santos c, Moroncini G, Allanore Y, Caetano J, Granel B, Groseanu L, De Santis M, Kuwana M, Codullo V, Pereira Silva M, Bearzi P, Belloli L, Taberner-Cortés A, Maglio C, Del Galdo F, Campochiaro C, Truchetet M, Cuomo G, Parvu M, Iannone F, Carreira P, Guiducci S, Franceschini F, Airò P, Lazzaroni M. The Clinical Phenotype of Anti-Th/To+ Patients in Systemic Sclerosis: A Case-control Study Within the European Scleroderma Trials and Research (EUSTAR) Cohort [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/the-clinical-phenotype-of-anti-th-to-patients-in-systemic-sclerosis-a-case-control-study-within-the-european-scleroderma-trials-and-research-eustar-cohort/. Accessed .
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