ArticleArthritis & rheumatology (Hoboken, N.J.)2022
Development of Pulmonary Hypertension in Over One-Third of Patients With Th/To Antibody-Positive Scleroderma in Long-Term Follow-Up.
Article in Arthritis & rheumatology (Hoboken, N.J.), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
11 citing papers in PubMed, 1 synthesis or guideline pooled it, 23 citations in OpenAlex.
- The association between the neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio, and monocyte-to-lymphocyte ratio and systemic sclerosis and its complications: a systematic review and meta-analysis.Frontiers in immunology · 2024Pooled it
- The clinical phenotype of anti-Th/To+ patients in systemic sclerosis: a case-control study within the European Scleroderma Trials and Research cohort.Therapeutic advances in musculoskeletal disease · 2026Article
- Case Report: Acute interstitial pneumonia as the initial presentation of Anti-EJ antisynthetase syndrome: a case of severe respiratory failure with rapid response to cyclophosphamide.Frontiers in immunology · 2026Article
- Article
- Racial variability in immune responses only partially explains differential systemic sclerosis disease severity.Annals of the rheumatic diseases · 2024Observational
- Anti-Th/To Antibodies in Scleroderma: Good Prognosis or Serious Concern?Journal of clinical medicine · 2024Review
- Localized scleroderma with pulmonary arterial hypertension and pulmonary interstitial fibrosis in a patient with positive Th/to antibodies: Case report and review of literature.Clinical case reports · 2023Article
- Advanced Autoantibody Testing in Systemic Sclerosis.Diagnostics (Basel, Switzerland) · 2023Review
- Very Early Diagnosis of Systemic Sclerosis: Deciphering the heterogeneity of systemic sclerosis in the very early stages of the disease.Journal of scleroderma and related disorders · 2023Article
- Systemic sclerosis.Lancet (London, England) · 2023Review
- Comprehensive autoantibody profiles in systemic sclerosis: Clinical cluster analysis.Frontiers in immunology · 2022Article
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
objectiveThis study was undertaken to describe clinical manifestations in patients with Th/To antibody-positive systemic sclerosis (SSc) during long-term follow-up.
methodsWe performed a case-control study involving anti-Th/To antibody-positive patients with SSc who were newly referred to the University of Pittsburgh Medical Center and the Pittsburgh Scleroderma Center from 1980 to 2015. For every case, 2 anti-Th/To antibody-negative SSc patients (the first 2 consecutively seen after a case) were used as controls. Long-term disease manifestations and survival were then compared between cases and controls.
resultsA total of 204 anti-Th/To antibody-positive SSc patients and 408 controls were identified. The cohort had a mean ± SD age of 52 ± 12.9 years, and 76% of individuals were women. Anti-Th/To antibody-positive patients more often presented without skin thickening (P < 0.0001) and had a higher rate of pulmonary hypertension (PH) (P < 0.0001) and interstitial lung disease (P = 0.05) compared to anti-Th/To antibody-negative SSc controls. Anti-Th/To antibody-positive SSc patients also had less frequent muscle and joint involvement than anti-Th/To antibody-negative SSc controls (P < 0.0001). After a median clinical follow-up period of 6.1 years (interquartile range 2.4-12.7), 38% of anti-Th/To-positive patients had developed PH compared to 15% of anti-Th/To antibody-negative SSc controls (P < 0.0001). The rate of PH classified as World Health Organization (WHO) Group 1 pulmonary arterial hypertension [PAH] was 23% in anti-Th/To-positive patients compared to 9% in anti-Th/To antibody-negative SSc controls (P < 0.0001). After adjusting for age and sex, anti-Th/To antibody positivity was associated with a hazard ratio (HR) of 3.3 (95% confidence interval 2.3-4.9) for increased risk of developing PH at 10 years of follow-up from the first scleroderma center visit.
conclusionThis is the largest cohort of patients with anti-Th/To antibody-positive SSc with long-term follow-up data. The very high rate (38%) and associated independent risk of anti-Th/To antibody-positive patients developing PH in follow-up, particularly in WHO Group 1 PAH patients, is striking. Patients presenting with limited skin involvement should be tested for Th/To antibodies, and if present, careful monitoring for PH is warranted.
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