Objective: To longitudinally evaluate esophageal dysmotility (ED) in patients with limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc).
Methods: We performed a retrospective review of all adult patients with SSc seen between 1995 and 2008. Patients were included if they had undergone 2 or more esophageal transit scintigraphy (ETS) studies at least 1 year apart. Data from 382 ETS studies of 102 patients with SSc were analyzed. Eighty patients had lcSSc and 22 patients had dcSSc. A grading system was used to quantify the degree of esophageal dysfunction, ranging from grade 0 (normal) to grade 3 (severe hypomotility). Change in esophageal motility over time was evaluated and compared between the limited and diffuse subtypes.
Results: Sixty-eight patients (66.7%) had an abnormal ETS study at any time. Of patients with dcSSc, 95.4% had an abnormal ETS study, compared to 58.5% of patients with lcSSc. dcSSc and regurgitation were independent risk factors for ED. There was no association between the presence of anticentromere antibodies or antitopoisomerase (anti-Scl-70) antibodies and an abnormal ETS study. Esophageal motility in patients with dcSSc worsened in 96% of cases compared with only 58.8% in those with lcSSc.
Conclusion: ED is more frequent in patients with dcSSc than in those with lcSSc, and is more likely to deteriorate over time. Given the potential associated risks of erosive esophagitis, Barrett's esophagus, and esophageal cancer in patients with SSc, routine screening and monitoring for ED is advised.
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http://dx.doi.org/10.3899/jrheum.110923 | DOI Listing |
Sci Rep
December 2024
Internal Medicine/Rheumatology, University of Texas Health Science Center at Houston, Houston, 77030, USA.
This study aimed to examine whether a reported SSc-associated SNP rs2841277 in the PLD4 gene identified in an Asian population was also associated with SSc in European American (EA). The EA cohort consisting of 1005 SSc patients and 961 healthy controls was examined in this study. TaqMan genotyping assays were performed to examine the SNP.
View Article and Find Full Text PDFRheumatology (Oxford)
December 2024
Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, the Netherlands.
Objectives: Cellular senescence and endothelial-to-mesenchymal transition (EndMT) are profibrotic cellular processes involved in systemic sclerosis (SSc), but how they respond to treatment is largely unknown.
Methods: Skin biopsies from diffuse cutaneous SSc (dcSSc) patients who underwent either autologous haematopoietic stem cell transplantation (aHSCT) or cyclophosphamide pulse (iv CYC) treatment were collected before and 6 months after randomisation in the Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial. The extent of fibrosis, inflammation, senescence, EndMT and tissue remodelling were examined in histopathology.
Semin Arthritis Rheum
February 2025
Rheumatology Unit, Department of Medicine-DIMED, Padova University Hospital, 35128 Padova, Italy; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, 35128 Padova, Italy. Electronic address:
Objectives: Anticentromere antibodies (ACA) are typically found in limited cutaneous systemic sclerosis (lcSSc), whereas patients with anti-topoisomerase I antibodies (ATA) usually exhibit diffuse cutaneous involvement (dcSSc). We aimed to investigate the clinical phenotype and outcome of ACA-dcSSc.
Methods: A systematic literature review was conducted (January 1970 to April 2023) across MEDLINE, Scopus and OVID, to define whether SSc patients (population) within the ACA-dcSSc subset (exposure) had higher/lower risk for major organ involvement (interstitial lung disease-ILD, pulmonary hypertension-PH, primary myocardial involvement-PMI, scleroderma renal crisis-SRC) and mortality (outcomes) compared to ACA-lcSSc and ATA-dcSSc.
RMD Open
November 2024
Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
Objective: Systemic sclerosis Impact of Disease (ScleroID) is the first comprehensive patient-reported outcome measure (PROM) specifically developed for systemic sclerosis (SSc). We investigated the performance of ScleroID in patients with diffuse cutaneous SSc (dcSSc), as a prerequisite for its use in randomised controlled trials (RCTs) testing potentially disease-modifying drugs.
Methods: All patients with dcSSc from the large, multicentric, ScleroID cohort were included.
Semin Arthritis Rheum
February 2025
Service de médecine interne et dermatologie, Hôpital Felix Guyon, CHU de la Réunion, 1 allée des Topazes 97400, Saint-Denis, France.
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