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Background And Aims: The aetiology of systemic scleroderma remains poorly understood. Twin studies suggest a low genetic input. Of the incriminated environmental agents, silica and vinyl chloride monomer exposure appear the most convincing. Spatiotemporal clustering has been demonstrated only three times previously. We now report a fourth cluster around Edenhope, western Victoria in terms of numerator and denominator estimates, cumulative incidence, distribution in time and space, and possible aetiological factors.
Methods: Prevalence/cumulative incidence numerator and denominator values were obtained and validated. Each case was age-and gender-matched with two controls. A standardized postal questionnaire was used to obtain data on current, past history, family history, and occupational and non-occupational environmental exposure.
Results: Six systemic scleroderma cases and one mixed connective tissue disease patient with a predominance of scleroderma features were identified. The 5-year cumulative incidence was 6.1/10,000, tenfold higher than the Sydney estimates for a similar, though non-identical time period. The gender ratio was 1:1. No cases were genetically related. A family history of scleroderma was validated in one instance and a family history of Raynaud's was noted in first degree relatives of two cases and one control. In all instances, Edenhope residence preceded disease onset. No one environmental agent was implicated in all cases.
Conclusion: A spatiotemporal cluster of systemic scleroderma was confirmed and validated. It occurred with a tenfold increased cumulative incidence to that expected and also extended beyond the initially defined 50 km radius of Edenhope. The cases identified were not related. Although no one specific environmental agent was identified, the spatiotemporal clustering would be compatible with an agent occurring at relatively high frequency, but with low disease conversion rates, such as silica inhalation (assuming sufficiently small particle size) or reaction to an infective agent.
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http://dx.doi.org/10.1111/j.1445-5994.2005.00783.x | DOI Listing |
Rheumatology (Oxford)
March 2025
Sanofi, Cambridge, Massachusetts, USA.
Objectives: To determine the efficacy, safety, and pharmacodynamics of belumosudil in patients with diffuse cutaneous systemic sclerosis (dcSSc) treated with background immunosuppressive therapies.
Methods: Eligible patients were randomised 1:1:1 to receive belumosudil 200 mg once daily (QD) or twice daily (BID), or placebo for 28 weeks (double-blind period). After unblinding, the patients who received belumosudil continued the same dose, whereas the patients who received placebo were re-randomised for one of the belumosudil doses for 24 weeks (open-label extension).
Rheumatol Int
March 2025
Physical Medicine and Rehabilitation Department, Rheumatology Division, Marmara University School of Medicine, Istanbul, Türkiye.
Objective: The Duruöz Hand Index (DHI) is a self-report questionnaire originally developed to assess hand function in rheumatoid arthritis patients and validated for various rheumatic conditions. The aim of the study is to evaluate the validity and psychometric features of the DHI in patients with systemic sclerosis (SSc).
Methods: SSc patients diagnosed using EULAR/ACR 2013 criteria were included in study.
J Assoc Physicians India
March 2025
DNB Resident, Department of General Medicine, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Mumbai, Maharashtra, India.
Purpose: Our research aimed at characterizing the range of signs and symptoms observed in scleroderma patients at a tertiary care hospital and investigating potential correlations between pulmonary and cutaneous involvement.
Materials And Methods: We obtained informed consent from scleroderma patients for conducting a comprehensive clinical assessment, including detailed medical histories and physical examinations. Standard diagnostic investigations like complete blood counts, erythrocyte sedimentation rate, renal function, liver function, electrocardiograms (ECG), high-resolution computed tomography (HRCT) scans, and pulmonary function tests were performed.
Sci Rep
March 2025
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Although higher prevalence of systemic sclerosis (SSc) in women than in men is well-known, it is unclear to what degree women are at higher risk of developing incident SSc. This study aims to assess the risk of incident SSc comparing women vs. men, and to identify subsets of individuals in whom the risk difference according to sex is more prominent.
View Article and Find Full Text PDFCureus
February 2025
Internal Medicine, Clinical Hospital Complex of the Federal University of Paraná, Curitiba, BRA.
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis and multi-organ dysfunction, primarily affecting the heart, lungs, and kidneys. Scleroderma renal crisis (SRC) can present as hypertensive or normotensive, with the latter being more challenging to diagnose due to the absence of hypertension at onset. Normotensive SRC carries a worse prognosis, with an increased risk of renal failure and a poor response to treatment.
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