To examine clinical course of early systemic sclerosis (SSc) and identify factors for progression of acro-osteolysis by a retrospective cohort study. Dual time-point hand radiography was performed at median interval (range 3.0 ± 0.4 years) in 64 recruited patients. Progressive acro-osteolysis was defined as the worsening of severity of acro-osteolysis according to rating scale (normal, mild, moderate, and severe). Incidence of the progression was determined. Cox regression was analyzed for the predictors. A total of 193.6 per 100 person-years, 19/64 patients had progressive acro-osteolysis with incidence of 9.8 per 100-person-years (95% CI 6.3-15.4). The median time of progressive acro-osteolysis was 3.5 years. Rate of progression increased from 1st to 3rd years follow-up with the progression rate at 1-, 2- and 3-years were 0, 2.0 and 18.3%, respectively. Patients with positive anti-topoisomerase I tended to have more progressive acro-osteolysis but no significant predictors on Cox regression. 44%, 18%, and 33% of who had no, mild, and moderate acro-osteolysis previously developed progression and 10 turned to be severe acro-osteolysis. In conclusion, the incidence of progressive acro-osteolysis was uncommon in early SSc but the rate of progression was pronouncedly increasing after three years follow-up. A half of the patients progressed to severe acro-osteolysis.

BACKGROUND: Skin tightness is a hallmark of systemic sclerosis (SSc), and the fingers are an affected body part, so much so that fingerprints can be significantly affected among those with extensive skin tightness of the finger.
OBJECTIVE: We aimed to compare the difference between the current and past (pre-disease onset) fingerprints of SSc patients.
METHODS: We conducted a cross-sectional study among adult SSc patients who attended the Scleroderma Clinic, Khon Kaen University, between October 2019, and September 2020. All eligible patients consented to obtaining their current and previous prints from the Central Registration Bureau, Department of Provincial Administration, Ministry of the Interior. The current prints were obtained using the Crossmatch (Lite UE) live scan from the Central Institute of Forensic Science of Thailand. We investigated the concordance between the prints before disease onset and the current (enroll date) via the Printquest AFIS system with officers from the Central Institute of Forensic Science, Thailand.
RESULTS: One hundred SSc patients, according to the sample size calculation, were enrolled (mean age 59.4 ± 9.6 years; 66% female). Most (70%) had the diffuse cutaneous SSc subset. A respective 59%, 55%, and 6% presented acro-osteolysis, hand deformities, and digital ulcers. Some challenges were experienced in obtaining prints from patients with loss of the fingertip fat pad, finger joint contracture, and/or acro-osteolysis; notwithstanding, all fingerprints were usable and without individualized changes.
CONCLUSIONS: Fingerprints were affected by fingertip lesions and finger joint contractures; notwithstanding, the prints remained usable for personal identification. Key Points • Skin involvement in systemic sclerosis (SSc) affects the prints, particularly at the fingertip. • Despite disease onset, the fingerprints of SSc patients do not change significantly. • Fingerprints are inadequate for personal identification among SSc patients with hand deformities due to poor quality or difficulty acquiring them.

Calcinosis usually represents a late manifestation of systemic sclerosis (SSc), inducing tissue damage and chronic calcifications. To analyze clinical and bone metabolism parameters associated with calcinosis in limited systemic sclerosis (lSSc), thirty-six female lSSc patients with calcinosis were compared with 36 female lSSc patients without calcinosis, matched by age, disease duration, and body mass index. Organ involvement, autoantibodies, bone density, and laboratory parameters were analyzed. Statistical significance was considered if p < 0.05. Calcinosis was significantly associated with acroosteolysis (69% vs. 22%, p < 0.001), higher modified Rodnan skin score (mRSS 4.28 ± 4.66 vs. 1.17 ± 2.50, p < 0.001), and higher 25-hydroxyvitamin D (25OHD) (24.46 ± 8.15 vs. 20.80 ± 6.60 ng/ml, p = 0.040) and phosphorus serum levels (3.81 ± 0.41 vs. 3.43 ± 0.45 mg/dl, p < 0.001). 25OHD levels > 30 ng/ml were also significantly more frequent in patients with calcinosis (p = 0.041). Regarding treatment, current use of corticosteroids was lower in patients with calcinosis compared with patients without calcinosis (8% vs. 28%, p = 0.032). On logistic regression analysis, acroosteolysis (OR = 12.04; 95% CI, 2.73-53.04; p = 0.001), mRSS (OR = 1.37; 95% CI, 1.11-1.69; p = 0.003), phosphorus serum levels (OR = 5.07; 95% CI, 1.06-24.23; p = 0.042), and lower glucocorticoid use (OR = 0.07; 95% CI, 0.007-0.66; p = 0.021) are independent risk factors for calcinosis. This study showed that limited SSc patients with calcinosis present a distinct clinic and biochemical profile when compared with a matched group without calcinosis, paired by disease duration, age and BMI. KEY POINTS: • Calcinosis in patients with limited SSc was associated with acroosteolysis, higher mRSS and higher serum levels of phosphorus.

We sought to identify the clinical factors associated with calcinosis in an international multicenter collaborative effort with the Scleroderma Clinical Trials Consortium (SCTC).
This is a retrospective cohort study of 5218 patients with systemic sclerosis (SSc). Logistic regression was used to obtain odds ratios (OR) relating calcinosis to various clinical features in multivariate analyses.
A total of 1290 patients (24.7%) had calcinosis. In univariate analyses, patients with calcinosis were older than patients without calcinosis, more likely to be female, and had longer disease duration from the first non-Raynaud phenomenon symptom. Patients with calcinosis were more likely to have digital ulcers, telangiectasias, acro-osteolysis, cardiac disease, pulmonary hypertension, gastrointestinal involvement, arthritis, and osteoporosis, but less likely to have muscle disease. Anti-Scl-70, RNA-polymerase-III, and U1-RNP autoantibodies were significantly less common in patients with calcinosis, while anticentromere (ACA), anti-PM/Scl, and anticardiolipin antibodies were more frequent. In multivariate analysis, the strongest associations with calcinosis were digital ulcers (OR = 3.9; 95% CI: 2.7-5.5; p < 0.0001) and osteoporosis (OR = 4.2; 95% CI: 2.3-7.9; p < 0.0001).
One quarter of patients with SSc have calcinosis at some time during their illness. Our data confirm a strong association of calcinosis with digital ulcers, and support a novel association with osteoporosis.

OBJECTIVE: To examine the outcomes of hand radiographic x-rays in patients with systemic sclerosis (SSc) and to identify risk factors for the progression of hand radiographic lesions in a prospective cohort.
METHODS: Dual time-point x-rays were systematically performed after a median interval of 5 years (range 4-7 years) in 103 consecutively recruited patients with SSc. Univariate and multivariate Cox proportional hazards models evaluated predictors of progression of hand radiographic lesions.
RESULTS: Radiographic progression of erosive arthritis, acro-osteolysis, calcinosis and flexion contracture occurred in 24, 22, 27 and 18 patients, respectively. Multivariate Cox regression analysis did not identify any predictor of the progression of erosive arthritis. Digital ulcers were shown independently to predict the progression of acro-osteolysis and calcinosis (HR 12.43, 95% CI 1.97 to 88.40 and 3.16, 95% CI 1.22% to 9.43%, respectively). The diffuse cutaneous subset was shown to be an independent predictor of the progression of flexion contracture (HR 7.52, 95% CI 1.21 to 43.93).
CONCLUSIONS: The results highlight the striking level of hand radiographic lesions in SSc and suggest close monitoring of patients with the diffuse cutaneous subset for the occurrence or worsening of this complication. The results also show that severe peripheral vascular involvement predicts both acro-osteolysis and calcinosis, highlighting their vascular background.

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