Juvenile localized and systemic scleroderma are rare autoimmune diseases which cause significant disability and morbidity in children. The mechanisms driving juvenile scleroderma remain unclear, neces...
The Localized Scleroderma Quality of Life Instrument (LoSQI) is a disease-specific patient-reported outcome (PRO) measure designed for children and adolescents with localized scleroderma (LS; morphea)...
The purpose of this study was to evaluate the psychometric properties of the LoSQI in a clinical setting....
Cross-sectional data from four specialized clinics in the US and Canada were included in the analysis. Evaluation included reliability of scores, internal structure of the survey, evidence of converge...
One hundred and ten patients with LS (age: 8-20 years) completed the LoSQI. Both exploratory and confirmatory factor analysis supported the use of two sub-scores: Pain and Physical Functioning, and Bo...
This study did not evaluate longitudinal validity or responsiveness of scores....
Results from a representative sample of children and adolescents with LS continue to support the validity of the LoSQI when used in a clinical setting. Future work to evaluate the responsiveness is on...
Patients with localized scleroderma (LS) often have to seek plastic surgery to improve facial esthetic impairment....
The authors reported a case of the reactivation of LS after autologous fat grafting (AFG)....
A man presented with facial atrophy and skin fibrosis on the cheek with a history of LS. The disease had remained stable for 9 years and he was suggested to stop oral medication. With irreversible est...
Six months after the last AFG, the patient found new lesions occur on the lateral forehead. The patient was later diagnosed with reactivation of LS....
The understanding of the surgical risk and perioperative management for patients with LS needs more research....
One of the key clinical challenges of systemic sclerosis (SSc) is diversity in clinical presentation, organ involvement and disease progression. Antinuclear autoantibodies (ANA) are central to the dia...
There has been further development in delineating clinical patterns in ANA, genetic susceptibility and antigen triggers predisposing to ANA subtypes. Sub-group analysis of recent clinical trials shows...
ANA subtyping is likely to be firmly embedded into future classification systems. Beyond informing current management and monitoring of scleroderma patients, ANA subsets have implication on future res...
People with systemic sclerosis (SSc) are vulnerable in COVID-19 and face challenges related to shifting COVID-19 risk and protective restrictions. We evaluated mental health symptom trajectories in pe...
The longitudinal Scleroderma Patient-centred Intervention Network (SPIN) COVID-19 cohort was launched in April 2020 and included participants from the ongoing SPIN Cohort and external enrolees. Analys...
Anxiety symptoms increased in early 2020 but returned to pre-COVID-19 levels by mid-2020 and remained stable through March 2022. Depression symptoms did not initially change but were slightly lower by...
People with SSc continue to face COVID-19 challenges related to ongoing risk, the opening of societies, and removal of protective restrictions. People with SSc, in aggregate, appear to be weathering t...
Systemic sclerosis (SSc) represents extremely rare disease with majority of data coming from adults. Studies comparing juvenile- (jSSc) and adult-onset (aSSc) patients are limited. We aimed to compare...
A retrospective study among pediatric and adult Scl patients has been performed. Demographic characteristics, clinical features, autoantibody profiles, and treatment data were retrieved from the datab...
A total of 158 adults and 58 juvenile Scl patients were identified. The mean age at the disease onset was 37±14.7 vs. 8.8 ± 4.1 years, mean age at diagnosis 42±15.2 vs. 10.4 ± 3.8 years and mean follo...
Juvenile and adult-onset Scl represent rarely seen conditions with different clinical phenotypes. Pediatric patients with LS are more commonly seen by pediatric rheumatologists, in contrary to adults....
We investigated the ability of a panel of immune-related cytokines and chemokines to predict the disease activity state in localized scleroderma (LS) subjects followed longitudinally. A total of 194 s...
Systemic sclerosis (SSc) is characterized by the presence of SSc-specific or SSc-associated antibodies (SSc-Abs): anti-topoisomerase I (ATA), anti-centromere (ACA), anti-RNA polymerase III (ARA), anti...
Erectile dysfunction (ED) has been reported among patients with systemic sclerosis (SSc) and primarily limited cutaneous SSc in Caucasians. While there is no data on ED among Thais in whom the diffuse...
We aimed to estimate the prevalence of ED among Thais with SSc, evaluate its severity, and determine the associated factors....
We did a cross-sectional study among adult Thai male SSc patients. All eligible patients: a) completed the IIEF-15 questionnaire by themselves; b) underwent a genital examination by an experienced uro...
A total of 60 male SSc patients were included. The respective mean age and median disease duration was 54.8±7.2 years and 3.1 years (IQR 1.2-7.2). The definition of ED was fulfilled in 53 cases for a ...
ED is a common problem in men with SSc and is mainly categorized as severe. The severity of SSc might increase the risk of developing ED. We found phimosis was a common genital abnormality co-occurrin...
Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a mortality of 20% at 6 months. Once the leading cause of mortality in scleroderma (SSc), it remains ...
