Systemic scleroderma (SSc) is a systemic immune-mediated connective tissue disease characterized by fibroproductive changes in connective tissue and microvascular disorders. The disease affects the skin, musculoskeletal system and internal organs. It is a disease with a significant rate of morbidity and mortality, significantly worsening the quality of life of patients. Early initiation of therapy is necessary to prevent disease progression. This review article discusses the current possibilities of early diagnosis of systemic scleroderma.

• Keywords
• diagnosis, diagnostics, early forms of scleroderma, systemic scleroderma, systemic sclerosis,
• MeSH
• Early Diagnosis MeSH
• Quality of Life MeSH
• Humans MeSH
• Connective Tissue Diseases * MeSH
• Scleroderma, Systemic * diagnosis   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Review MeSH

Although drug compliance is a crucial component of treatment effectiveness in chronic diseases, it has never been evaluated in patients with systemic scleroderma. Therefore, the aim of this descriptive study was to determine the drug compliance rate in systemic scleroderma patients and to identify risk factors for noncompliance in these patients. A cross-sectional observational study was conducted. All patients with systemic scleroderma (n = 41) who visited a rheumatic center and signed an informed consent form were included. Data were obtained during structured interviews with patients and from medical records. The Compliance Questionnaire Rheumatology (CQR) was used to determine patient compliance. The relationships between compliance rate and demographic and clinical characteristics were examined. The mean CQR score was 75 %. Based on a dichotomous rating, only 42 % of the patients achieved a satisfactory compliance rate (≥80 %). No relationships between various demographic and clinical characteristics and CQR score expressed as continuous or dichotomous variables were found. This study represents the first evaluation of drug compliance in patients with systemic scleroderma. Many noncompliant patients were identified, but no common risk factors for noncompliance were discovered. The reasons for noncompliance seem to depend on the personal features of the patients.

• MeSH
• Medication Adherence * MeSH
• Patient Compliance MeSH
• Chronic Disease MeSH
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Cross-Sectional Studies MeSH
• Surveys and Questionnaires MeSH
• Rheumatology methods   standards   MeSH
• Aged MeSH
• Scleroderma, Systemic drug therapy   MeSH
• Treatment Outcome MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH

The aim of this study was to assess the degradation of collagen type I and proinflammatory cytokines in systemic and localized scleroderma compared with psoriasis and healthy controls. Total 99 individuals were examined - 24 with SSc, 22 with LSc, 39 patients with PsV and 14 healthy controls. U-PD and U-DPD were measured using a sensitive isocratic HPLC method. Serum levels of IL-6 and soluble IL-2R were assayed using commercial ELISA kits. In the SSc group U-PD and U-DPD levels (nmol/mmol creatinine) were increased compared with controls (P = 0.001) and with PsV (P = 0.006). IL-6 levels were increased compared with controls (P = 0.004) and with PsV (P = 0.002). IL-2R concentrations were insignificantly increased in comparison with controls and were lower than in PsV, but the difference was not significant. In the LSc group excretion of U-PD and U-DPD did not differ from controls, but was insignificantly decreased compared with PsV. IL-6 levels were increased compared with controls (P = 0.001) and also with PsV (P = 0.03). IL-2R concentrations were significantly increased in comparison with controls only (P = 0.03). In patients with SSc our data have shown the most intensive collagen degradation and simultaneously an active inflammation, as documented by IL-6, which reflects the pathological processes in the skin and visceral organs compared with PsV patients and healthy individuals. In the LSc group collagen degradation was similar to that in control groups, but a certain inflammatory activity was observed.

• MeSH
• Cytokines blood   MeSH
• Demography MeSH
• Collagen metabolism   urine   MeSH
• Middle Aged MeSH
• Humans MeSH
• Scleroderma, Localized blood   metabolism   MeSH
• Inflammation Mediators blood   MeSH
• Protein Processing, Post-Translational * MeSH
• Scleroderma, Systemic blood   metabolism   MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Names of Substances
• Cytokines MeSH
• Collagen MeSH
• Inflammation Mediators MeSH

• Keywords
• ELECTROENCEPHALOGRAPHY *, SCLERODERMA/physiology *,
• MeSH
• Basidiomycota * MeSH
• Electroencephalography * MeSH
• Scleroderma, Localized * MeSH
• Scleroderma, Systemic physiology   MeSH
• Publication type
• Journal Article MeSH

• Keywords
• SCLERODERMA/complications *, SPINE/diseases *,
• MeSH
• Communication * MeSH
• Humans MeSH
• Scleroderma, Localized * MeSH
• Disease * MeSH
• Spinal Diseases * MeSH
• Spine * MeSH
• Scleroderma, Systemic complications   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH

OBJECTIVE: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis. METHODS: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months. RESULTS: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males. Race, age of onset, disease duration, and disease subtypes (70% diffuse cutaneous) were similar between males and females. Active digital ulceration, very low body mass index, and tendon friction rubs were significantly more frequent in males. Physician global assessment of disease severity and digital ulcer activity was significantly higher in males. Composite pulmonary involvement was also more frequent in males, though not statistically significantly. After 12 months, they are the pattern of differences changed female patients had significantly more frequent pulmonary involvement. CONCLUSION: In this cohort, juvenile onset systemic sclerosis had a more severe course in males at baseline and but the pattern changed after 12 months. Some differences from adult findings persisted, there is no increased signal of pulmonary arterial hypertension or heart failure in male pediatric patients. While monitoring protocols of organ involvement in juvenile onset systemic sclerosis need to be identical for males and females.

• Keywords
• Scleroderma, clinical characteristics, disease severity, gender, juvenile systemic sclerosis, male,
• Publication type
• Journal Article MeSH

Systemic scleroderma is a general disease of the connective tissue which may affect practically any organ. The authors describe the case of a man where in the course of the disease affection of the heart with symptoms of severe cardiac failure became the dominating symptom.

• MeSH
• Cardiomyopathies diagnosis   etiology   MeSH
• Humans MeSH
• Aged MeSH
• Scleroderma, Systemic complications   MeSH
• Cardiac Output, High etiology   MeSH
• Check Tag
• Humans MeSH
• Aged MeSH
• Female MeSH
• Publication type
• English Abstract MeSH
• Journal Article MeSH
• Case Reports MeSH

• Keywords
• POTASSIUM *, SCLERODERMA *,
• MeSH
• Potassium * MeSH
• Humans MeSH
• Scleroderma, Localized * MeSH
• Scleroderma, Systemic * MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Names of Substances
• Potassium * MeSH

RATIONALE & OBJECTIVE: Data for outcomes of patients with end-stage renal disease (ESRD) secondary to systemic sclerosis (scleroderma) requiring renal replacement therapy (RRT) are limited. We examined the incidence and prevalence of ESRD due to scleroderma in Europe and the outcomes among these patients following initiation of RRT. STUDY DESIGN: Registry study of incidence and prevalence and a matched cohort study of clinical outcomes. SETTING & PARTICIPANTS: Patients represented in any of 19 renal registries that provided data to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry between 2002 and 2013. PREDICTOR: Scleroderma as the identified cause of ESRD. OUTCOMES: Incidence and prevalence of ESRD from scleroderma. Recovery from RRT dependence, patient survival after ESRD, and graft survival after kidney transplantation. ANALYTICAL APPROACH: Incidence and prevalence were calculated using population data from the European Union and standardized to population characteristics in 2005. Patient and graft survival were compared with 2 age- and sex-matched control groups without scleroderma: (1) diabetes mellitus as the cause of ESRD and (2) conditions other than diabetes mellitus as the cause of ESRD. Survival analyses were performed using Kaplan-Meier analysis and Cox regression. RESULTS: 342 patients with scleroderma (0.14% of all incident RRT patients) were included. Between 2002 and 2013, the range of adjusted annual incidence and prevalence rates of RRT for ESRD due to scleroderma were 0.11 to 0.26 and 0.73 to 0.95 per million population, respectively. Recovery of independent kidney function was greatest in the scleroderma group (7.6% vs 0.7% in diabetes mellitus and 2.0% in other primary kidney diseases control group patients, both P<0.001), though time required to achieve recovery was longer. The 5-year survival probability from day 91 of RRT among patients with scleroderma was 38.9% (95% CI, 32.0%-45.8%), whereas 5-year posttransplantation patient survival and 5-year allograft survival were 88.2% (95% CI, 75.3%-94.6%) and 72.4% (95% CI, 55.0%-84.0%), respectively. Adjusted mortality from day 91 on RRT was higher among patients with scleroderma than observed in both control groups (HRs of 1.25 [95% CI, 1.05-1.48] and 2.00 [95% CI, 1.69-2.39]). In contrast, patient and graft survival after kidney transplantation did not differ between patients with scleroderma and control groups. LIMITATIONS: No data for extrarenal manifestations, treatment, or recurrence. CONCLUSIONS: Survival of patients with scleroderma who receive dialysis for more than 90 days was worse than for those with other causes of ESRD. Patient survival after transplantation was similar to that observed among patients with ESRD due to other conditions. Patients with scleroderma had a higher rate of recovery from RRT dependence than controls.

• Keywords
• Systemic sclerosis, dialysis, disease course, disease registry, end-stage renal disease (ESRD), incidence, kidney transplantation, mortality, outcomes, prognosis, renal recovery, renal replacement therapy (RRT), scleroderma,
• MeSH
• Survival Analysis MeSH
• Kidney Failure, Chronic etiology   mortality   therapy   MeSH
• Adult MeSH
• Risk Assessment MeSH
• Internationality MeSH
• Kaplan-Meier Estimate MeSH
• Middle Aged MeSH
• Humans MeSH
• Young Adult MeSH
• Renal Replacement Therapy methods   mortality   MeSH
• Predictive Value of Tests MeSH
• Cause of Death * MeSH
• Prognosis MeSH
• Proportional Hazards Models MeSH
• Registries * MeSH
• Retrospective Studies MeSH
• Aged MeSH
• Case-Control Studies MeSH
• Scleroderma, Systemic complications   diagnosis   therapy   MeSH
• Treatment Outcome MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Young Adult MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH
• Research Support, Non-U.S. Gov't MeSH
• Geographicals
• Europe MeSH

• Keywords
• HEART ANEURYSM *, HEART BLOCK *, HEART ENLARGEMENT *, HEART FAILURE, CONGESTIVE *, SCLERODERMA *,
• MeSH
• Heart Aneurysm * MeSH
• Cardiomegaly * MeSH
• Humans MeSH
• Heart Block * MeSH
• Heart Failure * MeSH
• Scleroderma, Systemic * MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH