Microstomia is associated with functional impairment and is a poor prognostic factor in systemic sclerosis - a single center observational study with survival analysis.

Background: Objectives were to assess the mouth opening ability (MOA) among patients with systemic sclerosis (SSc) in comparison to a healthy control population. The impact of microstomia (decrease in any of the MOA parameters) on physical performance and long-term survival was also investigated.

Methods: Interincisal (ID), interlabial (LD), the intercommissural distances at both opened mouth and closed mouth (OW, CW) and the oral area (OA) and circumference (OC) all were assessed in 131 SSc patients.

Diabetes mellitus-related musculoskeletal disorders: Unveiling the cluster of diseases.

The current study ushers in a comprehensive review in clinical research to demonstrate the prevalence of musculoskeletal (MSK) complications in diabetes mellitus and the most relevant clinical aspects. In particular, revealing the early symptoms of the disorders, the pathology lurking behind the complications and their optimal management. In diabetes mellitus, MSK complications are common and are largely due to similar pathogenetic factors responsible for the internal organ complications associated with diabetes leading to chronic low-intensity inflammatory processes.

Characteristics of ScleroID highlighting musculoskeletal and internal organ implications in patients afflicted with systemic sclerosis.

Background: Systemic sclerosis (SSc) is a multi-organ disease with impaired health-related quality of life (HRQoL). The EULAR SSc Impact of Disease (ScleroID) is a newly introduced SSc-specific patient-reported outcome to evaluate HRQoL in SSc.

Objective: To investigate the correlation between the ScleroID and the involvement of organ systems as well as disease activity/damage in a SSc cohort from a large tertiary care centre.

Multi-Organ System Screening, Care, and Patient Support in Systemic Sclerosis.

Systemic sclerosis (SSc) is a heterogenous systemic autoimmune disease of complex multi-organ manifestations with a disease-specific mortality of >50%. The patient journey is fraught with severe, diverse, and diffuse physical impairment, psychological burden, and diminishing health-related quality of life. SSc remains unfamiliar to many clinicians.

Minimal Clinically Important Differences (MCID) for the Functional Assessment of Chronic Illness Therapy Fatigue Scale in Patients with Systemic Sclerosis.

(1) Background: Systemic sclerosis (SSc) is characterized by significant fatigue, causing diminished quality of life (QoL). The aim of this study was to examine fatigue levels and their associations with clinical factors and determine the minimal clinically important difference (MCID) value for the Functional Assessment of Chronic Illness Therapy Fatigue Scale (FACIT-FS). (2) Methods: A total of 160 SSc patients and 62 individuals without SSc were followed-up over a 12-month period by measuring the FACIT-FS and the Visual Analogue Scale and the Short Form 36 Vitality Score analyzing changes in exhaustion.

World Health Organization (WHO) International Classification of Functioning, Disability and Health (ICF) Core Set Development for Interstitial Lung Disease.

The World Health Organization (WHO) introduced the International Classification of Functioning, Disability, and Health (ICF) as a scientific method of disability data collection comprised of >1,200 categories describing the spectrum of impairment types (functional, symptoms-based and anatomical) under the bio-psycho-social model with consideration of and (pf). ICF Core Sets and ICF Checklists are streamlined disease-specific resources for clinical use, service provision, and for use in health economics and health policy. ICF can disclose strengths and weaknesses across multiple patient-reported outcome measures (PROMs) and help consolidate best-fitting question-items from multiple PROMs.

A comprehensive framework for navigating patient care in systemic sclerosis: A global response to the need for improving the practice of diagnostic and preventive strategies in SSc.

Systemic sclerosis (SSc), the most lethal of rheumatologic conditions, is the cause of death in >50% of SSc cases, led by pulmonary fibrosis followed by pulmonary hypertension and then scleroderma renal crisis (SRC). Multiple other preventable and treatable SSc-related vascular, cardiac, gastrointestinal, nutritional and musculoskeletal complications can lead to disability and death. Vascular injury with subsequent inflammation transforming to irreversible fibrosis and permanent damage characterizes SSc.

Exercise as a multi-modal disease-modifying medicine in systemic sclerosis: An introduction by The Global Fellowship on Rehabilitation and Exercise in Systemic Sclerosis (G-FoRSS).

Systemic sclerosis (SSc) is a heterogeneous multisystem autoimmune disease whereby its main pathological drivers of disability and damage are vascular injury, inflammatory cell infiltration, and fibrosis. These mechanisms result in diffuse and diverse impairments arising from ischemic circulatory dysfunction leading to painful skin ulceration and calcinosis, neurovascular aberrations hindering gastrointestinal (GI) motility, progressive painful, incapacitating or immobilizing effects of inflammatory and fibrotic effects on the lungs, skin, articular and periarticular structures, and muscle. SSc-related impairments impede routine activities of daily living (ADLs) and disrupt three critical life areas: work, family, social/leisure, and also impact on psychological well-being.

Screening for the presence of scleroedema adultorum of Buschke in patients with diabetes mellitus: newly diagnosed patients had a high prevalence of dyslipidaemia.

Background: Scleroedema adultorum of Buschke is a rare disorder characterized by fibromucinous thickening of the dermis that manifests mainly at the nape of the neck and on the upper back and shoulders. This study screened patients with diabetes mellitus for skin hardening caused by scleroedema adultorum of Buschke and characterized the clinical and laboratory findings in patients with newly identified cases, with a focus on lipid metabolism abnormalities and vascular complications.

Methods: Out of 113 consecutive patients with diabetes, 11 (9.

Sensitivity to change of joint count composite indices in 72 patients with systemic sclerosis.

Objectives: We validated the responsiveness of joint count composite indices (JCCIs) in 72 patients with systemic sclerosis (SSc).

Methods: Changes in Disease Activity Score of 28 Joints using ESR and CRP (DAS28-ESR, DAS28-CRP), Simplified Disease Activity Index (SDAI) and the Clinical Disease Activity Index (CDAI) were evaluated in a one-year follow-up study. Charts of patients including swollen/tender joint counts, laboratory signs of inflammation, and visual analogue scales referring to disease activity, severity and pain were also blindly categorized by two rheumatologists as improved, unchanged or deteriorated.

Evaluation of osteoarthritis knee and hip quality of life (OAKHQoL): adaptation and validation of the questionnaire in the Hungarian population.

Background: At least 17% of the population suffers from osteoarthritis (OA) in Hungary, according to the European Health Interview Survey. In Hungary, until now there was no OA-specific questionnaire available for the lower limb, in order to monitor the health-related quality of life (HRQoL). This gap gave the relevance of this research.

Sclerodermalike syndromes: Great imitators.

Sclerodermalike syndromes (SLSs) comprise diseases with mucin deposition (eg, scleromyxedema, scleredema), with eosinophilia (eg, eosinophilic fasciitis), metabolic or biochemical abnormalities (eg, nephrogenic systemic fibrosis), or endocrine disorders (eg, POEMS syndrome, or polyneuropathy, organomegaly, endocrinopathy, monoclonal lymphoproliferative disorder, and hypothyroidism). Chronic graft-versus-host disease may also show sclerodermalike skin changes. Inherited progeria syndromes with early aging (eg, Werner syndrome) and a heterogeneous group of hereditary disorders with either skin thickening (eg, stiff skin syndrome) or atrophy and tightening (eg, acrogeria) can also imitate classic systemic sclerosis (SSc).

Efficacy of intensive hand physical therapy in patients with systemic sclerosis.

Objectives: To evaluate the efficacy of a three-week period of complex and intensive hand physical therapy on hand function in patients with systemic sclerosis (SSc).

Methods: Thirty-one patients with SSc were treated. Hand stretching exercises, ergotherapy supplemented with thermal and mud baths, whirlpool therapy and soft tissue massage were daily used during a three-week period.

The presence of small joint contractures is a risk factor for survival in 439 patients with systemic sclerosis.

Objectives: Analysis of risk factors and mortality of 439 patients with systemic sclerosis (SSc) in a tertiary care centre.

Methods: The mean follow up time was 8.4±5.

Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening.

Objective: To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients.

Methods: (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3- controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset.

Validation of disease activity indices using the 28 joint counts in systemic sclerosis.

Objectives: To validate the Disease Activity Score 28 using ESR (DAS28-ESR) and CRP (DAS28-CRP), the Simplified Disease Activity Index and the Clinical Disease Activity Index used in RA for SSc patients.

Methods: Seventy-seven SSc patients, 40 RA patients, 20 patients with primary RP (PRP) and 28 healthy volunteers were assessed. Besides the disease activity composite indices, the European Scleroderma Study Group Activity Index (EScSG-AI), the HAQ-DI, the Cochin Hand Function Scale and the Short Form Health Survey (SF36) were evaluated.

An observational cohort study of patients with newly diagnosed digital ulcer disease secondary to systemic sclerosis registered in the EUSTAR database.

Objectives: This study describes clinical characteristics, prognostic factors, and quality of life in patients with newly diagnosed (incident) digital ulcers (DU).

Methods: Observational cohort study of 189 consecutive SSc patients with incident DU diagnosis identified from the EUSTAR database (22 centres in 10 countries). Data were collected from medical charts and during one prospective visit between 01/2004 and 09/2010.

Clinical usefulness of measuring red blood cell distribution width in patients with systemic sclerosis.

Objective: Red blood cell distribution width (RDW) is a biomarker quantifying the variability of red blood cell size in peripheral blood. Elevated RDW has been found to be an independent prognostic factor for cardiovascular events. SSc is characterized by generalized micro- and macroangiopathy.

International cohort study of 73 anti-Ku-positive patients: association of p70/p80 anti-Ku antibodies with joint/bone features and differentiation of disease populations by using principal-components analysis.

Introduction: An international cohort study of 73 anti-Ku-positive patients with different connective tissue diseases was conducted to differentiate the anti-Ku-positive populations of patients based on their autoantibody profile and clinical signs/symptoms and to establish possible correlations between antibodies against Ku p70 and Ku p80 with autoimmune diseases.

Methods: Sera of anti-Ku-positive patients were collected from six European centers and were all secondarily tested (in the reference center); 73 were confirmed as positive. Anti-Ku antibodies were detected with counter-immunoelectrophoresis (CIE), line immunoassay (LIA), and immunoblot analyses.

Repeated teaching courses of the modified Rodnan skin score in systemic sclerosis.

Objectives: To evaluate the efficacy of a repeated teaching process of the modified Rodnan skin score (MRSS) in systemic sclerosis (SSc). The question is whether a repeated teaching course is required to maintain good results after a first, successful teaching process.

Methods: Two consecutive teaching courses were organised by two Romanian EUSTAR centres for the same rheumatologists, to evaluate and compare the inter-observer variability.

Construct validity evaluation of the European Scleroderma Study Group activity index, and investigation of possible new disease activity markers in systemic sclerosis.

Objectives: To evaluate the construct validity of the European Scleroderma Study Group (EScSG) activity index and to propose modifications if necessary.

Methods: One hundred and thirty-one consecutive patients were investigated and re-evaluated 1 year later. Modified Rodnan skin score (MRSS), skin ulcers and joint contracture numbers, hand anatomic index (HAI), BMI, spirometry, carbon monoxide diffusing capacity (DL(CO)), left ventricular ejection fraction, pulmonary arterial hypertension, HAQ Disability Index (HAQ-DI), patient skin self-assessment questionnaire and several biomarkers were recorded, in addition to the data required for the EScSG activity index.

Establishment and partial validation of a patient skin self-assessment questionnaire in systemic sclerosis.

Objectives: To construct a patient self-assessment questionnaire testing whether patients can provide valid information about their skin thickness, tethering and thinness.

Methods: The questionnaire contained questions about the 17 body areas identical to those investigated in the Modified Rodnan Skin Score (MRSS). The baseline and 1 yr follow-up values of thickening, tethering and thinning were scored on a scale of 0-3, by 131 consecutive patients.

Prevalence of rheumatoid arthritis in the South-Transdanubian region of Hungary based on a representative survey of 10,000 inhabitants.

Objective: To assess the prevalence of rheumatoid arthritis (RA) in a representative study of the South Transdanubian region of Hungary.

Methods: Ten thousand individuals aged between 14-65 years were interviewed. The stratified sample was representative for age, sex and urban/rural residence structure of the regional population of the South-West Hungarian region.