©2014 Turkish League Against Rheumatism. All rights reserved. Juvenile systemic scleroderma is a rare chronic multi-system connective tissue disease in childhood. Although rare in children, it is an important cause of morbidity and mortality. Juvenile systemic scleroderma is one of the most severe rheumatologic conditions diagnosed in children. In this article, we report a six-year-old boy diag...

Dobrota et al have analysed the EUSTAR (EULAR Scleroderma Trials and Research) systemic sclerosis (SSc, scleroderma) database using a subset of diffuse cutaneous SSc (dcSSc) to determine predictors of skin improvement over 1 year in randomised trials. The idea is to enrol more informative patients in a randomised controlled trial (RCT), as incident dcSSc is rare. However, you could want patient...

OBJECTIVE Patients with systemic rheumatic disease constitute a small percentage of admissions to the medical intensive care units (ICUs). Systemic sclerosis (SSc) is one of the rheumatic diseases that together with secondary complications may lead to a critical illness requiring hospitalization in the ICU. We present the features, clinical course and outcome of critically ill patients with scl...

Systemic sclerosis (SSc) is characterized by vascular abnormalities, fi brosis, infl ammatory changes, and late stage tissue atrophy of the skin and several internal organs. In scleroderma-like disorders the distribution/characteristics of skin involvement is different. The skin involvement of the acral regions including digits is usually missing. Lack of Raynaud’s phenomenon, and scleroderma-s...

OBJECTIVES The aim of this study was to evaluate human leukocyte antigen (HLA) involvement in the disease expression and poor prognostic clinical features (pulmonary fibrosis and pulmonary arterial hypertension) in patients diagnosed with systemic sclerosis (SSc) in a multiethnic population. METHODS SSc patients followed up between 2008 and 2011 were included, and clinical data were obtained ...

BACKGROUND The complement system has been implicated in pathogenesis of systemic sclerosis (SSc). The goal of the present study was to evaluate improved complement biomarkers in SSc. METHODS The presence of C4d, reflecting activation of the classical/lectin pathways, C3bBbP corresponding to activation of the alternative pathway, and soluble terminal complement complexes (all complement pathwa...

Accepted 19 April 1995 A 31-year-old black woman, with a nine-year history of systemic sclerosis, was admitted with an episode of jerking movements of both upper extremities, followed by unresponsiveness. In the emergency room, she had an episode of generalised tonic-clonic seizures. Her husband stated that she had never had seizures prior to these two episodes and had no headaches, nausea, vom...

Anti-NOR90 antibodies are usually found in patients with systemic sclerosis (SSc); however, their clinical relevance remains obscure. We previously developed an enzyme-linked immunosorbent assay (ELISA) for measuring them to investigate the features of anti-NOR90 antibodies. As we additionally obtained antibody-positive serum samples after our previous research, reexamined significance Serum fr...

BACKGROUND Previous reports have suggested that treatment with the selective estrogen antagonist tamoxifen may be effective in diminishing primary and secondary Raynaud's vasospasm, including cases occurring in the setting of scleroderma. Tamoxifen treatment has also been associated with improvement of retroperitoneal fibrosis and desmoid tumors, conditions also associated with abnormal fibrobl...

BACKGROUND Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare. OBJECTIVE To investigate the expression of periostin and other molecules in localized scleroderma. METHODS A retrospective study of 14 pati...