Anticentromere antibody positive Sjögren's Syndrome: a retrospective descriptive analysis.

INTRODUCTION: A subgroup of patients with primary Sjögren's Syndrome (SS) and positive anticentromere antibodies (ACA) were recognized as having features intermediate between SS and systemic sclerosis (SSc). Our goal was to describe this group clinically and serologically and define its tendency to evolve to full blown SSc. METHODS: Among 535 patients with primary SS we identified 20 ACA positive (ACA+/SS). We compared them to 61 randomly selected ACA negative SS patients (ACA-/SS), 31 ACA positive SSc patients with sicca manifestations [SSc/(+) sicca] and 20 ACA positive SSc patients without sicca manifestations [SSc/(-) sicca]. RESULTS: Prevalence of ACA among SS patients was 3.7%. Cases and controls did not differ in sex ratio and age at disease onset. ACA+/SS patients had a lower prevalence of dry eyes, hypergammaglobulinaemia, anti-Ro and anti-La antibodies and a higher prevalence of Raynaud's phenomenon and dysphagia compared to ACA-/SS patients. They also had lower prevalence of telangiectasias, puffy fingers, sclerodactyly, Raynaud's phenomenon, digital ulcers and gastroesophageal reflux in comparison to both of the SSc subgroups and a lower prevalence of dyspnoea and lung fibrosis compared to the SSc/(+) sicca subgroup. Two patients originally having ACA+/SS evolved to full blown SSc. Four deaths occurred, all among SSc patients. Kaplan Meier analysis showed a significant difference between cases and controls in time from disease onset to development of gastroesophageal reflux, telangiectasias, digital ulcers, arthritis, puffy fingers, xerostomia, hypergammaglobulinaemia and dysphagia. CONCLUSIONS: ACA+/SS has a clinical phenotype intermediate between ACA-/SS and SSc and shows little tendency to evolve to SSc.

Recent advances in the treatment of systemic sclerosis.

Systemic sclerosis (SSc) is a chronic autoimmune disease with clinical manifestations resulting from immune activation, fibrosis development, and damage of small blood vessels. Our aim was to critically illustrate the available data on the new treatments proposed for SSc to provide a clinically oriented overview of the current evidence. PubMed was used for literature search using "scleroderma" and "therapy" to identify all articles published on indexed journals between 1972 and 2008. The search was limited to publications in English and produced a total of 3,441 references, which included 735 review articles. These citations were then screened for articles dealing with the most recent therapy options for SSc, and 214 articles were selected for evaluation and discussion. Methotrexate, cyclophosphamide, calcium channel blockers, angiotensin converting enzyme inhibitors, prostacyclin analogues, D-penicillamine, and extracorporeal photopheresis are the most widely studied treatments for SSc and were considered as practiced treatments. Other therapeutic approaches have been developed more recently and include endothelin receptor antagonists and phosphodiesterase-5 inhibitors for pulmonary arterial hypertension and peripheral vascular disease. High-dose immunosuppression and stem cell transplantation constitute a promising treatment and data from randomized controlled trials are awaited. Intravenous gamma globulins, mycophenolate mophetil, collagen tolerance induction, rituximab, fluoxetine, pirfenidone, relaxin, halofuginone, anti-TGF-beta antibodies, and tyrosine kinase inhibitors awaits more solid data. The clinical management of patients with SSc remains a challenge and currently involves practiced and newly proposed therapeutic approaches. The disease pleiomorphism poses numerous difficulties to determine ideal outcomes to be used in clinical trials.