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OBJECTIVE To assess the safety and efficacy of rituximab in a randomized, double-blind, placebo-phase trial in adult and pediatric myositis patients. METHODS Adults with refractory polymyositis (PM) and adults and children with refractory dermatomyositis (DM) were enrolled. Entry criteria included muscle weakness and ≥2 additional abnormal values on core(More)
OBJECTIVE To determine the long-term outcome and associated clinical, serologic, and pathologic features in a cohort of patients with connective tissue disease (CTD) and the anti-signal recognition particle (anti-SRP) autoantibody. METHODS Sera and clinical data were collected prospectively from consecutive adult patients with polymyositis (PM; n = 134),(More)
OBJECTIVE To assess the efficacy of tacrolimus in patients with anti-aminoacyl-transfer RNA synthetase (anti-aaRS)-associated interstitial lung disease (ILD) and idiopathic inflammatory myopathy (IIM). METHODS Ninety-eight patients with anti-aaRS autoantibodies were identified in our IIM cohort of 536 patients. The medical records of 15 patients with(More)
OBJECTIVE Pulmonary arterial hypertension (PAH) and severe pulmonary fibrosis (SPF) are the most common causes of death in scleroderma. Our study focuses on lung disease in patients with a nucleolar antibody in comparison to other scleroderma-specific autoantibodies. METHODS Patients initially seen between 1972 and 1995 (and followed through 2004) with(More)
OBJECTIVE Epidemiology studies suggest that systemic sclerosis (SSc) is more common, occurs at a younger age, and is more severe in African Americans than Caucasians. However, the scleroderma autoantibody profile is very different between these 2 ethnic groups. This study was undertaken to examine the demographic and disease features, frequency and severity(More)
OBJECTIVE To describe the demographic, clinical, and laboratory features and natural history of patients with systemic sclerosis sine scleroderma (ssSSc), and to compare them with those of patients with SSc and limited cutaneous involvement (IcSSc). METHODS The University of Pittsburgh Scleroderma Databank served as the data source. Patients were divided(More)
OBJECTIVE To compare the cumulative survival and event free survival in patients with Jo-1 versus non-Jo-1 anti-tRNA synthetase autoantibodies (anti-synAb). METHODS Anti-synAb positive patients initially evaluated from 1985 to 2009 were included regardless of the connective tissue disease (CTD) diagnosis. Clinical data were extracted from a prospectively(More)
OBJECTIVE To determine the clinical and immunogenetic features of systemic sclerosis (SSc) patients with anti-RNA polymerase (RNAP) or anti-fibrillarin antibodies. METHODS DNA typing for HLA-DR and DQ alleles was performed in 292 patients with SSc, including 81 with anti-RNAP and 24 with anti-fibrillarin antibodies. The remaining patients had(More)
Evidence implicating histidyl-tRNA synthetase (Jo-1) in the pathogenesis of the anti-synthetase syndrome includes established genetic associations linking the reproducible phenotype of muscle inflammation and interstitial lung disease with autoantibodies recognizing Jo-1. To better address the role of Jo-1-directed B and T cell responses in the context of(More)
OBJECTIVE To characterize a new serum autoantibody in patients with systemic sclerosis (SSc) directed against U11/U12 RNP and to identify the clinical features associated with this autoantibody. METHODS We identified autoantibodies directed against the U11/U12 RNP complex in sera of patients with SSc and confirmed antibody specificity by(More)