Undifferentiated connective tissue disease: a seven-center cross-sectional study of 184 patients

Abstract

The purpose of this study was to characterize the clinical and serological features of a large cohort of patients with antinuclear antibody (ANA) positive undifferentiated connective tissue disease (UCTD). Consecutive patients with UCTD, followed up at the Rheumatology Clinic of the participating centers, were included. Data from these patients were obtained by clinical evaluation and chart review. All patients were diagnosed as having UCTD on basis of the following criteria: positive ANA plus at least one clinical feature of connective tissue disease, but not fulfilling classification criteria for any differentiated connective tissue disease. One hundred eighty-four patients were studied (female patients-94.5%; mean age at time of evaluation-47 years). The most prevalent manifestations were arthralgia (66%), arthritis (32%), Raynaud's phenomenon (30%), sicca symptoms (30%), and leukopenia (19%). The prevalence of ANA was 100%, anti-SSA 20%, antidsDNA 14%, and anti-SSB 7%. Patients with anti-dsDNA/ anti-Sm, anticentromere/anti-Scl70, or anti-SSA/anti-SSB antibodies more frequently presented a set of manifestations close to systemic lupus erythematosus (SLE), systemic sclerosis, or Sjögren syndrome, respectively. We analyze a large cohort of UCTD. Seventy-two percent of these UCTD patients present lupus-, scleroderma-, or Sjögren-like features but do not fulfill classification criteria and mostly present a mild disease.

The status of serum vitamin D in patients with rheumatoid arthritis and undifferentiated inflammatory arthritis compared with controls

“…The presence of these antibodies in UIA may be a marker of B-cell activation and progression to early RA [22]. The ability of autoantibodies such as anti-cyclic-citrullinated antibody, antinuclear antibodies, and rheumatoid factor in predicting the outcome of patients with UIA was shown in a number of previous studies [23][24][25][26][27]. A positive results for anti-CCP and RF in patients with UIA could be a useful indicator of the presence of future RA [23,24,26].…”

Autoimmune-Featured Interstitial Lung Disease

“…[6][7][8] In contrast, our AIF-ILD cohort is predominantly male (60%) and older (66 years old) and, by defi nition, has ILD. Because of the disparity in these characteristics, we opted to propose a new term, AIF-ILD, to describe patients with ILD with autoimmune features.…”

“…Currently, these patients are considered to have an idiopathic interstitial pneumonia, although it has been suggested that these patients may have an undifferentiated connective tissue disease (UCTD). 5 However, UCTD as described in the rheumatology literature is mild in nature [6][7][8] and has a low prevalence of ILD (1%). 8 With these considerations, we propose a new term to describe a subset of pulmonary ILD patients with features of a connective tissue disease that do not meet ACR criteria: autoimmune-featured ILD (AIF-ILD).…”

Interstitial Pneumonia With Autoimmune Features: Overview of Proposed Criteria and Recent Cohort Characterization

“…14 Those meeting proposed UCTD criteria were more likely to have surgical lung biopsy (SLB)-proven NSIP, supporting the hypothesis that NSIP was an autoimmune phenomenon and that UCTD-ILD was a pulmonary manifestation of CTD. Because UCTD, as defined by rheumatologists, describes patients with a milder disease course that is not frequently complicated by ILD 20 , Fischer and colleagues suggested that “lung-dominant” CTD (LD-CTD) was more appropriate nomenclature. 17 Criteria for LD-CTD included the combination of ILD, failure to meet definitive criteria for a CTD, no alternative etiology for ILD and one autoantibody or two histopathologic features suggestive of CTD.…”