Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features

Rheumatology (Oxford). 2011 Feb;50(2):317-23. doi: 10.1093/rheumatology/keq176. Epub 2010 Jun 18.

Abstract

Objective: To assess internal organ involvement in early SSc at presentation.

Methods: One hundred and fifteen patients admitted to a tertiary centre because of RP, who did not present any routinely detectable scleroderma-related internal organ involvement, were investigated for ANA and videocapillaroscopy, and underwent history and physical examination to detect symptoms/signs suggestive of SSc. Patients were then subdivided into three groups: (i) early SSc, constituted by patients without clinical manifestations other than RP, but with scleroderma marker autoantibodies and/or typical capillaroscopic abnormalities; (ii) probable SSc, constituted by patients with the same autoantibody and/or capillaroscopic status as early SSc patients, but with any of the following manifestations: digital ulcers/scars, puffy fingers, arthritis, telangiectasia, dysphagia/heartburn, shortness of breath; (iii) UCTD, constituted by patients with a specific (i.e. disease antibody marker) ANA and capillaroscopic findings plus any disease manifestation. All patients were investigated by lung functional study and B-mode echo-Doppler-cardiography. Patients who consented underwent oesophageal manometry.

Results: An inverted mitral E : A ratio (i.e. early scleroderma cardiac involvement) and/or a diffusing lung capacity for CO <80% of the predictive value (i.e. early lung involvement) and/or basal low oesophageal sphincter pressure <15 mmHg (i.e. early oesophageal involvement) were detected in 37/51 probable SSc patients (72%), 8/19 early SSc patients (42%) and 12/45 UCTD patients (27%).

Conclusion: A scleroderma-related internal organ involvement was detected in patients from each group and, more importantly, was pre-clinical in a number of cases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Disease Progression
  • Early Diagnosis
  • Female
  • Gastrointestinal Diseases / physiopathology
  • Heart Diseases / physiopathology
  • Humans
  • Kidney Diseases / physiopathology
  • Lung Diseases / physiopathology
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Raynaud Disease / complications*
  • Raynaud Disease / physiopathology
  • Scleroderma, Systemic / physiopathology*
  • Time Factors
  • Young Adult