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Int J Rheumatol. 2014;2014:716358. doi: 10.1155/2014/716358. Epub 2014 Nov 11.

Are There Clinical Differences in Limited Systemic Sclerosis according to Extension of Skin Involvement?.

International journal of rheumatology

Marina Scolnik, Luis J Catoggio, Eliana Lancioni, Mirtha R Sabelli, Carla M Saucedo, Josefina Marin, Enrique R Soriano

Affiliations

  1. Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina.
  2. Rheumatology Section, Medical Services, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina ; Fundación Dr. Pedro M. Catoggio para el Progreso de la Reumatologia, 1181 Buenos Aires, Argentina ; Instituto Universitario Escuela de Medicina, Hospital Italiano de Buenos Aires, 1181 Buenos Aires, Argentina.

PMID: 25435879 PMCID: PMC4243126 DOI: 10.1155/2014/716358

Abstract

Objectives. To examine the characteristics of our patients with limited systemic sclerosis (lSSc) for differences between Barnett Type 1 (sclerodactyly only) and Type 2 or intermediate (acrosclerosis-distal but may reach up to elbows and/or knees plus face) subsets. Methods. Records of patients between January 1, 2000, and December 31, 2011, with SSc or those with anti-Scl-70, anticentromere, or antinucleolar antibodies were reviewed. Only cases fulfilling ACR 1980 criteria were included and classified as diffuse or limited according to LeRoy's criteria. Limited SSc was separated into sclerodactyly and acrosclerosis (Barnett's Types 1 and 2). Results. 234 SSc patients (216 females) fulfilled criteria. Female/male ratio was 12 : 1; 24% had dSSc and 76% lSSC (64% Type 1 and 12% Type 2). Total follow-up was 688 patient-years. Within lSSC, the Type 2 group had significantly shorter duration of Raynaud's and more anti-Scl-70 and less anticentromere antibodies. In particular, interstitial lung disease (ILD) was significantly more prevalent in Type 2 group and similar to Type 3. Conclusions. These results appear to confirm that extension of skin involvement within limited SSc may identify two different subsets with clinical and serologic characteristics.

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