Rheumatology International

, 29:861

The many faces of scleroderma sine scleroderma: a literature review focusing on cardiopulmonary complications

Authors

    • Department of Pathophysiology, School of Medicine, Laiko University HospitalUniversity of Athens
    • Department of Pharmacology, Center for Lung and Vascular BiologyUniversity of Illinois at Chicago Medical School
  • George E. Tzelepis
    • Department of Pathophysiology, School of Medicine, Laiko University HospitalUniversity of Athens
Review Article

DOI: 10.1007/s00296-009-0878-7

Cite this article as:
Toya, S.P. & Tzelepis, G.E. Rheumatol Int (2009) 29: 861. doi:10.1007/s00296-009-0878-7

Abstract

Scleroderma sine scleroderma (ssSSc) is an occult form of systemic sclerosis that may cause diagnostic difficulties due to the absence of skin involvement. Delays in the diagnosis of ssSSc means lost opportunites to address and treat the often lethal involvement of internal organs such as the lungs and heart. In this systemic review we collected all published cases of ssSSc using EMBASE, MEDLINE, PubMed, and Web of Science from 1950 to present. Our purpose was to describe the range and frequency of the clinical manifestations of ssSSc. A total of 108 published cases of ssSSc were analyzed. Lung involvement was present in 66% of cases. Peripheral vascular system involvement was present in all patients whereas gastrointestinal manifestations were present in 82% of the cases. Overall the clinical presentation is subtle and heightened clinical awareness is required to facilitate prompt recognition and treatment.

Keywords

Scleroderma sine scleroderma Interstitial lung disease Raynaud’s Autoantibodies

Copyright information

© Springer-Verlag 2009