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

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


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.


Scleroderma sine scleroderma Interstitial lung disease Raynaud’s Autoantibodies 

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  1. 1.Department of Pathophysiology, School of Medicine, Laiko University HospitalUniversity of AthensAthensGreece
  2. 2.Department of Pharmacology, Center for Lung and Vascular BiologyUniversity of Illinois at Chicago Medical SchoolChicagoILUSA

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