Background There is substantial difficulty in the diagnosis of progressive systemic sclerosis where visceral involvement precedes cutaneous expression of the disease.

Objectives To describe clues to the diagnosis of progressive systemic sclerosis sine scleroderma (ssSSc).

Methods Report of 3 cases and review of the MEDLINE database concerning ssSSc.

Results Tweny five patients from the literature and 3 from our unit fulfilled the inclusion criteria, 13 females and 15 males, mean age 51.2(SD17.2) years. Conditions motivating the search for ssSSc were pulmonary fibrosis (44%), congestive heart failure (19%), esophageal dysfunction (7%), malabsorption (11%), nephropathy (11%), fever of unknown origin (3.5%) and seizures (3.5%). An overt undifferentiated connective tissue disease was present in 7 pts. Multisystem involvement became apparent when carefully searched for in all but one patient. ANA and RF were found in 13 of 17 and “scleroderma pattern” was noted in 9 of 13 patients on nailfold capillaroscopy. Taut skin developed later in 57% of pts following extracutaneous presentation by 1 month to 7 yrs (mean 2.3 yrs).

Conclusion Clues to the diagnosis of ssSSc can be classified as: 1) background conditions – unclassified system disorder, Raynaud’s, polyarthritis 2) evolving visceropathy of obscure provenience – dysphagia, malabsoption, pulmonary fibrosis, heart failure, nephropathy 3) corroborative laboratory and clinical features – hyper/hypopigmentation, telangiectasis, calcinosis, typical nailfold capillaroscopy 4) confirmatory evidence – visceral biopsy, appearance of taut skin.