Anti-pm/scl antibodies in connective tissue disease: Clinical and biological assessment of 14 patients

F. Vandergheynst, A. Ocmant, C. Sordet, R.L. Humbel, J. Goetz, F. Roufosse,E. Cogan, J. Sibilia

Internal Medicine Department, C.H.U. Erasme (Université Libre de Bruxelles), Bruxelles, Belgium

2006 Vol.24, N°2 - PI 0129, PF 0133
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Rheumatology Article



INTRODUCTION:Anti-PM/Scl antibodies (Anti-PM/Scl) represent a rarely encountered type of antinuclear antibodies. They have mainly been reported in association with idiopathic inflammatory myositis – systemic sclerosis overlap syndromes (also called scleromyositis or sclerodermatomyositis) but also with polymyositis, dermatomyositis and systemic sclerosis without features of overlap syndromes. Studies concerning characteristics of patients with anti-PM/SCl are rare and include small numbers of patients.PATIENTS AND
Retrospective review of clinical and biological characteristics of 14 patients with anti-PM/Scl in two University Hospitals: one in Belgium (Erasme Hospital, Bruxelles) and one in France (Hautepierre Hospital, Strasbourg).
Seven patients were identified in Erasme and 7 in Strasbourg: 5 with systemic sclerosis – (dermato)myositis overlap syndromes, 4 with dermatomyositis, 1 with polymyositis, 3 with systemic sclerosis, 1 with primary Sjögren`s syndrome. The most frequently observed clinical characteristics (85% of patients) were: pulmonary interstitial disease and arthralgia or arthritis. No patient of our series died or developed cancer (mean follow-up:6.1 years).
Our study failed to identify an homogeneous clinical pattern in patients with anti-PM/Scl, except for 2 characteristics shared by 85% of the patients. This lack of homogeneity is in agreement with preceding literature. We confirm the favourable prognosis associated with the presence of anti-PM/Scl, despite the high incidence of interstitial pulmonary disease. The absence of cancer associated with presence of anti-PM/Scl represents a partial explanation. Finally, we report herein the second case of primary Sjögren`s syndrome associated with anti-PM/Scl.

PMID: 16762146 [PubMed]