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


METHODS
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).


RESULTS
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).


CONCLUSIONS
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.
Vandergheynst
F
F
Internal Medicine Department, C.H.U. Erasme (Université Libre de Bruxelles), Bruxelles, Belgium. fvdgheyn@ulb.ac.be
Ocmant
A
A
Sordet
C
C
Humbel
R L
RL
Goetz
J
J
Roufosse
F
F
Cogan
E
E
Sibilia
J
J
D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood*; D018771:Arthralgia / Q000276:immunology; D001168:Arthritis / Q000276:immunology; D005260:Female; D006785:Hospitals, University; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000276:immunology; D008297:Male; D008875:Middle Aged; D017285:Polymyositis / Q000276:immunology*; D011379:Prognosis; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000276:immunology*
D000974:Antibodies, Antinuclear