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Long‐term outcome of patients with polymyositis/ dermatomyositis and anti‐PM‐Scl antibody
Author(s) -
Marie I.,
Lahaxe L.,
Benveniste O.,
Delavigne K.,
Adoue D.,
Mouthon L.,
Hachulla E.,
Constans J.,
Tiev K.,
Diot E.,
Levesque H.,
Boyer O.,
Jouen F.
Publication year - 2010
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2009.09484.x
Subject(s) - medicine , dermatomyositis , polymyositis , antisynthetase syndrome , gastroenterology , interstitial lung disease , discontinuation , antibody , cohort , myositis , surgery , immunology , lung
Summary Background To date, no series has analysed long‐term outcome in patients with polymyositis/dermatomyositis (PM/DM) with anti‐PM‐Scl antibody. Objectives The aims of the present study were: (i) to assess clinical features and long‐term outcome, including organ complications, functional course and mortality rate, in patients with isolated PM/DM with anti‐PM‐Scl antibody; and (ii) to evaluate prevalence, characteristics and long‐term outcome of interstitial lung disease (ILD) in patients with isolated PM/DM with anti‐PM‐Scl antibody. Methods The medical records of 20 consecutive patients with isolated PM/DM with anti‐PM‐Scl antibody were reviewed. Results Two patients (10%) achieved remission of PM/DM, whereas 14 (70%) improved and four (20%) had a worsened clinical status. Short‐term recurrences (during tapering of therapy) occurred in nine patients and long‐term recurrences (after discontinuation of therapy) in three patients. Moreover, patients with PM/DM with anti‐PM‐Scl antibody exhibited severe complications, as follows: oesophageal involvement ( n = 4) requiring enteral feeding in three cases, ventilatory insufficiency ( n = 3) requiring mechanical ventilation in two cases; three other patients had cancer. Interestingly, patients with PM/DM with anti‐PM‐Scl antibody often presented symptoms that are usually found in antisynthetase syndrome, i.e. hyperkeratotic rhagadiform hand symptoms ( n = 2; 10%), Raynaud’s phenomenon ( n = 8; 40%), arthralgia/arthritis ( n = 7; 35%) and ILD ( n = 12; 60%). In our cohort, the associated ILD often required combined therapy of steroids and immunosuppressive agents. Conclusions Our series suggests that the presence of anti‐PM‐Scl antibody is not a good prognostic factor in patients with PM/DM, as there appears to be an association with lung and oesophageal involvement; in addition, anti‐PM‐Scl antibody may coexist with malignancy in patients with PM/DM. Furthermore, anti‐PM‐Scl antibody‐positive patients with PM/DM often exhibit ‘mechanic’s hands’, Raynaud’s phenomenon and joint involvement. Our latter findings raise the possibility that the immunogenetic background influences the autoantibody status of these patients; HLA‐DR3 has, in fact, been found in association with antisynthetase syndrome antibodies and with anti‐PM‐Scl antibodies.