Distinctive features of idiopathic inflammatory myopathies inFrench Canadians
Identifieur interne : 002A46 ( Main/Exploration ); précédent : 002A45; suivant : 002A47Distinctive features of idiopathic inflammatory myopathies inFrench Canadians
Auteurs : Imad Uthman [Canada, États-Unis] ; Dolores Vázquez-Abad [Canada, États-Unis] ; Jean-Luc Senécal [Canada, États-Unis]Source :
- Seminars in Arthritis and Rheumatism [ 0049-0172 ] ; 1996.
English descriptors
- KwdEn :
- Teeft :
- Abnormality, Adult onset, Adult patients, Arthritis, Arthritis rheum, Articular complaints, Autoantibody, Autoantibody specificities, Biopsy, Clin, Clin immunol, Clinical features, Common skin lesions, Computerassisted analysis, Connective tissue disease, Creatine kinase, Cumulative survival rates, Current study, Dermatomyositis, Disease course, Dysphagia, Dyspnea, Educational levels, Esophageal carcinoma, Exact test, First report, French canadian, French canadian patients, French canadians, Good response, Heliotrope rash, Humoral immunity, Idiopathic, Inflammatory myopathies, Initial evaluation, Interstitial lung disease, Laboratory abnormalities, Laboratory features, Larger group, Lower dysphagia, Malignancy, Malignant disease, Median, Median duration, Muscle biopsy, Myopathy, Myositis, Nailfold capillary microscopy, Nuclear pattern, Nuclear pore, Nuclear pore complexes, Other features, Other series, Polymyositis, Pore, Prednisone, Prednisone therapy, Proximal, Proximal muscle weakness, Proximal weakness, Pulmonary fibrosis, Pulmonary function tests, Rash, Restrictive syndrome, Rheum, Rheumatic diseases, Rheumatoid arthritis, Semin arthritis rheum, Sen6cal, Significant difference, Skin cancer, Skin lesions, Study population, Swedish patients, Symptom, Syndrome, Systemic sclerosis, Uthman.
Abstract
Abstract: This is the first report on idiopathic inflammatory myopathies (IIM) in FrenchCanadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4,13%), anti-U1RNP (n = 3,10%), and anti-Ro (n = 2,7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1, autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.
Url:
DOI: 10.1016/S0049-0172(96)80025-4
Affiliations:
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Le document en format XML
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<term>French canadian patients</term>
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<front><div type="abstract" xml:lang="en">Abstract: This is the first report on idiopathic inflammatory myopathies (IIM) in FrenchCanadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4,13%), anti-U1RNP (n = 3,10%), and anti-Ro (n = 2,7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1, autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.</div>
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