Long-term outcome in polymyositis and dermatomyositis

Abstract

Background: Although polymyositis and dermatomyositis are regarded as treatable disorders, prognosis is not well known, as in the literature long-term outcome and prognostic factors vary widely. Aim: To analyse the prognostic outcome factors in polymyositis and adult dermatomyositis. Methods: We determined mortality, clinical outcome (muscle strength, disability, persistent use of drugs and quality of life) and disease course and analysed prognostic outcome factors. Results: Disease-related death occurred in at least 10% of the patients, mainly because of associated cancer and pulmonary complications. Re-examination of 110 patients after a median follow-up of 5 years showed that 20% remained in remission and were off drugs, whereas 80% had a polycyclic or chronic continuous course. The cumulative risk of incident connective tissue disorder in patients with myositis was significantly increased. 65% of the patients had normal strength at follow-up, 34% had no or slight disability, and 16% had normal physical sickness impact profile scores. Muscle weakness was associated with higher age (odds ratio (OR) 3.6; 95% confidence interval (CI) 1.3 to 10.3). Disability was associated with male sex (OR 3.1; 95% CI 1.2 to 7.9). 41% of the patients with a favourable clinical outcome were still using drugs. Jo-1 antibodies predicted the persistent use of drugs (OR 4.4, 95% CI 1.3 to 15.0). Conclusions: Dermatomyositis and polymyositis are serious diseases with a disease-related mortality of at least 10%. In the long term, myositis has a major effect on perceived disability and quality of life, despite the regained muscle strength.

Nodular Amyloidosis Overlapping and Polymyositis in a Patient with Skin Manifestations Lupus Fax: Case Report

“…Initial symptoms are usually weakness and weight loss, later on the appearance of cutaneous lesions, particularly in the periorbital and facial regions, bone pain, more intense bleeding and, finally, those related to neuropathies [17] [18] [19] [20] [21]. The patient presented his with a complaint of myalgia, paranesthesia and paresis of the pelvic girdle, accompanied by cutaneous lesions on the scalp, face, ears, shoulder, back and neck, a course similar to that found in previous studies, which draws attention to the behavior.…”

Síndrome antissintetase anti-Jo-1

“…4,31,33,34 Furthermore, positive anti-Jo-1 and/or interstitial pneumopathy as prognostic factors remain controversial. [34][35][36][37] In our patients, there was one death attributed to septic shock (hospital bronchopneumonia).…”

IVIG tedavisine dramatik yanıt veren dermatomiyozit olgusu

“…Sıklığı ortalama yılda 2-10 yeni olgu olarak bildirilmiştir (2). DM, subakut başlangıçlı, simetrik, proksimal kas güçsüzlüğü ile karakterizedir (3). Tipik cilt bulguları olan heliotropik raş ve gottron papülleri ile polimiyozitten ayrılır (2).…”