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01-05-2014 | Original Communication

Long-term follow-up of 62 patients with myositis

Authors: Janneke van de Vlekkert, Jessica E. Hoogendijk, Marianne de Visser

Published in: Journal of Neurology | Issue 5/2014

Abstract

The aim of this work is to evaluate disease-related mortality and the course of the disease including functional outcome and quality of life. We did a follow-up study on a large prospective cohort of 62 patients with subacute-onset idiopathic inflammatory myopathy (IIM) (dermatomyositis (n = 24), nonspecific myositis (n = 34), necrotizing autoimmune myopathy (n = 4)) after treatment with corticosteroids only (randomized controlled trial comparing daily high-dosage prednisone with pulse therapy of dexamethasone). Development of connective tissue disease (CTD) or malignancy, disease course and mortality, functional outcome and quality of life were evaluated. After a mean follow-up of 3 years (SD 1.5), 22 % had developed a CTD and 17 % a malignancy. Disease-related mortality was 15 %. A monophasic disease course was found in 27 %. Most patients had a chronic (35 %) or polyphasic disease (35 %) course and experienced single or multiple relapses. Sixteen patients (33 %) were off medication after a mean of 1 year of treatment. Disability scores improved particularly in the first 18 months. At follow-up, 68 % still perceived disabilities. Quality of life scores as measured by the short-form (SF)-36 improved in the first 18 months. After 18 months, scores remained stable during the next years of follow-up and remained low compared to a normal population. (1) Two-thirds of the patients with an IIM have a polyphasic or chronic disease course and need maintenance treatment. (2) The impact on functional outcome and quality of life is considerable and does not improve further after 18 months.

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Title: Long-term follow-up of 62 patients with myositis
Authors: Janneke van de Vlekkert
Jessica E. Hoogendijk
Marianne de Visser
Publication date: 01-05-2014
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 5/2014
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-014-7313-z

At a glance: The STEP trials

Springer Medicine

Long-term follow-up of 62 patients with myositis

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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