Idiopathic Inflammatory Myopathies: Clinical Approach and Management

Abstract

Idiopathic inflammatory myopathies (IIM) are a group of chronic, autoimmune conditions affecting primarily the proximal muscles. The most common types are dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myopathy (NAM), and sporadic inclusion body myositis (sIBM). Patients typically present with sub-acute to chronic onset of proximal weakness manifested by difficulty with rising from a chair, climbing stairs, lifting objects, and combing hair. They are uniquely identified by their clinical presentation consisting of muscular and extramuscular manifestations. Laboratory investigations, including increased serum creatine kinase (CK) and myositis specific antibodies (MSA) may help in differentiating clinical phenotype and to confirm the diagnosis. However, muscle biopsy remains the gold standard for diagnosis. These disorders are potentially treatable with proper diagnosis and initiation of therapy. Goals of treatment are to eliminate inflammation, restore muscle performance, reduce morbidity, and improve quality of life. This review aims to provide a basic diagnostic approach to patients with suspected IIM, summarize current therapeutic strategies, and provide an insight into future prospective therapies.

A study on demographic pattern of idiopathic inflammatory myopathies in a tertiary care hospital

“…Arthritis was a relatively common symptom noticed, about 68 % presented with this feature. this correlates with the studies done by Malik et al, and Muggi N et al 6,7 Raynaud's phenomenon was seen to affect 37.5% of patients, it was predominantly noted in DM than PM, this data concurs with study done by Clark et al 8 Other significant parameters noted in our study were elevated muscle enzymes in 85.1% patients, positive electromyogram in 60%. 9 Hypothyroidism noted in 37.5% of patients by Gamsky et al 9 In this study describe the coexistent autoimmune thyroiditis and dermatomyositis, showing clearly that these diseases can coexist.…”

The Disappearance of a Hepatic Mass in Anti-Synthetase Syndrome

“…(1,10) Those not presenting with respiratory symptoms should have pulmonary function tests, and a thoracic highresolution CT. High-dose corticosteroids are the first-line treatment for ASyS. (11) Although corticosteroids are considered the mainstay of treatment, additional immunosuppressive agents, such as azathioprine and methotrexate, are often used, both as corticosteroid sparing agents and to achieve disease control. In those with severe or refractory disease, rituximab may be used.…”

Profile of Pediatric Idiopathic Inflammatory Myopathies from a Tertiary Care Center of Eastern India

“…The duration of the MUAPs increase in chronic cases due to re-innervation of split fibers [1]. In s-IBM, MUAPs are characteristically of longduration and large-amplitude while pseudomyotonic discharges may be found in NAM [24]. However, these varieties of IIM are not common in pediatric age group.…”