Abstract

Objectives: Idiopathic inflammatory myopathies (IIM) are a group of inflammatory muscle diseases with a paucity of data and literature from Saudi Arabia. The aim was to describe the demographics, evaluate clinical features, organ involvement, investigations, treatment strategies, and to assess factors affecting remission in IIM patients. Methods: We conducted this retrospective study at 5 medical tertiary centers in Saudi Arabia to analyze the records of patients with IIM from 1999 to 2014. Results: A total of 28 patients with IIM were identified with a female to male ratio of 3:1. Pure polymyositis accounted for (32.1%), pure dermatomyositis (21.4%), juvenile dermatomyositis (10.7%), and IIM mixed with connective tissue diseases (35.7%) of the patients. The most common presentation was proximal myopathy (93.9%). The musculoskeletal system was the most commonly involved (78.6%), followed by the gastrointestinal system (39.3%). Investigations revealed an elevated CK of 2995.12 ± 3431.51 (mean ± SD), EMG was positive in (92.9%) of patient, and muscle biopsy in (42.9%). Treatment data showed a good response to Prednisolone (96.4%). Conclusion: The results of this multiregional retrospective study from Saudi Arabia on IIM patients showed similar clinical features to other reviews; however, malignancy percentages were lower. We found a strong relation between comorbid illnesses and relapse. Normalization of muscle power is a reliable prognostic factor that may help assess the efficacy of treatment.

Highlights

  • The idiopathic inflammatory myopathies (IIM) are systemic connective tissue diseases characterized by symmetrical, proximal muscle weakness and chronic inflammation in muscle tissue

  • Patients with the diagnosis of pure PM, pure DM, juvenile dermatomyositis (JDM), and PM/DM/JDM mixed with other connective tissue diseases; namely, rheumatoid arthritis (RA), scleroderma, and seronegative arthritis were included

  • Demographic and general background A total of 28 patients with IIM were identified, 9 patients had PM, 6 had DM, 3 had JDM, and 10 patients had IIM mixed with connective tissue diseases (6 scleroderma, 3 ankylosing spondylitis, and one with RA)

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Summary

Introduction

The idiopathic inflammatory myopathies (IIM) are systemic connective tissue diseases characterized by symmetrical, proximal muscle weakness and chronic inflammation in muscle tissue. They can be subclassified into dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM) according to differences in clinical as well as histopathological features, and they frequently involve other systems with interstitial lung disease, esophageal dis-motility, and heart failure being the most common [1,2,3,4]. Many reports proved the effectiveness of corticosteroids in most of the cases, with some patients requiring a combined therapy of corticosteroid with methotrexate or azathioprine [10,11]

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