Rapidly progressing treatment resistant anti-TIF1 gamma antibody positive dermatomyositis

Dermatomyositis is an idiopathic inflammatory myositis involving progressive muscle weakness with skin manifestations. Incidence of dermatomyositis is 1 per 100,000 in general population. Diagnosis is based on characteristic skin rashes, progressive muscleweakness and elevated muscle enzymes levels (creatine phosphokinase). The diagnosis is confirmed by clinical examination, abnormal electromyogram and autoimmune workup. In this case report, we report a case of difficult to treat dermatomyositis with proximal muscle weakness in an elderly male patient with classical skin lesions. The patient was resistant to steroid and immunoglobulin therapy.

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

Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.