Systemic sclerosis-related myopathy

Systemic sclerosis (SSc) is an autoimmune disorder which can affect nearly every body organ. Muscle involvement is one of the most serious manifestations of SSc. It can present itself in a wide range of pathologies. It can be as indolent as a subclinical myopathy which manifests simply as a mild muscle enzyme level elevation, or it can present itself in a similar manner to other SSc organ involvements in the form of fibrosing myopathy without any existing evidence of inflammation. It can also present itself aggressively as an idiopathic inflammatory myopathy, the overlap syndrome of SSc-myositis. Due to the wide range of witnessed pathologies and the different diagnostic criteria that are used, opinions vary on the estimated prevalence of myopathy in SSc with estimates ranging from 3.3% to 14%. The severity and distribution of clinical manifestations differ among SSc-myopathy and SSc patients. These manifestations have different effects on the survival rates of patients, which will be discussed in this review. This paper will also focus on the existing treatment methods for SSc patients suffering from myopathy and their challenges.