At present, the treatment of CTD-ILDs is mainly a combination of immunosuppressive drugs, and these treatments depend about clinicians, which vary widely (27). (ANA) positive; anti-Ro52 antibody positive; and anti-MDA5 antibody positive. Pulmonary high-resolution CT (HRCT) scan showed pulmonary interstitial inflammatory changes, and mediastinal and subcutaneous emphysema. She was finally diagnosed with anti-MDA5 antibody-positive ADM accompanied by RP-ILD. She was first given high-dose-steroid pulse therapy with methylprednisolone (500 mg per day for 3 days) followed by methylprednisolone (40 mg, daily), cyclosporine A (100 mg, twice per day time), and hydroxychloroquine (200 mg, twice per day time). Since her discharge from our hospital in March of 2018, she has managed the methylprednisolone therapy (tapered to 10 mg daily), cyclosporine A (100 mg, twice per day time), and hydroxychloroquine (200 mg, twice per day time). Results: Pulmonary HRCT scans taken on 4, AM251 9, and 26 weeks after her discharge from our hospital showed the interstitial pneumonitis experienced significantly improved and that mediastinal and subcutaneous emphysema had been gradually absorbed. The individual can now participate in regular work and activities of daily living. Conclusion: The treatment of methylprednisolone pulse AM251 therapy combined with cyclosporine A and hydroxychloroquine may be an option for the RP-ILD accompanied by anti-MDA-positive ADM. After the acute phase, this combination therapy strategy is helpful to the disease control of individuals. Keywords: combination treatment strategy, interstitial lung diseases, anti-MDA antibody, cyclosporine A, hydroxychloroquine, methylprednisolone pulse therapy, amyopathic dermatomyositis Intro Idiopathic inflammatory myopathies (IIMs) are heterogeneous diseases characterized by symmetrical proximal muscle mass weakness, elevated muscle mass enzymes, CD127 and chronic skin or muscle mass inflammation (1). Since the 1970s, several sets of requirements have been published for the classification and/or analysis of IIMs (2). In 1975, Bohan and Peter proposed several subgroups of IIMs: polymyositis (PM), dermatomyositis (DM), juvenile dermatomyositis, overlap myositis, and myositis associated with malignancy (3, 4). In 2005, Troyanov et al. proposed a classification system based on clinicalCserological meanings and introduced a new subgroup that was called clinicoserologic overlap myositis (5). Currently, IIM is definitely most often classified into PM, DM, and inclusion body myositis (1). Amyopathic dermatomyositis (ADM) is definitely a medical subtype of DM, distinguished from additional DM subtypes by demonstration without symptoms of muscular disease. Euwer et al. 1st reported six instances of DM without evidence of muscle mass disease in 1991 (6). Sontheimer formally defined ADM in 2002 (7). According to the Classification and Diagnostic Criteria for IIMs released from the Western Center for Neuromuscular Diseases and the American Myopathy Study Collaborative Group in 2004, ADM individuals have standard rash manifestations of DM. ADM individuals do AM251 not have objective muscle mass weakness, and their creatine kinase (CK) levels and electromyograms are normal. ADM accompanied by rapidly progressive interstitial lung diseases (RP-ILD) has been reported primarily in Asia, with low treatment success (8). To our knowledge, there is no medical trial for this disease, since the prevalence is definitely too low and only a few instances are reported. The analysis of interstitial lung diseases (ILDs) is based on irregular imaging findings with respiratory symptoms (9). Individuals with ILDs often present with active dyspnea, restrictive air flow disorder, decreased diffusion function, and hypoxemia. Pathologically, ILD is definitely characterized by diffuse pulmonary parenchyma, alveolar swelling, interstitial fibrosis, and diffuse shadow on chest X-rays. According to the medical manifestations of ILDs, individuals were divided into two types: acute/subacute type and chronic type (9). According to the International Consensus Statement on Idiopathic Pulmonary Fibrosis of the American Thoracic Society AM251 and the Western Respiratory Society, RP-ILD is definitely defined as a progressive ILD within 3 months after the onset of respiratory symptoms (10). Suda et al. reported that acute/subacute ILDs were generally resistant to medicines, while chronic ILDs responded well. At the same time, the mortality rate of acute/subacute ILDs was much higher than that of chronic ILDs (67 and 0%, respectively) (9). There may be a link between ADM and.
