We believe that the most important point is to suspect myositis-related ILDs and to develop the ability to diagnose myositis-related ILDs by combining interviews, physical examinations, and imaging findings. and suspect these pathologies early. This section reviews what clinicians need to look for and what findings are evaluated in patients when diagnosing myositis associated with ILD. Keywords:polymyositis, dermatomyositis, anti-ARS antibody, anti-MDA5 antibody, rapid progressive interstitial lung disease, progressive fibrosing interstitial lung disease == 1. Introduction == Idiopathic inflammatory myositis (IIM) is an umbrella term for a spectrum of pathologies involving muscle inflammation of unknown origin, including dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis, malignancy-associated myositis, and immune-mediated necrotizing myopathy. Among the IIMs, DM and PM are both connective tissue CDKN2AIP diseases (CTDs) that cause interstitial lung disease (ILD). PM can almost always be improved or prevented with anti-inflammatory drugs and DM is sometimes improved with anti-inflammatory drugs, but anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive ILD is sometimes unimproved by such agents and follows a rapidly progressive (RP) course. Autoantibodies against aminoacyl-tRNA synthetases (ARSs) are detected in 2535% of patients with IIM, and this condition is referred to as anti-synthetase syndrome (ASS). ASS represents a group of diseases strongly associated with arthritis, ILD, and so-called mechanics hands [1]. The combination of different classes of anti-inflammatory drugs, particularly steroids and immunosuppressive drugs, is effective in ASS, and these drugs thus represent the first line of therapy [2]. Early diagnosis is therefore important to enable prompt treatment. Although most cases respond readily to anti-inflammatory treatment, many relapse when pharmacotherapies are reduced or stopped. In some cases, fibrosis progresses to respiratory failure and the early administration of antifibrotic agents may be necessary. At present, however, patients with progressive fibrosis cannot be reliably identified at an early stage, so the evaluation of the progression of fibrosis within a relatively short period of time is necessary. In anti-MDA5 antibody-positive ILD, early diagnosis and early triple therapy with anti-inflammatory drugs are considered important [3], as about half of all patients with anti-MDA5 antibody-positive ILD die. However, some cases of anti-MDA5 antibody-positive ILD do not progress rapidly and do not necessarily require strong immunosuppression [4]. In any case, IIMs, particularly ASS and anti-MDA5 antibody-positive ILD, need to be treated early if treatment is actually required, and early diagnosis is therefore very important for clinicians. Diagnostic criteria from the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) are shown inTable 1[5]. The score using these criteria is characteristically higher if a muscle biopsy specimen is available for testing. Although the EULAR/ACR criteria do not mention the presence or absence of ILD, suspicion of IIM is important in patients with ILD because, as mentioned above, early treatment is crucial in ASS and anti-MDA5 antibody-positive ILD. The purpose of this review was to present the latest findings, with expert opinions, regarding what findings should be considered for suspected myositis-related ILD when examining ILD from the perspective of a respiratory physician. The paper is divided into an Dolasetron Mesylate interview section, an objective findings section, and an examination section with reference to the EULAR/ACR classification to explain what is necessary in order to diagnose myositis-related ILD from the perspective of the respiratory physician. == Table 1. == Point scores for the European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic Dolasetron Mesylate inflammatory myopathies, to be used in the absence of better explanations for symptoms or signs (from the figure in Reference [5]). Serum levels above upper limit of normal. == 2. Diagnostic Points == == Dolasetron Mesylate 2.1. Interview == Questions regarding the characteristics of myositis include looking for the presence of progressive, symmetrical muscle weakness, particularly with a proximal muscle dominance. Specific questions for muscle weakness include: Do you feel weakness in your thighs when climbing stairs?, Can you squat and stand up?, Do you feel weak when lifting a load onto an upper shelf?, and Do you find it difficult to support your neck? We need to check for the presence of any skin rashes, whether the fingers become pale and painful on exposure to cold air or water, and the presence of any dysphagia.