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Inflammatory Myopathy
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
Inflammatory myopathy (IM) comprises a diverse group of acquired skeletal muscle diseases often occurring in complex clinical settings. It is a diagnostic challenge in clinical practice that usually requires multidisciplinary expertise to reach an accurate diagnosis. Since the 1990s, the classification of IMs has been limited by specific pathological variants, defined by Bohan and Peter et al. These variants include polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM). The only distinction between these conditions is the skin involvement. Several attempts have been made to enroll serological autoantibodies in the diagnostic workup. Love et al. and Troyanove et al., at the end of the nineteenth century, created a novel classification of IMs based on autoantibody profiles. Unfortunately, it has recently been proven that fewer than 50% of patients with IMs present with known antibodies, and that classification lacks the association with morphological data. In 2003, immune-mediated myopathy (IMM) was included in the classification of IMs as a new entity.
Sjögren syndrome and mixed connective tissue disease
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Arthritis is a common feature of MCTD, seen in about 60% patients. Usually, it is nonerosive but occasionally erosions and an arthritis mutilans-like picture may develop [40]. Most patients develop an inflammatory myopathy during the course of their disease; however, it is rarely present at the onset of the disease.
Classification and clinical features
Published in Aparna Palit, Arun C. Inamadar, Systemic Sclerosis, 2019
Aparna Palit, Arun C. Inamadar
The musculoskeletal system is prominently involved in patients with PSS. Restriction of movements, joint pain, and the feelings of weakness experienced by these patients are of multifactorial origin. These include3,4: Sclerosis of the skin overlying the joints and peri-articular fibrosisErosive polyarthritis specific for SScOverlap of scleroderma and rheumatoid arthritisDisuse atrophy of musclesExtension of the fibrotic process to involve skeletal muscles in the background of severe cutaneous involvement in patients with dSSc (fibrosing myopathy).Associated inflammatory myopathy (5%–10%).Malnutrition (impaired food intake due to microstomia, dysphagia, and gastro-intestinal involvement related to the disease).
New paradigm in the treatment of myositis-associated interstitial lung disease
Published in Expert Review of Respiratory Medicine, 2023
Takahisa Gono, Masataka Kuwana
Idiopathic inflammatory myopathy or myositis encompasses polymyositis (PM), immune-mediated necrotizing myopathy, inclusion body myositis, dermatomyositis (DM), amyopathic DM (ADM), juvenile DM, and other juvenile myositis based on the classification criteria proposed jointly by the European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) [1]. Extramuscular manifestations include cutaneous manifestations, such as heliotrope rash, Gottron’s papules/sign, panniculitis, subcutaneous calcinosis, and ulceration, arthritis, cardiomyopathy, interstitial lung disease (ILD), upper gastrointestinal involvement, and concomitant malignancy [2,3]. Notably, ILD and malignancy are the leading causes of mortality in patients with myositis [4]. Clinical characteristics of myositis-associated ILD are highly variable among patients regarding the course of ILD onset, rate of progression, radiological and pathohistological morphologies of the lungs, extent and distribution of inflammation and fibrosis, responses to treatment, recurrence rate, and prognosis [5]. Physicians are required to assess not only the severity of ILD but also the future prognosis based on the stratification of mortality risk for individual patients with myositis-associated ILD to select optimal management aiming to ameliorate or maintain pulmonary function as well as the quality of life for the long term [6].
Acute interstitial pneumonia due to amyopathic dermatomyositis
Published in Baylor University Medical Center Proceedings, 2022
Simón Esteva, Erin Tuttle, He Huang, Nishith Mewada
Idiopathic inflammatory myopathies, otherwise known as myositis, are a group of muscular disorders characterized by muscular inflammation that can manifest in other organs such as the skin, lungs, and joints. Up to 20% of patients with dermatomyositis have clinically amyopathic disease, meaning that for at least 6 months patients have no radiological or laboratory markers and lack muscle weakness of myositis despite the presence of cutaneous findings.1 Though the clinical presentation and severity of these diseases can vary, the novel use of autoantibodies has been instrumental in the characterization and classification of each specific idiopathic inflammatory myopathy. Anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibodies have a well-documented association with acute interstitial pneumonia (AIP), particularly in patients with clinically amyopathic dermatomyositis.2
Clinical experience in anti-synthetase syndrome: a monocentric retrospective analytical study
Published in Acta Clinica Belgica, 2022
Quentin Maloir, Seidel Laurence, Von Frenckell Christian, Gester Fanny, Louis Renaud, Guiot Julien
Currently, treatment management is still challenging in ASS [9]. There have been some recent studies comparing available immunosuppressive therapeutics and their efficacy on PFT, HRCT findings and corticosteroids sparing’s efficiency [14,24]. Our study did not identify significant differences between immunosuppressive drugs or their regimens. Trying to find the best therapy for such diseases should be the main objective for further multicentric prospective longitudinal trials. Historically, corticosteroids have been the first-line therapy for idiopathic inflammatory myopathy (IIM). When corticosteroid tapering is used in monotherapy in ILD-IIM, the risk of lung disease persistence is high. Moreover, the severity of ILD presentation frequently requires the rapid use of aggressive immunosuppressive agents (e.g. cyclophosphamide, rituximab, etc.) [25]. Azathioprine is used as first-line therapy when an ILD is present, while mycophenolate mofetil is used by rheumatologists as the first-line to treat IIM without lung involvement. Other therapeutics, such as tacrolimus, rituximab, intravenous immunoglobulins and cyclophosphamide, should be considered second-line or rescue therapy in cases of refractory situations, such as acute respiratory distress syndrome. These specific therapies need to be closely monitored in their administration and require an expert multidisciplinary team to assess their efficacy and side effects.