Idiopathic inflammatory myopathies (IIM) are rare disorders characterised by the presence of skeletal muscle inflammation, with interstitial lung disease (ILD) being the most frequent pulmonary manifestation. The spectrum of clinical presentations of myositis related ILD (M-ILD) encompasses a chronic process to a rapidly progressive ILD (RP-ILD); which is associated with a high mortality rate. The most effective treatments remain controversial and poses a unique challenge to both rheumatologists and respiratory physicians to manage. Given the rare heterogenous nature of M-ILD, there is a paucity of data to guide treatment. The cornerstone of existing treatments encompasses combinations of immunosuppressive therapies, as well as non-pharmacological therapies. In this review, we aim to summarize the current pharmacological therapies (including its dosing regimens and side effects profiles) and non-pharmacological therapies. Based on the existing literature to date, we propose a treatment algorithm for both chronic M-ILD and RP-ILD.
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http://dx.doi.org/10.1007/s00296-023-05336-z | DOI Listing |
Expert Rev Respir Med
January 2025
Respiratory Research @ Alfred, School of Translational Medicine, Monash University, VIC, Australia.
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Department of Rheumatology of Lucania - UOSD of Rheumatology, "Madonna delle Grazie" Hospital, Matera, Italy.
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View Article and Find Full Text PDFCase Rep Pulmonol
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Prisma Health, University of South Carolina-School of Medicine, Columbia, South Carolina, USA.
Diffuse alveolar hemorrhage (DAH) is a potentially life-threatening condition which can present with hemoptysis, diffuse alveolar infiltrates, anemia, and hypoxic respiratory failure. Antisynthetase syndrome (AS) is a rare autoimmune disorder most often characterized by nonerosive arthritis, proximal muscle weakness with elevated muscle enzymes, Raynaud's phenomenon, hyperkeratosis of the digits (mechanic's hands), and interstitial lung disease. According to large population studies, AS has an annual incidence of 0.
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