Myositis-Associated Interstitial Lung Disease

A special issue of Medicina (ISSN 1648-9144). This special issue belongs to the section "Pulmonary".

Deadline for manuscript submissions: closed (31 May 2021) | Viewed by 23897

Special Issue Editors


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Guest Editor
Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
Interests: idiopathic inflammatory myopathies; connective tissue disease-associated-interstitial lung disease; prediction of outcome; cytokines; signaling pathway

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Guest Editor
Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan
Interests: interstitial lung disease; interstitial pulmonary fibrosis; connective tissue disease-associated-interstitial lung disease; mechanism of RA-ILD; anti-fibrotic drug

Special Issue Information

Dear Colleagues,

Inflammatory myopathies (myositis) are one of the autoimmune diseases, affecting the skeletal muscle primarily. Patients with myositis often have also extramuscular features, such as dermatitis, arthritis, interstitial lung disease (ILD), myocarditis and gastroenteropathy. Out of those extramuscular features, ILD is the most crucial complication because ILD is the leading cause of death in myositis, and develops frequently, in approximately half of patients with myositis. Thus, we need to evaluate and manage ILD appropriately in patients with myositis to prevent progression of ILD leading to fatal outcome.

Clinical characteristics of myositis-associated ILD (myositis-ILD), such as clinical course, patterns of lung imaging on high-resolution computed tomography, response to immunosuppressive therapy and prognosis, are highly variable among patients with myositis. A useful mean of resolving the complexity of these clinical features in myositis-ILD is the measurement of myositis-specific autoantibodies (MSAs). MSA are detected in over 60% of patients with myositis. Each MSA is closely related to relatively homogenous clinical features associated with myositis. The measurement of MSA is highly useful and important for support of making a diagnosis, prediction of prognosis, and organization of therapeutic strategies. However, these clinical characteristics are not always consistent among patients with a concordant MSA. That might be attributed to several factors including genetic factors, races, and aging. We need to provide a personalized medicine to individual patients with myositis-ILD based on the stratification of prognosis.

In the past decade, there is emerging evidence regarding myositis-ILD. We need to better understand how to predict clinical outcome in individual patients with myositis-ILD because physicians have a responsibility to improve quality of daily living and prognosis in those patients. Therefore, the journal Medicina is launching this Special Issue.

We encourage you and your co-workers to submit your articles reporting on this topic. Reviews regarding clinical aspect in myositis-ILD, in particular, articles providing an up-to-date overview of the use of biomarkers in prediction and management are particularly welcome. In addition, we warmly invite you to submit articles reporting on basic molecules contributed to development of myositis- ILD, and evidence and expectations from innovative molecular therapeutics.

Prof. Dr. Takahisa Gono
Dr. Yasuhiko Yamano
Guest Editors

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Keywords

  • Myositis
  • Interstitial lung disease
  • Phenotype
  • Prediction
  • Biomarkers
  • Prognosis
  • Personalized medicine

Published Papers (3 papers)

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Review

12 pages, 20823 KiB  
Review
High-Resolution CT Findings of Myositis-Related Interstitial Lung Disease
by Ryoko Egashira
Medicina 2021, 57(7), 692; https://doi.org/10.3390/medicina57070692 - 06 Jul 2021
Cited by 12 | Viewed by 6779
Abstract
Myositis-related interstitial lung disease presents with a wide variety of lesions, ranging from chronic to acute. It can be divided into two main forms by the types of onsets, namely, chronic to subacute type showing nonspecific interstitial pneumonia (NSIP) or NSIP with an [...] Read more.
Myositis-related interstitial lung disease presents with a wide variety of lesions, ranging from chronic to acute. It can be divided into two main forms by the types of onsets, namely, chronic to subacute type showing nonspecific interstitial pneumonia (NSIP) or NSIP with an organizing pneumonia (OP)/fibrosing OP (FOP) pattern and acute type showing acute lung injury (ALI) to diffuse alveolar damage (DAD) pattern. Anti-aminoacyl tRNA Synthetase antibody-positive cases mainly show an NSIP or FOP pattern, whereas anti-melanoma differentiation-associated gene 5 antibody-positive cases show ALI to DAD pattern. Bilateral consolidation with or without ground-glass opacification with lower lobe predominance is common as a major pattern in all types, but the distribution or extent is sometimes different. The early detection of findings that indicate a rapid progressive course is vital. Diffuse cranio-caudal distribution and multiple ground-glass opacifications with random distribution might indicate a poorer prognosis. Full article
(This article belongs to the Special Issue Myositis-Associated Interstitial Lung Disease)
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11 pages, 1640 KiB  
Review
Myositis-Related Interstitial Lung Disease: A Respiratory Physician’s Point of View
by Yuko Waseda
Medicina 2021, 57(6), 599; https://doi.org/10.3390/medicina57060599 - 10 Jun 2021
Cited by 6 | Viewed by 5920
Abstract
Idiopathic inflammatory myositis (IIM) is an umbrella term for diseases of unknown origin that cause muscle inflammation. Dermatomyositis and polymyositis are IIMs that commonly cause interstitial lung disease (ILD). When a patient presents with ILD, the evaluation of whether the case displays the [...] Read more.
Idiopathic inflammatory myositis (IIM) is an umbrella term for diseases of unknown origin that cause muscle inflammation. Dermatomyositis and polymyositis are IIMs that commonly cause interstitial lung disease (ILD). When a patient presents with ILD, the evaluation of whether the case displays the characteristics of myositis should be determined by interview, physical examination, imaging findings, the measurement of myositis-related antibodies, and the determination of disease severity after diagnosis. Rapidly progressing anti-melanoma differentiation-associated gene 5 antibody-positive ILD may require rapid multi-drug therapy, while anti-aminoacyl tRNA synthetase (ARS) antibody-positive ILD can be treated with anti-inflammatory drugs. Importantly, however, anti-ARS antibody-positive ILD often recurs and sometimes develops into fibrosis. Early diagnosis is crucial for treatment, and we therefore need to clarify the features of myositis associated with ILD 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. Full article
(This article belongs to the Special Issue Myositis-Associated Interstitial Lung Disease)
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15 pages, 591 KiB  
Review
Management of Myositis-Associated Interstitial Lung Disease
by Tomoyuki Fujisawa
Medicina 2021, 57(4), 347; https://doi.org/10.3390/medicina57040347 - 03 Apr 2021
Cited by 38 | Viewed by 10553
Abstract
Idiopathic inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), and clinically amyopathic DM (CADM), are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations. Interstitial lung disease (ILD) has major pulmonary involvement and is associated with increased mortality in PM/DM/CADM. [...] Read more.
Idiopathic inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), and clinically amyopathic DM (CADM), are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations. Interstitial lung disease (ILD) has major pulmonary involvement and is associated with increased mortality in PM/DM/CADM. The management of PM-/DM-/CADM-associated ILD (PM/DM/CADM-ILD) requires careful evaluation of the disease severity and clinical subtype, including the ILD forms (acute/subacute or chronic), because of the substantial heterogeneity of their clinical courses. Recent studies have highlighted the importance of myositis-specific autoantibodies’ status, especially anti-melanoma differentiation-associated gene 5 (MDA5) and anti-aminoacyl tRNA synthetase (ARS) antibodies, in order to evaluate the clinical phenotypes and treatment of choice for PM/DM/CADM-ILD. Because the presence of the anti-MDA5 antibody is a strong predictor of a worse prognosis, combination treatment with glucocorticoids (GCs) and calcineurin inhibitors (CNIs; tacrolimus (TAC) or cyclosporin A (CsA)) is recommended for patients with anti-MDA5 antibody-positive DM/CADM-ILD. Rapidly progressive DM/CADM-ILD with the anti-MDA5 antibody is the most intractable condition, which requires immediate combined immunosuppressive therapy with GCs, CNIs, and intravenous cyclophosphamide. Additional salvage therapies (rituximab, tofacitinib, and plasma exchange) should be considered for patients with refractory ILD. Patients with anti-ARS antibody-positive ILD respond better to GC treatment, but with frequent recurrence; thus, GCs plus immunosuppressants (TAC, CsA, azathioprine, and mycophenolate mofetil) are often needed in order to achieve favorable long-term disease control. PM/DM/CADM-ILD management is still a therapeutic challenge for clinicians, as evidence-based guidelines do not exist to help with management decisions. A few prospective clinical trials have been recently reported regarding the treatment of PM/DM/CADM-ILD. Here, the current knowledge on the pharmacologic managements of PM/DM/CADM-ILD was mainly reviewed. Full article
(This article belongs to the Special Issue Myositis-Associated Interstitial Lung Disease)
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