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. 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.
Highlights
Idiopathic inflammatory myopathies (IIMs) are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations, including those in the skin and lungs [1,2]
This review mainly describes the existing evidence on the pharmacologic managements of PM-/DM-/clinically amyopathic DM (CADM)-interstitial lung disease (ILD)
These findings suggest that treatment with PSL and calcineurin inhibitors (CNIs) is a promising initial therapy for PM-/DM-/CADM-ILD patients
Summary
Idiopathic inflammatory myopathies (IIMs) are a diverse group of autoimmune diseases characterized by muscular involvement and extramuscular manifestations, including those in the skin and lungs [1,2]. Optimal management of PM-/DM-/CADM-associated ILD (PM-/DM-/CADM-ILD) is important for clinicians in real-world clinical practice. The chronic form is defined as a slowly progressive ILD presenting with gradual deterioration over a period greater than 3 months, or as stable ILD without any progression for more than 3 months—the latter of which is less frequent. Recent studies have emphasized the importance of the assessment of myositis-specific autoantibodies (MSAs), which are closely linked to clinical phenotypes of myositis-associated ILD [17,18]. In real-world clinical practice, clinicians should consider therapeutic strategies for patients with PM-/DM-/CADM-ILD based on disease progression, clinical phenotype, and potential prognostic factors (e.g., ILD form and MSA status). This review mainly describes the existing evidence on the pharmacologic managements of PM-/DM-/CADM-ILD
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