2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease

Kondoh, Yasuhiro; Makino, Shigeki; Ogura, Takashi; Suda, Takafumi; Tomioka, Haruaki; Amano, Hirofumi; Anraku, Masaki; Enomoto, Noriyuki; Fujii, Tomoyuki; Fujisawa, Tomoyuki; Gono, Takahisa; Harigai, Masayoshi; Ichiyasu, Hidenori; Inoue, Yoshikazu; Johkoh, Takeshi; Kameda, Hideto; Kataoka, Kensuke; Katsumata, Yasuhiro; Kawaguchi, Yasushi; Kawakami, Atsushi; Kitamura, Hideya; Kitamura, Noboru; Koga, Tomohiro; Kurasawa, Kazuhiro; Nakamura, Yutaro; Nakashima, Ran; Nishioka, Yasuhiko; Nishiyama, Osamu; Okamoto, Masaki; Sakai, Fumikazu; Sakamoto, Susumu; Sato, Shinji; Shimizu, Toshimasa; Takayanagi, Noboru; Takei, Reoto; Takemura, Tamiko; Takeuchi, Tohru; Toyoda, Yuko; Yamada, Hisakata; Yamakawa, Hideaki; Yamano, Yasuhiko; Yamasaki, Yoshioki; Kuwana, Masataka

doi:10.1016/j.resinv.2021.04.011

Cited by 70 publications

(66 citation statements)

References 207 publications

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“…One factor favoring inflammatory change is the presence of MPA-like disease (mock MPA), as indicated by recurrent unexplained fever and/or higher CRP/ESR and an upward trend in the MPO-ANCA titer. Moreover, the second factor is increased attenuation around fibrotic change seen on HRCT and prominent inflammatory cell infiltration in pathological lung lesions [1][2][3]47,48]. Regarding the former (i.e., mock MPA), if patients with MPO-ANCA-positive idiopathic ILD develop MPA, they should be treated with antiinflammatory therapy because untreated MPA (particularly that with DAH) is normally progressive and fatal [13,31].…”

Section: Mpo-anca-positive Ild Patients Without Systemic Vasculitis A...mentioning

confidence: 99%

“…Therefore, if these conditions are present, anti-inflammatory therapy should be considered. reticulation with traction bronchiectasis and honeycombing on HRCT without increasing attenuation (i.e., ground-glass opacity [GGO] and consolidation), and pathologically patchy fibrosis as a UIP pattern and/or fibroblastic foci in a lung specimen without prominent inflammatory cells [1][2][3]30,31,33,34,[47][48][49][50]. As a caveat, GGO, which is a finding typically associated with inflammation or infiltration, does not always represent reversible lung disease, but rather in some cases, it may represent microscopic fibrosis [51].…”

Section: Mpo-anca-positive Ild Patients Without Systemic Vasculitis A...mentioning

confidence: 99%

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Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review

Yamakawa

Toyoda

Baba

et al. 2022

JCM

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The presence of a lung lesion is common in microscopic polyangiitis (MPA), and interstitial lung disease (ILD) can lead to a poor prognosis. Although myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) are often present in patients with MPA, patients with ILD and MPO-ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Therefore, the possible association between MPO-ANCA, MPA, and idiopathic ILD remains unclear. This problematic matter has influenced the treatment strategy of MPO-ANCA-positive ILD patients without systemic vasculitis. Clinicians should undertake treatment with careful consideration of the four major causes of death in MPO-ANCA-positive ILD: acute exacerbation of ILD, progressive lung fibrosis, infectious comorbidities, and diffuse alveolar hemorrhage. Further, clinicians need to carefully judge whether inflammation or fibrosis is the dominant condition with reference to the patient’s clinical domain and radiopathological lung features. Recently, anti-fibrotic agents such as nintedanib and pirfenidone were shown to be effective in treating various etiologies associated with ILD and have thus led to the widening of treatment options. In this review, the clinical characteristics, radiopathology, prognosis, and therapeutic options in patients with MPO-ANCA-positive ILD are summarized using limited information from previous studies.

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Section: Mpo-anca-positive Ild Patients Without Systemic Vasculitis A...mentioning

confidence: 99%

Section: Mpo-anca-positive Ild Patients Without Systemic Vasculitis A...mentioning

confidence: 99%

Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review

Yamakawa

Toyoda

Baba

et al. 2022

JCM

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“…In particular, interstitial lung disease (ILD) is one of the major manifestations associated with poor mortality in patients with polymyositis (PM)/dermatomyositis (DM) ( 2 ). The management of ILD in patients with PM/DM aims to ameliorate, stabilize, or slow its progression based on the disease behavior of ILD ( 3 ). In terms of treatment for myositis-associated ILD, systemic corticosteroid therapy is usually combined with immunosuppressive agents, such as azathioprine, cyclophosphamide, mycophenolate mofetil, methotrexate, cyclosporine, tacrolimus, and/or rituximab, although there is little evidence to support the efficacy of these individual agents ( 4 ).…”

Section: Introductionmentioning

confidence: 99%

“…In terms of treatment for myositis-associated ILD, systemic corticosteroid therapy is usually combined with immunosuppressive agents, such as azathioprine, cyclophosphamide, mycophenolate mofetil, methotrexate, cyclosporine, tacrolimus, and/or rituximab, although there is little evidence to support the efficacy of these individual agents ( 4 ). Since the clinical course, response to treatment, and prognosis are highly variable among patients with myositis-associated ILD ( 3 , 5 ), the treatment regimen is decided based mainly on the progression speed and severity of ILD. On the other hand, many studies have reported clinical, laboratory, and imaging features predicting subsequent treatment response and prognosis ( 6 – 11 ).…”

Section: Introductionmentioning

confidence: 99%

Mortality Risk Stratification Using Cluster Analysis in Patients With Myositis-Associated Interstitial Lung Disease Receiving Initial Triple-Combination Therapy

et al. 2022

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ObjectiveTo stratify patients with polymyositis/dermatomyositis-associated interstitial lung disease (ILD) who were initially treated with an intensive regimen consisting of high-dose corticosteroids, a calcineurin inhibitor, and intravenous cyclophosphamide (triple-combo therapy) into subgroups based on mortality outcomes by a cluster analysis using a large-scale multicenter retrospective cohort of Japanese patients with myositis-associated ILD (JAMI).MethodsTwo-step cluster analysis of preclustering and subsequent hierarchical clustering was conducted in 185 patients who received triple-combo therapy in an unbiased manner. Initial predictors for mortality previously reported in patients with myositis-associated ILD were used as variables and included age, sex, disease duration, classification of myositis, requirement of supplemental oxygen, anti-aminoacyl tRNA synthetase (ARS) antibody, anti-melanoma differentiation-associated gene 5 (MDA5) antibody, and serum levels of C-reactive protein (CRP) and Krebs von den Lungen-6 (KL-6). The cluster model was further applied to 283 patients who received conventional regimens consisting of corticosteroids with or without a single immunosuppressive agent (dual-combo therapy or monotherapy). Cumulative survival rates were compared using Kaplan-Meier analysis, and the log-rank test was used to test for significant differences between two groups.ResultsWe developed a cluster model consisting of 6 clusters, which were categorized by age at onset, clinically amyopathic dermatomyositis, CRP, KL-6, requirement of supplemental oxygen, anti-ARS antibody, and anti-MDA5 antibody. This model was judged to be of good quality based on the silhouette measure of cohesion and separation of 0.6. These clusters were regrouped into three subsets based on low (<10%), moderate (10-50%), and high (>50%) mortality rates. The performance of the clustering was generally replicated in patients who received initial dual-combo therapy or monotherapy. Survival benefits of triple-combo therapy over dual-combo therapy or monotherapy were not observed in any of the clusters.ConclusionWe successfully developed a cluster model that stratified patients with myositis-associated ILD who were treated with initial triple-combo therapy into subgroups with different prognoses, although this model failed to identify a patient subgroup that showed survival benefits from triple-combo therapy over dual-combo therapy or monotherapy.

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“…The discovery of anti-MDA5 antibody and the development of a convenient measurement procedure for this autoantibody have led physicians to manage anti-MDA5-positive DM patients earlier and more readily before devastating RP-ILD develops ( 2 , 3 ). Researchers across the world have been encouraged to determine new aspects regarding the etiology, pathogenesis, pathophysiology, variability of clinical features, independent prognostic factors, and therapeutic strategies of this disease ( 4 – 9 ). However, sufficient research has not yet been carried out to achieve satisfactory outcomes for anti-MDA5-positive DM patients.…”

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confidence: 99%

Editorial: Anti-MDA5-Positive Dermatomyositis

Gono

Han

2022

Front. Med.

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2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease

Cited by 70 publications

References 207 publications

Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review

Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review

Mortality Risk Stratification Using Cluster Analysis in Patients With Myositis-Associated Interstitial Lung Disease Receiving Initial Triple-Combination Therapy

Editorial: Anti-MDA5-Positive Dermatomyositis

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