Treatment of idiopathic inflammatory myositis associated interstitial lung disease: A systematic review and meta-analysis

Barba, Thomas; Fort, Romain; Cottin, Vincent; Provencher, Steeve; Durieu, I.; Jardel, S.; Hot, A.; Reynaud, Quitterie; Lega, Jean‐Christophe

doi:10.1016/j.autrev.2018.07.013

Cited by 114 publications

(88 citation statements)

References 60 publications

(20 reference statements)

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“…6 In chronic presentations of IIM-ILD, a recent metaanalysis reported a >80% efficacy of CS prescribed as the only treatment. 75 Although likely biased due to the retrospective design of the studies included in the meta-analysis, these findings support the use of CS alone as first-line therapy Fig. 8 Tentative treatment algorithm in patients with ILD associated with inflammatory myopathy.…”

Section: Corticosteroidsmentioning

confidence: 69%

“…Data regarding the efficacy of CS in rapidly progressive forms of IIM-ILD are scarce 19,36 ; when pooled, these data suggest a response rate of 50% for CS alone, therefore, mandating the upfront combined use of immunosuppressive therapies in rapidly progressive IIM-ILD. 75 Immunosuppressive therapies may also be justified by relapsing disease or may be used as steroid-sparing agents. Given the necessity of long-term treatment, the relatively poor tolerance of long-term use of oral CS, and the poor prognosis of severe IIM-ILD refractory to CS alone, many authors advocate the concomitant initiation of oral CS and immunosuppressive therapy, especially in patients with metabolic comorbidities.…”

Section: Corticosteroidsmentioning

confidence: 99%

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Lung Diseases in Inflammatory Myopathies

Barba

Mainbourg

Nasser

et al. 2019

Semin Respir Crit Care Med

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Lung involvement is the leading cause of mortality in inflammatory myopathy. A careful assessment of clinical and serologic manifestations especially myositis-associated autoantibodies allows precise classification of the different phenotypes of inflammatory myopathy and stratification of the risk of lung involvement. About three out of four patients with inflammatory myopathy develop interstitial lung disease (ILD), which represents the main cause of morbidity and mortality. In patients with a confirmed diagnosis of inflammatory myopathy, the approach to the diagnosis of ILD includes assessment of clinical and functional severity, evaluation of the high-resolution computed tomography pattern of disease, which often suggests nonspecific interstitial pneumonia or organizing pneumonia. Bronchoalveolar lavage to rule out infection is often performed; however, video-assisted thoracoscopic lung biopsy is now generally discouraged, unless malignancy is suspected. The so-called antisynthetase syndrome characterized by the combination of mechanics' hands, Raynaud' phenomenon, myositis often mild or absent, and presence of one of the anti-tRNA synthetase antibodies is associated with a 70% risk of ILD, especially in subjects with antibodies other than anti-Jo1 antibodies (i.e., anti-PL7 or -PL12 antibodies). Treatment depends on both severity and progression of ILD, often including a combination of corticosteroids and immunosuppressive therapy. Rituximab-based regimen has showed promising results in retrospective studies for the management of refractory or rapidly progressive forms of ILD. Clinical trials are ongoing to evaluate the actual efficacy of this strategy on mortality related to lung disease. Secondary pulmonary complications of inflammatory myopathy include opportunistic infections, aspiration pneumonia, pneumomediastinum, ventilatory failure due to diaphragmatic muscular weakness, drug-induced pneumonitis, and rarely pulmonary hypertension.

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Section: Corticosteroidsmentioning

confidence: 69%

Section: Corticosteroidsmentioning

confidence: 99%

Lung Diseases in Inflammatory Myopathies

Barba

Mainbourg

Nasser

et al. 2019

Semin Respir Crit Care Med

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“…Multiple organs apart from muscle are often affected as well, leading to critical worsening of the life quality and outcome of these patients (5). Among the multiple extramuscular complications of IIM, interstitial lung disease (ILD) was identified as both the most frequent and severe involvement, leading to a significant elevation in mortality rate (6). Moreover, acute exacerbation of ILD (AE-ILD), which used to be mainly studied in patients with idiopathic pulmonary fibrosis, has also been noticed in patients with connective tissue disease (CTD).…”

Section: Introductionmentioning

confidence: 99%

Acute Exacerbation of Interstitial Lung Disease in Adult Patients With Idiopathic Inflammatory Myopathies: A Retrospective Case-Control Study

Liang

Sun

et al. 2020

Front. Med.

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This study aimed at clarifying the prevalence, risk factors, outcome, and outcome-related factors of acute exacerbation of interstitial lung disease (AE-ILD) in patients with idiopathic inflammatory myopathy (IIM). Methods: Data of IIM patients who were admitted to the First Affiliated Hospital of Zhejiang University (FAHZJU) from September 2007 to September 2019 were retrospectively collected. And the IIM patients with AE-ILD formed the case group. In addition, age and sex matched IIM patients without AE-ILD were randomly selected to constitute the control group. A 1:2 case-control study and intragroup analysis were performed to identify risk factors for development of AE-ILD in IIM patients and unfavorable short-term outcome in AE-ILD patients through comparison, univariate and multivariate logistic regression analysis. Results: AE-ILD occurred in 64 out of 665 IIM patients (9.6%) with a short-term mortality rate of 39.1%. And the 64 IIM patients with AE-ILD formed the case group. Besides, 128 age and sex matched IIM patients without AE-ILD were randomly selected to constitute the control group. The retrospective case-control study revealed that elevated on-admission disease activity (P < 0.001), lower percent-predicted diffusing capacity of the lung for carbon monoxide (DLCO%, P = 0.013) and diagnosis of clinically amyopathic dermatomyositis (CADM, P = 0.007) were risk factors for development of AE-ILD in IIM patients. The following intragroup analysis indicated that elevated on-admission disease activity (P = 0.008) and bacterial infection (P = 0.003) were significantly correlated with the unfavorable short-term outcome of patients complicated with AE-ILD. In addition, combined use of steroid and disease modifying antirheumatic drugs (DMARDs, P = 0.006) was found to significantly reduce the short-term mortality in IIM patients with AE-ILD. Conclusion: AE-ILD is a less frequent but fatal complication in IIM patients with elevated on-admission disease activity, lower DLCO% and diagnosis of CADM working as risk factors, indicating the potential roles of autoimmune abnormality and hypoxia in development of AE-ILD. Elevated on-admission disease activity and bacterial infection could predict unfavorable short-term outcome of IIM patients with AE-ILD. A therapeutic regimen of steroid and DMARDs was found to reduce short-term death in these patients.

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“…OP, NSIP, mixed NSIP-organizing pneumonia pattern are more frequent than UIP pattern in PM/DM-ILD (12). The ARS antibodies, one of the myositis speci c antibodies (MSA), are positive in 30-45% of patients with a myopathic in ammatory disease (3,15), PM/DM patients with positive ARS had higher prevalence of ILD than those without such antibodies. ARS can also be positive in ILD patients who don't meet criteria of in ammatory myositis of any other CTDs, who were considered as idiopathic interstitial pneumonitis (IIP) with positive ARS antibodies, or could be classi ed as interstitial pneumonia with autoimmune features (IPAF) since 2015 (16).…”

Section: Discussionmentioning

confidence: 99%

Clinical features of Anti-synthetase syndrome associated interstitial lung disease: a retrospective cohort in China

Zhan¹,

Yan

Wang

et al. 2020

Preprint

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Background Antisynthetase Syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, interstitial lung disease (ILD). ASSD is highly heterogenous due to the different organs involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behavior of the ASSD) associated ILD. Methods Retrospectively collected the data of 108 cases of ASSD associated ILD in Beijing Chaoyang Hospital since 2017.12 to 2019.3. Data including age, gender, physical examination, laboratory test, pulmonary function and High Resolution Computer Tomography (HRCT), treatment, were obtained from the Electronic Medical Record (EMR) system. Patients were divided into 5 groups according to the distinct Aminoacyl tRNA synthetases (ARS) antibodies, and all patients had a multiple discussion team (MDT) to make a radiological and pathological diagnosis of the ILD pattern. Each patient had at least 1 follow up for no less than 6 months. Patients with missing data of MDT evaluation or follow up were excluded. Results: 108 consecutive patients were recruited in this retrospective cohort. 30 cases received bronchoscopy for a transbronchial lung biopsy. 3 had bronchoscopy for a transbronchial cryobiopsy. 33 were with JO-1 positive and 30 were with PL-7 positive. 23 with EJ positive, 13 with PL12 positive and 9 with OJ positive. JO-1 group had a significant higher rate of mechanic’s hand (57.6%) than other 4 groups, the skin involvement (Gottron Papules and/or Heliotrope Rash) was found in 38 (35%) and no difference was found among the 5 groups. Polymyositis/Dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The anti-PL7 positive group had a higher frequency of UIP pattern (13.3%) than other 4 groups but the difference was not significant due to the small sample size, and EJ group had the most frequent OP pattern(78.2%), which was significantly higher than PL-7 group (P<0.001) and PL-12 group (P=0.025). The median follow-up time were 10.7 months. All received prednisone treatment, with or without immunosuppressants ,and at the 6-month-follow up, the JO-1 group and EJ group had the significantly higher improvement of forced vital capacity that the other 3 groups (P<0.05), and PL-7 group had the lowest FVC improvement (P<0.05). The anti-Jo1-positive group and anti-EJ-positive group had significantly higher anti-RO52-positive occurrence than other 3 groups (P<0.05).Conclusion Anti PL-7 antibody had the same frequency as anti-JO1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. All ASSD-ILD responded to therapy of steroids, with or without immunosuppressants. PL-7 group had a highest occurrence of UIP pattern, and significantly lower respondence to therapy.

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Treatment of idiopathic inflammatory myositis associated interstitial lung disease: A systematic review and meta-analysis

Cited by 114 publications

References 60 publications

Lung Diseases in Inflammatory Myopathies

Lung Diseases in Inflammatory Myopathies

Acute Exacerbation of Interstitial Lung Disease in Adult Patients With Idiopathic Inflammatory Myopathies: A Retrospective Case-Control Study

Clinical features of Anti-synthetase syndrome associated interstitial lung disease: a retrospective cohort in China

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