Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

Li, Ting; Guo, Li; Chen, Zhiwei; Gu, Liyang; Sun, Fangfang; Tan, Xiaoming; Chen, Sheng; Wang, Xiaodong; Ye, Shuang

doi:10.1038/srep33226

Cited by 77 publications

(65 citation statements)

References 20 publications

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“…In contrast with other MSAs, 20 which are associated with infrequent pulmonary involvement, anti-MDA5 antibodies are associated with rapidly progressive forms of ILD (i.e., acute or subacute ILD), potentially leading to death. 21,22 One report suggests that pirfenidone added to corticosteroids (CSs) and/or immunosuppressive therapy may be beneficial in patients with rapidly progressive ILD associated with CADM 23 ; however, this observation warrants confirmation.…”

Section: Anti-mda5 Autoantibodies-associated Ildmentioning

confidence: 99%

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: Anti-mda5 Autoantibodies-associated Ildmentioning

confidence: 99%

Lung Diseases in Inflammatory Myopathies

Barba

Mainbourg

Nasser

et al. 2019

Semin Respir Crit Care Med

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“…These two clinical trials and the long-term follow-up of the subjects showed that the most common adverse events were skin-related like rash and gastrointestinal-related containing nausea and dyspepsia [9][10][11]. Research has been conducted on the use of pirfenidone in other types of pulmonary fibrosis, such as scleroderma-associated interstitial lung disease [12][13][14] and clinically amyopathic dermatomyositis [15], and potential benefits were reported.…”

mentioning

confidence: 99%

Real-world experiences: Efficacy and tolerability of pirfenidone in clinical practice

et al. 2020

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BackgroundThe safety of pirfenidone on pulmonary fibrosis patients with other kinds of interstitial lung diseases (ILDs) in addition to idiopathic pulmonary fibrosis (IPF) is unknown. Furthermore, its effectiveness-related factors on IPF patients are not quite explored. MethodsA retrospective study, on patients prescribed pirfenidone for pulmonary fibrosis, was conducted to assess effectiveness on IPF patients and tolerability of all patients with lung fibrosis. The effectiveness of pirfenidone was tested on 110 IPF subjects receiving treatment for �3 months by high-resolution computed tomography (HRCT). Response-linked factors and progression-free survival (PFS) were also analyzed. The data about safety outcomes and drug dose adjustments were collected from all included subjects. ResultsA total of 176 subjects were included: 117 were IPF, 19 connective tissue disease-associated interstitial lung disease (CTD-ILD), and 40 unclassifiable ILD. Out of the 110 IPF subjects, 89 subjects were assessed as stable and 21 as progressive, out of which 10 died of acute exacerbation and 11 progressed. The effectiveness was significantly related to their baseline body mass index (BMI). IPF subjects with BMI>25kg/m 2 or diffusion capacity of carbon monoxide (DLco)>30% had higher PFS rate. The most common adverse events were skin-related and gastrointestinal-related. Drug discontinuation owing to adverse events occurred similarly in these three groups.

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“…ILD in DM/PM proceeds rapidly and the condition is usually severe, such as rapidly progressive ILD (RP-ILD) [11] [12]. We didn't find the reports about the correlation between miR-200c, miR-125b and ILD in DM/PM.…”

Section: Introductionmentioning

confidence: 92%

miR-125b, miR-200c Are Correlated with the Severity of Interstitial Lung Disease in Dermatomyositis/Polymyositis

Jiang¹,

Tao²,

Li³

2018

OJRA

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Objective: To explore the correlations between miR-125b, miR-200c, and the severity of interstitial lung disease associated with dermatomyositis/polymyositis (DM/PM-ILD). Methods: 30 consecutive patients with DM/PM and 23 healthy controls were recruited into current study. Anti-JO-1, anti-SSA, muscle enzymes, the data of chest HRCT and pulmonary function test were collected. 9 consecutive DM/PM-ILD patients underwent bronchoalveolar lavage (BAL). TGF-β1 and surfactant protein D (SP-D) in BAL fluid (BALF) and plasma were detected by ELISA. miR-125b and miR-200c in PBMCs and bronchoalveolar cells were detected by QRT-PCR. All patients were classified into three groups: Mild or non-ILD group, moderate ILD group, and severe ILD group. The correlations between miRNAs and the severity of ILD, the lung damage markers, auto-antibodies, were analyzed. Results: The levels of miR-125b and miR-200c in bronchoalveolar cells were higher than in PBMCs, and the levels of TGF-β1 and SP-D were higher in BALF than in plasma in DM/PM-ILD patients. There were positive correlations between miR-125b, miR-200c in bronchoalveolar cells and SP-D in BALF. The levels of miR-125b and miR-200c in severe ILD group were higher than in mild or non-ILD and moderate ILD groups. There were negative correlations between miR-125b, miR-200c, and FEV1, and between miR-200c and DLCO. The patients with anti-JO-1 antibody had higher levels of miR-125b and miR-200c, and had more severe condition of ILD. Conclusion: miR-125b and miR-200c were positively correlated with the lung damage and severity of ILD in DM/PM, which could be important markers for judgement of disease condition in clinic.How to cite this paper: Jiang, Z., Tao, J.H. and Li, X.P. (2018)

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Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis

Cited by 77 publications

References 20 publications

Lung Diseases in Inflammatory Myopathies

Lung Diseases in Inflammatory Myopathies

Real-world experiences: Efficacy and tolerability of pirfenidone in clinical practice

miR-125b, miR-200c Are Correlated with the Severity of Interstitial Lung Disease in Dermatomyositis/Polymyositis

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