Polymyositis/dermatomyositis is a potential risk factor for acute respiratory failure: a pulmonary heart disease

Syue, Shih-Huei; Chang, Yu Chi; Shih, Pei-Ju; Lin, Cheng‐Li; Yeh, Jun-Jun; Kao, Chia‐Hung

doi:10.21037/atm.2020.01.56

Cited by 4 publications

(4 citation statements)

References 49 publications

(74 reference statements)

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“…Thus, a high glucocorticoids (GC) dose is recommended to avoid myositis while keeping the treatment period as short as possible [8]. This burden could be the reason for the difference in the choice of treatment between two LoTs, which is also reported in previous studies that comorbidities do influence treatment choice and might also further complicate treatment operation for patients who switched multiple therapies in the past [26]. Other studies have also reported systemic corticosteroids as the most recommended initial therapeutic agent in PM/DM patients [8] while immunosuppressors being useful in inducing/maintaining remission/ experiencing intolerable side effects with steroids in DM patients [33,34].…”

Section: Discussionmentioning

confidence: 99%

“…In our study, 60% were women, consistent to other evidence [ 17 , 24 , 25 ], and frequently observed baseline comorbidities were heart diseases, respiratory diseases, diabetes mellitus, and hypertension. Studies from Taiwan [ 26 ] and Canada [ 27 ] reported high prevalence of CVD and pulmonary diseases as baseline comorbidity. Other reports also suggested that comorbid complications develop in majority of patients at their first PM/DM diagnosis before initiating first LoT, mainly systemic steroid therapy.…”

Section: Discussionmentioning

confidence: 99%

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Disease burden and treatment sequence of polymyositis and dermatomyositis patients in Japan: a real-world evidence study

Miyazaki¹,

Ishii²,

Stelmaszuk³

2021

Clin Rheumatol

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Introduction/objectives Since new consensus on polymyositis (PM) and dermatomyositis (DM) were released in Japan, an updated evidence on treatment landscape and PM/DM burden was essential. This study evaluates treatment burden and overall treatment cost of PM/DM-related inpatient and outpatient visits, treatments, and procedures/patient/year. Method This retrospective, observational study analyzed insurance claims from Japan Medical Data Center (JMDC) database. Patients with at least one PM/DM diagnosis/one dispensation of treatment between 1 January 2009 and 31 December 2019 were enrolled. Patient characteristics, treatment patterns and sequence, treatment choices, healthcare resource utilization (HCRU), and related costs were assessed. Chi-square test and linear regression model were used to assess impact of patient characteristics on treatment choice. Results Patients (836/4,961) receiving a relevant treatment were analyzed. Heart disease (35%), interstitial lung disease (27%), and diabetes mellitus (26%) were frequently identified as comorbidities. Concomitant dispensation of immunosuppressants and systemic steroids was largely found in first and second line of treatment (LoT) while systemic steroids remained as single dominant treatment across all LoTs. HCRU was very low for inpatient visits (0.68 [1.43]) or rehabilitation (4.74 [14.57]). The mean (SD) number of inpatient visits decreased from first (1.23 [2.32]) to third year (0.11 [0.54]). Total mean (SD) healthcare cost per patients per year was ¥ 3,815,912 (7,412,241), with overall drug dispensation compounding to 80% of total cost. Conclusions High concomitant immunosuppressant and systemic steroid prescriptions in first LoT recommend early optimal treatment to manage PM/DM. Although inpatient costs are low, outpatient dispensation costs increase overall economic burden. Key Points• Japan faces treatment challenges in the prognosis of polymyositis (PM) and dermatomyositis (DM) and thus, we assessed the real-world treatment landscape, practice, patterns, and healthcare resource utilization as an evidence to support healthcare outcome improvement and treatment burden reduction.• Systemic steroids were the dominant preferred choice of treatment, but it increases the overall cost of the treatment due to the resultant comorbidities considering possible side-effects promotion.• Thus, an increased awareness towards the disease management among patients and medical doctors is required for better management goals based on this real-world practice evidence.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Disease burden and treatment sequence of polymyositis and dermatomyositis patients in Japan: a real-world evidence study

Miyazaki¹,

Ishii²,

Stelmaszuk³

2021

Clin Rheumatol

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“…ILD, especially PF-ILD, may develop into pulmonary heart disease, including pulmonary embolism, pulmonary artery hypertension (PAH), and pulmonary circulation disease—pulmonary vascular diseases (PVD) [ 3 , 4 , 5 ]. Meanwhile, infections, such as those that are viral, may be concurrent with or associated with the initiation or exacerbation of ILD; this vicious coexistence may also contribute to PVD ( Figure 1 ) [ 3 , 5 , 6 , 7 , 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

“…However, the prolonged use of HCQ has been implicated in the development of conduction disturbances and myocardial dysfunction, leading to heart failure [ 11 ]. Owing to the cross-reaction of the heart and lung, heart failure was predisposed to PAH [ 6 ]. Moreover, long-term HCQ use may contribute to respiratory muscle weakness, leading to RF [ 12 ].…”

Section: Introductionmentioning

confidence: 99%

Hydroxychloroquine on the Pulmonary Vascular Diseases in Interstitial Lung Disease: Immunologic Effects, and Virus Interplay

et al. 2022

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To investigate the effects of hydroxychloroquine (HCQ) drug use on the risk of pulmonary vascular disease (PVD) in an interstitial lung disease cohort (ILD cohort, ILD+ virus infection), we retrospectively enrolled the ILD cohort with HCQ (HCQ users, N = 4703) and the ILD cohort without HCQ (non-HCQ users, N = 4703) by time-dependence after propensity score matching. Cox models were used to analyze the risk of PVD. We calculated the adjusted hazard ratios (aHRs) and their 95% confidence intervals (CIs) for PVD after adjusting for sex, age, comorbidities, index date and immunosuppressants, such as steroids, etc. Compared with the HCQ nonusers, in HCQ users, the aHRs (95% CIs) for PVD were (2.24 (1.42, 3.54)), and the women’s aHRs for PVD were (2.54, (1.49, 4.35)). The aHRs based on the days of HCQ use for PVD of 28–30 days, 31–120 days, and >120 days were (1.27 (0.81, 1.99)), (3.00 (1.81, 4.87)) and (3.83 (2.46, 5.97)), respectively. The medium or long-term use of HCQ or young women receiving HCQ were associated with a higher aHR for PVD in the ILD cohort. These findings indicated interplay of the primary immunologic effect of ILD, comorbidities, women, age and virus in the HCQ users.

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Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis

Palmucci¹,

Mari²,

Cancemi³

et al. 2022

Medicina

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Polymyositis and dermatomyositis are autoimmune idiopathic systemic inflammatory diseases, characterized by various degrees of muscle inflammation and typical cutaneous lesions—the latter found in dermatomyositis. The underlying pathogenesis is characterized by a high level of uncertainty, and recent studies suggest diseases may have different immunopathological mechanisms. In polymyositis, components of the cellular immune system are involved, whereas in dermatomyositis, the pathogenesis is mainly mediated by the humoral immune response. The interstitial lung disease occurs in one-third of polymyositis and dermatomyositis patients associated with worse outcomes, showing an estimated excess mortality rate of around 40%. Lung involvement may also appear, such as a complication of muscle weakness, mainly represented by aspiration pneumonia or respiratory insufficiency. The clinical picture is characterized, in most cases, by progressive dyspnea and non-productive cough. In some cases, hemoptysis and chest pain are found. Onset can be acute, sub-acute, or chronic. Pulmonary involvement could be assessed by High Resolution Computed Tomography (HRCT), which may identify early manifestations of diseases. Moreover, Computed Tomography (CT) appearances can be highly variable depending on the positivity of myositis-specific autoantibodies. The most common pathological patterns include fibrotic and cellular nonspecific interstitial pneumonia or organizing pneumonia; major findings observed on HRCT images are represented by consolidations, ground-glass opacities, and reticulations. Other findings include honeycombing, subpleural bands, and traction bronchiectasis. In patients having Anti-ARS Abs, HRCT features may develop with consolidations, ground glass opacities (GGOs), and reticular opacities in the peripheral portions; nonspecific interstitial pneumonia or nonspecific interstitial pneumonia mixed with organizing pneumonia have been reported as the most frequently encountered patterns. In patients with anti-MDA5 Abs, mixed or unclassifiable patterns are frequently observed at imaging. HRCT is a sensitive method that allows one not only to identify disease, but also to monitor the effectiveness of treatment and detect disease progression and/or complications; however, radiological findings are not specific. Therefore, aim of this pictorial essay is to describe clinical and radiological features of interstitial lung diseases associated with polymyositis and dermatomyositis, emphasizing the concept that gold standard for diagnosis and classification–should be based on a multidisciplinary approach.

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Polymyositis/dermatomyositis is a potential risk factor for acute respiratory failure: a pulmonary heart disease

Cited by 4 publications

References 49 publications

Disease burden and treatment sequence of polymyositis and dermatomyositis patients in Japan: a real-world evidence study

Disease burden and treatment sequence of polymyositis and dermatomyositis patients in Japan: a real-world evidence study

Hydroxychloroquine on the Pulmonary Vascular Diseases in Interstitial Lung Disease: Immunologic Effects, and Virus Interplay

Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis

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