Initial predictors of poor survival in myositis-associated interstitial lung disease: a multicentre cohort of 497 patients

Sato, Shinji; Masui, Kenichi; Nishina, Naoshi; Kawaguchi, Yasushi; Kawakami, Atsushi; Tamura, Masahito; Ikeda, Kei; Nunokawa, Takahiro; Tanino, Yoshinori; Asakawa, Katsuaki; Kaneko, Yuko; Gono, Takahisa; Ukichi, Taro; Kaieda, Shinjiro; Naniwa, Taio; Kuwana, Masataka; investigators, Jami

doi:10.1093/rheumatology/key060

Cited by 121 publications

(109 citation statements)

References 40 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…For example, characteristics of anti‐ARS‐associated ILD include a better response to initial treatment, frequent relapse during the tapering of the dosage of corticosteroids, and poor long‐term prognosis because of deterioration of pulmonary function due to progression of ILD . On the other hand, patients with anti‐MDA5 antibody frequently develop rapidly progressive ILD, and ~30% of them die within 3 months after diagnosis due to respiratory failure . In contrast, it has been reported that ILD is relatively uncommon in DM patients without anti‐ARS or anti‐MDA5 antibodies .…”

Section: Introductionmentioning

confidence: 99%

Two cases with autoantibodies to small ubiquitin‐like modifier activating enzyme: A potential unique subset of dermatomyositis‐associated interstitial lung disease

et al. 2019

Self Cite

View full text Add to dashboard Cite

| INTRODUC TI ONDermatomyositis (DM) is characterized by inflammatory myopathy with typical skin rashes, such as Gottron`s papules or sign and heliotrope rash. Myositis-specific autoantibodies (MSAs) are detected in ~80% of patients with DM 1 and include anti-aminoacyl tRNA synthetase (ARS), anti-melanoma differential-associated gene 5 (MDA5), anti-Mi-2, antinuclear matrix protein 2 (NXP2), anti-transcription intermediary factor 1-γ (TIF1-γ), and anti-small ubiquitinlike modifier activating enzyme (SAE). 2 Basically, MSAs are mutually exclusive and are closely linked to unique clinical features, including the distribution and severity of organ involvement, response to treatment, and prognosis. 3 Interstitial lung disease (ILD) is one of the major organ involvements and is associated with poor prognosis in patients with DM. Among MSAs, anti-ARS and anti-MDA5 antibodies are strongly related to the presence of ILD but to different forms of ILD. 3 For example, characteristics of anti-ARS-associated ILD include a better response to initial treatment, frequent relapse during the tapering of the dosage of corticosteroids, and poor long-term prognosis because of deterioration of pulmonary function due to progression of ILD. 3 On the other hand, patients with anti-MDA5 antibody frequently develop rapidly progressive ILD, and ~30% of them die within 3 months after diagnosis due to respiratory failure. 4 In contrast, it has been reported that ILD is relatively uncommon in DM patients without anti-ARS or anti-MDA5 antibodies. 3,5,6 However, AbstractThe presence of anti-aminoacyl tRNA synthetase (ARS) or anti-melanoma differential-associated gene 5 (MDA5) is strongly related to interstitial lung disease (ILD) in patients with dermatomyositis (DM). Several studies suggest a potential relationship between ILD and anti-small ubiquitin-like modifier activating enzyme (SAE) antibody in DM patients, but detailed clinical characteristics of anti-SAE-associated ILD still remain unknown. We have experienced 2 cases who were positive for anti-SAE antibody, who presented with ILD in the context of clinically amyopathic DM. These 2 patients had the following common ILD characteristics: an insidious course with preserved pulmonary function; a limited extent of pulmonary lesions with subpleural peripheral-dominant small ground glass opacity/consolidation on high-resolution computed tomography; and a favorable treatment response. These findings suggest that anti-SAE-associated ILD is unique in terms of clinical and imaging features and differs from ILD associated with anti-ARS or anti-MDA5 antibody. K E Y W O R D Santi-small ubiquitin-like modifier activating enzyme antibody, dermatomyositis, human leukocyte antigen, interstitial lung disease

show abstract

Section: Introductionmentioning

confidence: 99%

Two cases with autoantibodies to small ubiquitin‐like modifier activating enzyme: A potential unique subset of dermatomyositis‐associated interstitial lung disease

et al. 2019

Self Cite

View full text Add to dashboard Cite

show abstract

“…Patients with anti-MDA5 antibody positive have the worst prognosis of all IIM types. Anti-MDA5 provided a 7.5-fold increase in the risk of mortality in the Japanese largest multicenter retrospective cohort of patients with IIM-ILD [14]. Data from the largest Chinese single-center PM/DM-ILD cohort also showed that the 5-year survival rate of patients with anti-MDA5 antibody positive is also signi cantly lower than patients with anti-ARS antibodies positive [5].…”

Section: Discussionmentioning

confidence: 90%

Outcomes and prognostic factors of patients with anti-melanoma differentiation-associated protein 5 and anti-aminoacyl-RNA synthetase antibodies positive idiopathic inflammatory myopathies in intensive care unit

Wang

Zhang

Ren

et al. 2020

Preprint

View full text Add to dashboard Cite

Background: This study aims to identify prognostic factors for mortality of patients with anti-melanoma differentiation-associated protein 5 (anti-MDA5) or anti-aminoacyl-RNA synthetase (anti-ARS) antibodies positive and acute respiratory failure in the intensive care unit.Methods: Clinical characteristics, laboratory test findings, imaging performance, and management were retrospectively collected in all cases with anti-MDA5 and anti-ARS antibodies positive, as well as follow-up survival data. Risk factors related to prognosis were identified by Cox regression analysis.Results: The 28-day mortality of all patients was 68.8% (n=44/64). The patients who died were more likely to have anti-MDA5 antibody(p<0.001), presented more Gottron papules(p=0.021) or heliotrope rash(p=0.008), had a relatively lower level of WBC(p=0.038), CRP(p=0.004), and had a higher level of LDH(p=0.029), serum ferritin(p=0.002). The main risk factors associated with 28-day mortality were anti-MDA5 antibody positive [HR 10.827 (95% CI: 4.261-27.514), p<0.001], presence of Gottron papules [2.299 (1.203-4.394), p=0.012], heliotrope rash [3.423 (1.773-6.606), p<0.001], and arthritis/arthralgia [2.365 (1.130-4.948), p=0.022). At a median of 14 (IQR 6.33-35.0) months of follow-up, the overall mortality of all patients was 75.0% (n=48/64). The non-survivors were more likely to own anti-MDA5 antibody(p<0.001), had a higher rate of Gottron papules(p=0.020) or heliotrope rash(p=0.014), had lower PFR(p=0.032) while ICU admission, and existed a higher level of serum ferritin(p=0.005). Main risk factors associated with overall mortality were consistent with risk factors for 28-day mortality. Conclusions: Anti-MDA5 antibody positive, presence of Gottron papules, heliotrope rash, or arthritis/arthralgia were the main independent risk factors of poor prognosis for IIM patients admitted to the ICU due to acute respiratory failure.

show abstract

“…Similar to IPF, rheumatoid arthritis associated with ILD (RA‐ILD) can be associated with MUC5B, a minor allele, with an odds ratio of 3.8 and is linked explicitly to UIP pattern on HRCT. Idiopathic inflammatory myopathy (IMM) may also affect the lung, and CD4 + CXCR4+ T cells are a novel prognostic biomarker in IMM‐ILD while disease progression has been studied recently . A retrospective work about radiographic fibrotic score showed that a high radiographic fibrosis score is a poor prognostic factor in systemic sclerosis (SScl) …”

Section: Connective Tissue Disease Associated With Ildmentioning

confidence: 99%

Contemporary Concise Review 2018: Interstitial lung disease

2019

View full text Add to dashboard Cite

Initial predictors of poor survival in myositis-associated interstitial lung disease: a multicentre cohort of 497 patients

Abstract: We established a large cohort of incident cases of PM/DM-associated ILD, and successfully identified independent predictors of short-term ILD mortality.

Cited by 121 publications

References 40 publications

Two cases with autoantibodies to small ubiquitin‐like modifier activating enzyme: A potential unique subset of dermatomyositis‐associated interstitial lung disease

Two cases with autoantibodies to small ubiquitin‐like modifier activating enzyme: A potential unique subset of dermatomyositis‐associated interstitial lung disease

Outcomes and prognostic factors of patients with anti-melanoma differentiation-associated protein 5 and anti-aminoacyl-RNA synthetase antibodies positive idiopathic inflammatory myopathies in intensive care unit

Contemporary Concise Review 2018: Interstitial lung disease

Contact Info

Product

Resources

About