Clinical features and poor prognostic factors of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with rapid progressive interstitial lung disease

Motegi, Sei‐ichiro; Sekiguchi, Akiko; Toki, Sayaka; Kishi, Chikako; Endo, Yukie; Yasuda, Masahito; Ikeuchi, Hidekazu; Sakairi, Toru; Hara, Kenichiro; Yamaguchi, Kazutaka; Maeno, Toshitaka; Hiromura, Keiju; Ishikawa, Osamu

doi:10.1684/ejd.2019.3634

Cited by 88 publications

(61 citation statements)

References 27 publications

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“…1,2 Regarding cutaneous manifestations, skin ulcers, palmar violaceous papules/macules and antihelices/helices violaceous macules have been reported to be associated with DM patients with anti-MDA5 Ab. 5,37,[39][40][41][42][43] However, we did not find any association between plasma homocysteine levels and these clinical features associated with anti-MDA5 Ab in DM patients.…”

Section: Discussioncontrasting

confidence: 88%

Plasma homocysteine levels are positively associated with interstitial lung disease in dermatomyositis patients with anti‐aminoacyl‐tRNA synthetase antibody

et al. 2020

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Homocysteine is a sulfhydryl-containing amino acid that is derived from dietary methionine, and there has been increasing evidence that elevated plasma homocysteine levels are associated with increased risk of central and peripheral vascular disorders, including carotid, coronary and peripheral arterial diseases, and Raynaud's phenomenon. Recently, associations of plasma homocysteine levels with autoimmune diseases such as systemic lupus erythematodes and systemic sclerosis have been reported. However, no study analyzed the association between plasma homocysteine levels and dermatomyositis (DM). The objective of this study was to examine plasma homocysteine levels and their clinical associations in patients with DM. Plasma homocysteine levels in 28 Japanese patients with DM and 22 healthy controls were examined. We found that the plasma homocysteine levels in DM patients were significantly higher than those in healthy individuals (15.8 AE 1.1 vs 8.5 AE 0.5 µmol/L, P < 0.01). Presence of mechanic's hand, complication of interstitial lung disease (ILD), high serum Krebs von den Lungen-6 (KL-6), surfactant protein-D and creatine kinase levels, and anti-aminoacyl-tRNA synthetase (ARS) antibody (Ab) positivity were significantly more prevalent among DM patients with elevated plasma homocysteine levels. The plasma homocysteine levels in DM patients with mechanic's hand, ILD and anti-ARS Ab were significantly higher than those in DM without those features. Furthermore, the plasma homocysteine levels were positively correlated with serum KL-6 levels. These results suggest that the pathogenesis of elevated plasma homocysteine levels may be associated with ILD in DM patients, especially with anti-ARS Ab, and further examination is required.

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Section: Discussioncontrasting

confidence: 88%

Plasma homocysteine levels are positively associated with interstitial lung disease in dermatomyositis patients with anti‐aminoacyl‐tRNA synthetase antibody

et al. 2020

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“…Although our research showed that relatively lower WBC counts and CRP levels are risk factors, the magnitude of its impact on prognosis is relatively limited. Numerous studies have con rmed that elevated serum ferritin is an independent risk factor for RP-ILD and poor prognosis in IIM patients [16,17,26,[29][30][31][32]. Our results showed that the elevation of serum ferritin still brings a trend of poor prognosis in IIM patients admitted to the ICU because of acute respiratory failure, although the statistical difference is not signi cant enough.…”

Section: Discussionmentioning

confidence: 44%

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

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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.

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“…Anti-MDA5, acute onset and susceptibility to disease progression were representative features in cluster 4. Among DM patients in the U.S. and Japan, 13.1-37.3% were positive for anti-MDA5 [43,44]. Anti-MDA5 was found to be associated with rapidly progressive ILD and poor survival with mortality as high as 71.4% [43].…”

Section: Discussionmentioning

confidence: 97%

“…Anti-MDA5 was found to be associated with rapidly progressive ILD and poor survival with mortality as high as 71.4% [43]. The initial triple therapy, including high-dose prednisolone, calcineurin inhibitor and intravenous cyclophosphamide were commonly used in survivors of rapidly progressive ILD with anti-MDA5 than in non-survivors, indicating that initial triple therapy may improve prognosis of these patients [44]. For IP patients with anti-MDA5 who failed to respond to triple therapy, combined treatment with Tofacitinib might be bene cial in controlling disease progression [45].…”

Section: Discussionmentioning

confidence: 99%

Phenotypic Clusters and Survival Analyses in Interstitial Pneumonia with Myositis-Specific Autoantibodies

et al. 2020

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Abstract Background: Interstitial pneumonia (IP) is one of the common pulmonary complications of idiopathic inflammatory myopathy (IIM), among which myositis-specific autoantibodies (MSA) are specific for IIM diagnosis and prognosis. However, IP patients with MSA (MSA-IP) have not been well described. The study aimed to explore the phenotypic clusters and prognosis of MSA-IP patients.Methods: A total of 124 MSA-IP patients were prospectively enrolled for analysis. Serum MSA were detected using immunoprecipitation. Radiographic patterns of IP were determined according to the classification of idiopathic IPs. The clusters of MSA-IP patients were identified using cluster analysis. Potential risk factors of acute onset and short-term prognosis were also analyzed. Results: There were four clusters of MSA-IP patients. Cluster 1 patients were elders with chronic onset and usual interstitial pneumonia pattern on CT scan. Cluster 2 patients were all positive for anti-aminoacyl-tRNA antibodies, predominantly females and had frequent respiratory symptoms. Patients of cluster 3 showed multi-system involvements with nonspecific interstitial pneumonia pattern. Patients of cluster 4 had severe respiratory symptoms with anti-MDA5. The patients of cluster 3 (OR 6.682, 95% CI 1.560–28.622, P=0.011) or cluster 4 (OR 6.057, 95% CI 1.715–21.388, P=0.005) were susceptible to acute onset. The patients of cluster 4 were prone to disease progression (HR 2.711, 95% CI 1.128–6.519, P=0.034), which was consistent with the Kaplan–Meier curves.Conclusions: Four distinctive clusters were determined by cluster analysis suggesting the characteristics, serological antibodies and prognosis of MSA-IP.

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Clinical features and poor prognostic factors of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis with rapid progressive interstitial lung disease

Cited by 88 publications

References 27 publications

Plasma homocysteine levels are positively associated with interstitial lung disease in dermatomyositis patients with anti‐aminoacyl‐tRNA synthetase antibody

Plasma homocysteine levels are positively associated with interstitial lung disease in dermatomyositis patients with anti‐aminoacyl‐tRNA synthetase antibody

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

Phenotypic Clusters and Survival Analyses in Interstitial Pneumonia with Myositis-Specific Autoantibodies

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