The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients

Chen, Xixia; Zhang, Lu; Jin, Qiwen; Lü, Xin; Lei, Jieping; Peng, Qinglin; Wang, Guochun; Ge, Yongpeng

doi:10.3389/fimmu.2022.987841

Cited by 6 publications

(6 citation statements)

References 37 publications

(51 reference statements)

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“…HRCT scan patterns were classified as nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia (UIP), and diffuse alveolar damage (DAD) by 2 experienced radiologists. RP‐ILD was defined as a worsening of radiologic interstitial changes with progressive dyspnea and hypoxemia within 3 months after the onset of respiratory symptoms 14 …”

Section: Methodsmentioning

confidence: 99%

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Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

Zhang,

Wang,

Zhou

et al. 2023

Immunity Inflam & Disease

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ObjectivesTo identify the differences of clinical characteristics, laboratory findings, and the long‐term outcomes in patients with anti‐synthetase syndrome (ASS) of different anti‐aminoacyl‐transfer RNA synthetase antibodies.MethodsWe retrospectively enrolled 119 patients with ASS, and the clinical characteristics and laboratory findings were collected. Additionally, multivariate COX regression analysis was performed to estimate the risk factors of prognosis in patients with ASS.ResultsThe frequency of interstitial lung disease (ILD) reached 93.3% in our cohort, of 28 (23.5%) was classified as rapidly progressive (RP)‐ILD. The highest incidence of RP‐ILD was 36.4% in the PL12 group of ASS patients. The ILD group was characterized by an older age, a lower prevalence of V sign, and a higher prevalence of pulmonary symptoms when contrasted with the non‐ILD group. There were statistical differences of clinical significance in arthritis, myositis, mechanic's hands, triad, shawl sign, V sign, and Raynaud's phenomenon among the four subgroups (all p < .05). Additionally, the prevalence rates of arthritis, myositis, mechanic's hands, triad, and V sign in the anti‐Jo1 antibody‐positive group were significantly higher than anti‐Jo1 antibody‐negative patients with ASS (all p < .05). Multivariate Cox regression analysis showed mechanic's hands (odds ratio [OR] = 6.47, p < .001), anti‐nuclear antibodies (ANA) (OR = 2.13, p = .026), ILD (OR = 10.50, p < .001), and V sign (OR = 0.30, p = .007) were independent factors affecting the prognosis of patients with ASS. The incidences of RP‐ILD, arthritis, myositis, triad, mechanic's hands, and shawl sign were more frequent in the anti‐Ro52 antibody‐positive group than the anti‐Ro52 antibody‐negative patients with ASS (all p < .05).ConclusionsPatients with ASS accompanied with ILD are highly prevalent. Mechanic's hands, ANA, and ILD may be a potential biomarker for predicting a poor prognosis in patients with ASS. Additionally, the detection of the anti‐Ro52 antibody provides valuable insights for managing and predicting disease progression and long‐term outcomes.

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Section: Methodsmentioning

confidence: 99%

“…RP-ILD was defined as a worsening of radiologic interstitial changes with progressive dyspnea and hypoxemia within 3 months after the onset of respiratory symptoms. 14…”

Section: Definitionsmentioning

confidence: 99%

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

Zhang,

Wang,

Zhou

et al. 2023

Immunity Inflam & Disease

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“…The result presented that anti-MDA+/ARS + group had higher ferritin levels than Anti-MDA5-/ARS + group and higher CD4 + T-cell counts than Anti-MDA5+/ARS-group. Individuals with anti-MDA+/ARS + antibodies combined the features of anti-MDA5 + and anti-ARS + individuals and respond well to glucocorticoid therapy [14] . A latest review summarized the lung manifestations of MDA5 + DM, commonly including organizing pneumonia, which typically presents with subpleural consolidation during the early stage of the disease, non-speci c interstitial pneumonia and non-speci c interstitial pneumonia-organizing pneumonia overlap [2] , while grid shadows and pleura thicken seldom appeared as our case.…”

Section: Completely Learning Of Clinical Conditions Is Helpful For Pr...mentioning

confidence: 99%

Triple Positive for Anti-MDA5, Anti-EJ and Anti-Ro52 Antibodies Dermatomyositis Associated Interstitial Lung Disease：a case report

Guo,

Du,

et al. 2024

Preprint

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Background Clinically amyopathic dermatomyositis (CADM) is a disease with the typical skin manifestations of dermatomyositis (DM) and few or no obvious muscle weakness. The anti-melanoma differentiation associated protein 5（MDA5）antibody positive CADM patients are at a high risk of developing rapidly progressive interstitial lung disease (RP-ILD)，and exhibit immensely poor prognosis and high mortality. The disease course and severity of MDA5+CAMD are highly different, wherein some patients presented marked treatment response and didn’t occur exacerbation. Analysis to these special groups bring us better understanding of MDA5+CADM. Case presentation We presented a patient with anti-MD5 anti-Ro52 anti-EJ-triple positive DM patient. She was initially characteristic with darken skin for 2 years before gradually occurred dry cough, fever and dyspnea. She had a low oxygen saturation and diffusing capacity of the lungs for carbon monoxide. With combination therapy of GCs and two immunosuppressants, the woman obtained a rapid remission and persisted for a long time. Conclusions Patients with anti-MD5 anti-Ro52 anti-EJ-triple positive DM-ILD may present a less aggressive disease course and an individual appropriate therapy may be promising for long-term maintenance.

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“…The presence of these Abs correlates with distinct clinical manifestations, malignancy, and interstitial lung disease, and are predictive of organ manifestations and potentially of prognosis. Anti-TIF1g, Anti-MDA5, and Anti-SAE1/2 are MSAs associated with severe cutaneous involvement in DM, and Anti-Mi2a, Anti-Mi2b, Anti-NPX2, and Anti-SAE1/2 are MSAs associated with intense muscle involvement in DM [1,2,[17][18][19][20][21].…”

Section: Introductionmentioning

confidence: 99%

Trichoscopic, oral, and periungual fold findings as activity and damage markers in dermatomyositis patients and their correlation with myositis antibodies

et al. 2023

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There is little clarity about the clinical manifestations of dermatomyositis (DM) in the periungual folds, scalp, and oral cavity and their association with disease activity and damage. The objective of this study was to compare the prevalence of trichoscopic, oral, and periungual changes between DM and healthy patients and assess their possible association with disease activity and damage. We conducted an observational, transversal, and analytical study between 2020 and 2021. Forty DM patients were matched by sex and age with 40 healthy individuals. On the same day, all patients had a clinical evaluation of the hands, periungual folds, scalp, and oral cavity. Photographs of these areas and peripheral venous blood tests, including myositis-associated (MAAs) and myositis-specific antibodies (MSAs), were taken. Two dermatologists blinded to their diagnosis, damage, and activity levels registered the lesions. The disease activity and damage were evaluated using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI). The presence of mechanic’s hands, Gottron’s sign, and Gottron’s papules in hands; capillary dilation, capillary tortuosity, cuticular hemorrhage, avascular areas, and cuticular hyperkeratosis in periungual folds; thick tortuous capillaries in scalp; gingival telangiectasias in the oral cavity; and positive MSAs associated with severe cutaneous involvement in DM patients (Anti-TIF1g, Anti-MDA5, Anti-SAE1/2) were associated with a higher CDASI activity score. The presence of MSAs associated with intense muscle involvement in DM patients (Anti-Mi2a, Anti-Mi2b, Anti-NPX2, and Anti-SAE1/2) was related to a lower CDASI activity score. Gottron’s sign and Gottron’s papules in hands; capillary dilation, capillary tortuosity, cuticular hemorrhage, avascular areas, and cuticular hyperkeratosis in periungual folds; basal erythema in scalp; and gingival telangiectasias in the oral cavity were associated with a higher CDASI damage score. There are trichoscopic, oral and periungual fold findings and some myositis-specific antibodies that correlate with disease activity and damage in DM patients. Supplementary Information The online version contains supplementary material available at 10.1007/s00403-023-02554-0.

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The clinical features and prognoses of anti-MDA5 and anti-aminoacyl-tRNA synthetase antibody double-positive dermatomyositis patients

Cited by 6 publications

References 37 publications

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

Triple Positive for Anti-MDA5, Anti-EJ and Anti-Ro52 Antibodies Dermatomyositis Associated Interstitial Lung Disease：a case report

Trichoscopic, oral, and periungual fold findings as activity and damage markers in dermatomyositis patients and their correlation with myositis antibodies

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