Prognostic values of anti-Ro52 antibodies in anti-MDA5-positive clinically amyopathic dermatomyositis associated with interstitial lung disease

Xu, Antao; Yan, Yonghong; Fu, Qiong; Lian, Xin‐Yue; Chen, Sheng; Guo, Qiang; Lu, Liangjing; Dai, Min; Lv, Xia; Bao, Chunde

doi:10.1093/rheumatology/keaa786

Cited by 72 publications

(73 citation statements)

References 38 publications

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“…Anti-RO52 antibodies often co-exists with anti-MDA5 antibodies, and their occurrence correlates with a more severe clinical phenotype and poorer prognosis. 27 The positive rate of MSAs in this study was 70%, consistent with a previous study showing over 50% of positive rate of MSAs. 28 Among the 136 patients subjected to myositis antibodies test, 6 (4.4%) had more than one MSAs, which was higher than 0.2% in a previous study, 29 and was inconsistent with the mutual exclusivity of MSA.…”

Section: Discussionsupporting

confidence: 92%

Characterisation of Disease Patterns of Dermatomyositis with Different Initial Manifestations

Sun¹,

Li²,

Zhang³

et al. 2022

IJGM

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Purpose To determine the characteristics and prognoses of dermatomyositis (DM) by comparing the difference in initial symptoms. Patients and Methods A retrospective analysis was performed on the patients diagnosed with DM from 1 January 2019 to 1 January 2021. Based on the firstly presented symptoms, patients were divided into five groups, namely rash group, muscle weakness group, arthritis group, respiratory symptom group and atypical symptom group. Clinical and laboratory data were recorded. All patients were followed up until 31 May 2021. Results In total 136 DM patients, rash (40%) was the most common initial symptom, followed by respiratory symptoms (22%), arthritis (20%), muscle weakness (10%) and atypical symptoms (8%). Rash group and atypical group had a higher positive rate of anti-TIF1γ antibodies than arthritis group and respiratory symptom group ( P < 0.05). Respiratory symptom and arthritis groups had a higher positive rate of anti-Ro52 antibodies than rash and muscle weakness groups ( P < 0.05). Respiratory group had a higher incidence of ILD than rash and atypical groups. The FVC and DLCO in respiratory group were significantly lower than rash group, arthritis group and atypical group ( P < 0.05). The survival rate of rash group was significantly higher than muscle weakness group and arthritis group ( P < 0.05). Conclusion DM patients with different initial manifestations had different myositis antibodies and prognoses.

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Section: Discussionsupporting

confidence: 92%

Characterisation of Disease Patterns of Dermatomyositis with Different Initial Manifestations

Sun¹,

Li²,

Zhang³

et al. 2022

IJGM

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“…In addition, the anti-Ro52 antibody, a myositis-associated antibody, was also found to be quite prevalent in DM, especially in MDA5 + DM. It has also been related to higher mortality because of rapidly progressive interstitial lung disease ( 21 ). Actually, the anti-Ro52 antibody was also quite common in our analysis, with >50% incidence, although no significant difference was found between the deceased and survivors.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Intramuscular Hemorrhage in Anti-MDA5 Positive Dermatomyositis: A Case Series and Literature Review

et al. 2022

Front. Med.

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ObjectiveSpontaneous intramuscular hemorrhage (SIH) is a rare but life-threatening complication associated with dermatomyositis (DM). This study reported a case series of SIH associated with DM. In addition, the characteristics and prognostic effects for this complication were analyzed based on literature review.MethodsWe reported seven cases of anti-melanoma differentiation-associated gene five positive dermatomyositis (MDA5+DM) complicated by SIH in our single-center cohort, and a comprehensive literature review was performed. Clinical characteristics, treatment, and outcome data of all eligible reported cases were summarized. Potential prognostic effects were identified by comparisons between the deceased and survivors.ResultsAmong cumulatively reported patients with DM patients and SIH, the overall mortality was 60.9% (14/23) (including our cases). Fourteen out of nineteen (73.7%) hemorrhagic events occurred within 6 months of disease onset. Anti-MDA5 antibody predominated in those myositis-specific antibodies available cases (8/10), although patients with positive anti-NXP2 and anti-Mi2 have also been documented. Iliopsoas (52.2%, 12/23) was the most frequently involved bleeding location. Bleeding in deep muscles was identified to be associated with poorer prognosis. The mortality of patients with DM and deep muscular hematoma (non-palpable) (80%, 12/15) was significantly higher than that of patients with only superficial muscular hematoma (palpable) (25%, 2/8) (p =0.023).ConclusionSpontaneous hematoma in non-palpable deep muscles probably leads to excess mortality in dermatomyositis, particularly for those with anti-MDA5 antibody, which often occurs within 6 months of disease onset. Clinicians should be vigilant to this rare but potentially fatal complication and carefully balance the risks and benefits of prophylactic anti-thrombotic treatment.

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“…As reported in our systematic review, two out of two patients positive for anti-MDA5 antibodies died. In one case (23) anti-Ro52 antibodies, which are associated with rapidly progressive ILD and worse prognosis (51), were also present; the woman succumbed to macrophage activation syndrome, and there's no reason to attribute her death to COVID-19, since anti-MDA5 syndrome appeared two months after recovery from the infection. Meanwhile, the patient described by Gokhale et al (20) tested positive for anti-SAE1 antibodies, which are associated with ILD and increased cancer risk (52).…”

Section: Case Discussion and Pathogenic Hypothesismentioning

confidence: 99%

Anti-MDA5 Antibody Linking COVID-19, Type I Interferon, and Autoimmunity: A Case Report and Systematic Literature Review

et al. 2022

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IntroductionThe SARS-CoV-2 infection has been advocated as an environmental trigger for autoimmune diseases, and a paradigmatic example comes from similarities between COVID-19 and the myositis-spectrum disease associated with antibodies against the melanoma differentiation antigen 5 (MDA5) in terms of clinical features, lung involvement, and immune mechanisms, particularly type I interferons (IFN).Case ReportWe report a case of anti-MDA5 syndrome with skin manifestations, constitutional symptoms, and cardiomyopathy following a proven SARS-CoV-2 infection.Systematic Literature ReviewWe systematically searched for publications on inflammatory myositis associated with COVID-19. We describe the main clinical, immunological, and demographic features, focusing our attention on the anti-MDA5 syndrome.DiscussionMDA5 is a pattern recognition receptor essential in the immune response against viruses and this may contribute to explain the production of anti-MDA5 antibodies in some SARS-CoV-2 infected patients. The activation of MDA5 induces the synthesis of type I IFN with an antiviral role, inversely correlated with COVID-19 severity. Conversely, elevated type I IFN levels correlate with disease activity in anti-MDA5 syndrome. While recognizing this ia broad area of uncertainty, we speculate that the strong type I IFN response observed in patients with anti-MDA5 syndrome, might harbor protective effects against viral infections, including COVID-19.

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Prognostic values of anti-Ro52 antibodies in anti-MDA5-positive clinically amyopathic dermatomyositis associated with interstitial lung disease

Cited by 72 publications

References 38 publications

Characterisation of Disease Patterns of Dermatomyositis with Different Initial Manifestations

Characterisation of Disease Patterns of Dermatomyositis with Different Initial Manifestations

Spontaneous Intramuscular Hemorrhage in Anti-MDA5 Positive Dermatomyositis: A Case Series and Literature Review

Anti-MDA5 Antibody Linking COVID-19, Type I Interferon, and Autoimmunity: A Case Report and Systematic Literature Review

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