Clinical features and prognosis of anti-MDA5-positive dermatomyositis with coexistent anti-aminoacyl-tRNA synthetase antibodies.
This study aimed to evaluate the incidence and clinical significance of coexistence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibody in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5 + DM).
We assessed a cohort of 246 consecutive patients with anti-MDA5 + DM. Clinical characteristics and survival rates were compared between patients with and without anti-ARS antibodies.
Of these 246 patients, 15 (15/246, 6.1%) were positive for anti-ARS antibodies. The anti-ARS-positive group had a higher proportion of mechanic's hands (53.3% vs 25.5%, P = 0.019), and the positivity rates for antinuclear antibody (ANA) (80.0% vs 50.6%, P = 0.033) and anti-Ro52 antibodies (93.3% vs 62.3%, P = 0.013) were also higher. However, the CRP levels were comparatively low [4.0 (0.5, 7.8) vs 6.0 (3.1, 12.2), P = 0.019]. In the overall population, no difference in survival rates was observed between anti-MDA5 + DM patients with or without anti-ARS antibodies (log-rank P = 0.339). The multivariate Cox regression analysis revealed that elevated lactate dehydrogenase (LDH) levels and the presence of rapidly progressive interstitial lung disease (RPILD) were associated with poor prognosis [hazard ratios of 1.002 (95% CI 1.001, 1.002, P < 0.001) and 11.096 (95% CI 5.006, 24.598, P < 0.001), respectively].
Among the 246 patients with anti-MDA5 + DM, only 15 patients (6.1%) tested positive for anti-ARS antibodies. The presence of anti-ARS antibodies is associated with the occurrence of mechanic's hands, as well as an increased positive rate of ANA and anti-Ro52 antibodies. Elevated LDH levels and the presence of RPILD are poor prognostic risk factors for patients with anti-MDA5 + DM. Key Points • The presence of anti-ARS antibodies is associated with the occurrence of mechanic's hands, as well as an increased positive rate of ANA and anti-Ro52 antibodies. • Elevated LDH levels and the presence of RPILD are poor prognostic risk factors for patients with anti-MDA5 + DM. • Understanding the clinical characteristics of these patients will help clinicians to develop individualised treatment plans for each patient.
Wang G
，Yan D
，Weng C
，Xue L
，Liu Z
... -
《-》
Clinical features and survival analysis of 40 cases of anti-MDA5 antibody-positive dermatomyositis complicated with interstitial lung disease.
Several studies have shown that patients with anti-MDA5 antibody-positive dermatomyositis (anti-MDA5 antibody + DM) have an increased risk of developing rapid progression of interstitial lung disease (RPILD), which is associated with poor prognosis and high mortality. However, diagnosis and treatment are often delayed due to atypical early clinical features and heterogeneity. Therefore, clinical features should be identified to establish a prognosis model for early identification and intervention, thereby improving the clinical prognosis of patients.
The study aimed to investigate the clinical features, risk factors, treatment strategy, and construct a survival prognosis model for anti-MDA5 antibody + DM patients with ILD.
A total of 40 anti-MDA5 antibody + DM-ILD patients admitted to the Department of Pulmonary and Critical Care Medicine and the Department of Rheumatology and Immunology in the Second Affiliated Hospital of Xi 'an Jiaotong University from September 2018 to May 2022 were retrospectively analyzed. Prognostic factors correlated with overall survival (OS) during hospitalization were identified by multivariate Cox regression analysis, and a nomogram was established. The nomogram was internally validated using C-index and time-dependent (at 1-, 2-, and 3- months) calibration curves with 1000 iterations of bootstrap resampling. Moreover, the optimal truncation values for continuous variables and Kaplan-Meier (K-M) curves were determined, which were used to analyze the difference in survival between groups. Finally, time-dependent decision curve analysis (DCA) was employed to validate the clinical value of the nomogram.
Significant differences were found between the survival group and the non-survival group in terms of age, oxygenation index, extent of lung lesions, diffuse alveolar damage (DAD) and nonspecific interstitial pneumonia (NSIP), and LDH, GLU, CEA, ferritin, CRP levels in serum (P < 0.05). Multivariate regression analysis revealed that increased NSIP in high-resolution computed tomography (HRCT) and ALT,LDH,CEA,CRP were risk factors for poor prognosis (P < 0.05). A nomogram diagram was constructed according to the final multiple Cox model to predict the 1-, 2-, and 3-month OS. According to ALT, AST, LDH, CEA, and CRP cutoff values, the KM algorithm was used to estimate the survival curve (P < 0.05). DCA curves were drawn for the model-dependent variables included treatment style, NSIP, ALT, AST, LDH, CEA, and CRP. This indicated that the nomogram yielded a higher net benefit compared to other single prognostic factors, and the cutoff value grouping model showed better practical application value. Combined treatment with glucocorticoids and immunosuppressants was a protective factor for long-term survival. Survival analysis indicated that patients with anti-MDA5 + DM-ILD could benefit from combined treatment for longer survival.
Anti-MDA5 antibody + DM is prone to interstitial lung disease, poor prognosis, and high mortality. Risk prediction model could help us paying attention to these features which may allow the early identification of high-risk patients and promote timely diagnosis and treatment.
Zhao K
，Zhang J
，Kong Q
，Zhang Y
，Li C
，Huo K
，Fan N
，Deng W
，Shi J
，Wang C
，Li X
，Yang S
，Fang P
... -
《-》
Prognostic analysis of MDA5-associated clinically amyopathic dermatomyositis with interstitial lung disease.
To investigate the prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (MDA5) positive clinically amyopathic dermatomyositis (CADM) and interstitial lung disease (ILD).
A retrospective analysis was conducted on clinical data of 125 patients with anti-MDA5 + CADM-ILD collected from 10 branches in eastern China between December 2014 and December 2022. Prognostic factors were analyzed using χ2 test, Log-rank test, COX and logistic regression analysis.
In this cohort, 125 anti-MDA5 + CADM-ILD patients exhibited a rapidly progressive interstitial lung disease (RPILD) incidence of 37.6%, and an overall mortality rate of 24.8%. One patient was lost to follow-up. After diagnosis of RPILD, a mortality rate of 53.2% occurred in patients died within 3 months, and that of 5.6% appeared in those who survived for more than 3 months. Multiple factor analysis revealed that C-reactive protein (CRP) ≥ 10 mg/L (p = 0.01) and recombinant human tripartite motif containing 21 (Ro52) (+) (p = 0.003) were associated with a higher risk of RPILD in anti-MDA5 + CADM-ILD patients; CRP ≥ 10 mg/L (p = 0.018) and the presence of RPILD (p = 0.003) were identified as the factors influencing survival time in these patients, while arthritis was the protective factor (p = 0.016).
Patients with anti-MDA5 + CADM-ILD will have a higher mortality rate, and the initial 3 months after diagnosis of RPILD is considered the risk window for the dismal prognosis. Patients with CRP ≥ 10 mg/L, Ro52 (+) and RPILD may be related to a shorter survival time, while patients complicated with arthritis may present with relatively mild conditions.
Wang W
，Sun X
，Xu Y
，Tan W
，Liu Y
，Zhou J
... -
《Immunity Inflammation and Disease》
ResearchGate
(全网免费下载)
钛学术
(全网免费下载)
