Objective This study aims to analyze the clinical characteristics of idiopathic inflammatory myopathy (IIM) associated interstitial lung disease (ILD) with positive anti melanoma differentiation-associated gene 5 (MDA5) antibody. Methods The clinical data of 91 patients with IIM in Shanxi Bethune Hospital from Jan 2019 to Mar 2022 were collected. Then various comparisons and analyzed retrospectively in the clinical symptoms, laboratory indicators and imaging characteristics between the negative MDA5-IIM and the positive anti-MDA5-IIM. ResultsCompared with the negative anti-MDA5-IIM patients, the significantly higher incidences of Gottron sign (71.0% vs. 38.3%), ulcerative rash (45.2% vs.16.7%), dyspnea (64.5% vs. 36.7%), and ILD (71.0% vs. 43.3%), in the positive anti-MDA5-IIM patients (P<0.05). Compared with the negative anti-MDA5-IIM patients, the more ground-glass opacification (GGO) in the positive anti-MDA5-IIM patients on high resolution computed tomography (81.8% vs.53.8%, P<0.05). Compared with the positive anti-MDA5-IIM patients without ILD, the positive anti-MDA5-IIM patients with ILD had significantly higher incidence of Gottron sign (77.3% vs. 33.3%), the increased levels of lactate dehydrogenase [(525.20±203.09) IU/L vs. (321.73±188.88) IU/L], Krebs von den Lungen-6 [(1399.55±869.64) U/mL vs. (371.56±128.92) U/mL], and D-dimer [(1226.65±902.31) U/L vs. (703.73±160.11) U/L], as well as higher positive rate of anti-Ro-52 antibody (68.2% vs. 22.2%) and the higher case fatality rate (54.5% vs. 0.0%), with all P<0.05. The triple treatment of high-dose glucocorticoid (GC) combined with intravenous cyclophosphamide (IVCY) and another immunosuppressive agent (tacrolimus or tofacitinib) was found to be effective in the MDA5-IIM associated ILD group, with a lower case fatality rate (28.6%) than high-dose GC combined with IVCY group (47.1%). Conclusions The positive anti-MDA5-IIM with ILD of patients have the higher incidence of rash and dyspnea, with GGO as the most common imaging manifestation. When rapidly progressive interstitial lung disease (RPILD) is combined, the case fatality rate higher. Meanwhile, early intensive immunosuppressive therapy may improve the survival rate of RPILD patients with positive anti-MDA5 antibody.
Objective To evaluate the clinical relationship between serum carcinoembryonic antigen (CEA) and mortality of anti-melanoma differentiation associated gene 5 (MDA5) antibody positive dermatomyositis with interstitial lung disease (ILD). MethodsThe consecutive clinical data of 214 patients with anti MDA5 antibody positive dermatomyositis from West China Hospital of Sichuan University from February 2017 to September 2019 were collected retrospectively, including demographic, laboratory examination and imaging examination data. Patients were divided into CEA elevated group (CEA≥4.63 ng/mL) and CEA normal group (CEA<4.63 ng/mL) according to CEA level. R4.1.2 software was used for statistical analysis of all data, and Kaplan Meier method was used to draw the survival curve. Cox proportional hazard model was used to analyze the survival of patients with ILD, and to explore the risk factors associated with the survival of patients with anti-MDA5 antibody positive dermatomyositis with ILD. Results There were 180 patients with ILD who met the inclusion and exclusion criteria, 57 patients with rapidly progressive pulmonary interstitial fibrosis (RPILD), and 123 patients without RPILD; 121 women and 59 men, with an average age of 50.2±10.7 years; The average follow-up was 23.5 months, and 52 patients died. Univariable analysis suggested that CEA≥4.63 ng/mL, smoking, RPILD, lactate dehydrogenase (LDH) ≥321 IU/L, albumin<30 g/L and dyspnea were risk factors associated with death in patients with anti MDA5 dermatomyositis combined with ILD. Multivariable Cox regression analysis showed that CEA≥4.63 ng/mL [hazard ratio (HR) =3.01, 95% confidence interval (CI) 1.23 - 7.32, P=0.015], RPILD (HR=3.87, 95%CI 2.09 - 7.19, P<0.001), smoking (HR=2.37, 95%CI 1.25 - 4.47, P=0.008), LDH≥321 IU/L (HR=2.47, 95%CI 1.23 - 4.96, P=0.011), albumin<30 g/L (HR=2.57, 95%CI 1.38 - 4.78, P=0.003) were independent predictors for mortality. ConclusionsSerum CEA level can be used as a clinical prognostic predictor in patients with anti-MDA5 positive dermatomyositis and ILD. RPILD, smoking, LDH≥321 IU/L, and albumin<30 g/L are independent predictors for mortality.