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Objective: To explore the clinical characteristics, efficacy and safety of different therapeutic regimens in patients with anti-MDA5 antibody positive dermatomyositis (anti-MDA5⁺DM) stratified by disease risk, and to analyze the influencing factors for prognosis. Methods: This was a retrospective cross-sectional study. Clinical data of 82 patients with anti-MDA5⁺DM admitted to the First Affiliated Hospital of Guangxi Medical University from January 2018 to June 2024 were retrospectively analyzed. All patients were stratified into low-risk group and intermediate-high-risk group based on the FLAIR model, a predictive model for mortality risk in amyopathic dermatomyositis combined with interstitial lung disease. Meanwhile, patients were divided into four groups according to treatment regimens: Regimen A: glucocorticoids combined with calcineurin inhibitors and cyclophosphamide; Regimen B: glucocorticoids combined with Janus kinase inhibitors with or without calcineurin inhibitors; Regimen C: glucocorticoids combined with calcineurin inhibitors; Regimen D: glucocorticoids combined with cyclophosphamide. The clinical manifestations, laboratory parameters, infection events and survival outcomes were compared among groups with different risk stratification and different treatment regimens. Multivariate logistic regression analysis was performed to identify clinical indicators independently associated with all-cause mortality. Results: Among the 82 patients with anti-MDA5⁺DM, 19 (23.2%) were males and 63 (76.8%) were females, with a median age of 50 years (range, 37-63 years). The incidence of cough was higher in the intermediate-high risk group [44 cases (74.6%)] than that in the low-risk group [10 cases (43.5%)] (χ²=7.12, P=0.008). The intermediate-high risk group also exhibited higher frequencies of expectoration [35 cases (59.3%) vs. 7 cases (30.4%), χ²=5.53, P=0.019], dyspnea [37 cases (62.7%) vs. 8 cases (34.8%), χ²=5.21, P=0.022] and fever [36 cases (61.0%) vs. 6 cases (26.1%), χ²=8.08, P=0.004]. Median serum ferritin level was 1 545.3 (936.8, 2 376.9) μg/L in the intermediate-high risk group versus 442.8 (138.9, 759.0) μg/L in the low-risk group (Z=-4.89, P<0.001). The intermediate-high risk group had significantly elevated median levels of lactate dehydrogenase [371.0 (302.0, 473.0) vs. 242.0 (208.0, 318.0) U/L, Z=-4.95, P<0.001], creatine kinase [87.0 (60.0, 254.0) vs. 45.0 (34.0, 113.0) U/L, Z=-2.83, P=0.005], carcinoembryonic antigen [6.8 (3.2, 12.5) vs. 3.8 (2.4, 6.0) μg/L, Z=-2.32, P=0.021], aspartate aminotransferase [74.0 (47.0, 133.0) vs. 45.0 (31.0, 55.0) U/L, Z=-3.44, P=0.001], C-reactive protein [7.6 (2.6, 16.3) vs. 2.4 (0.7, 5.0) mg/L, Z=-3.24, P=0.001] and erythrocyte sedimentation rate [42.0 (23.8, 63.5) vs. 23.0 (13.0, 34.0) mm/1 h, Z=-3.36, P=0.001].The prevalence of periorbital edematous erythema was significantly lower in the intermediate-high risk group [29 cases (49.2%)] compared with the low-risk group [17 cases (73.9%)] (χ²=4.12, P=0.042). The median immunoglobulin M level was markedly decreased in the intermediate-high risk group [1.2 (0.9, 1.6) g/L] relative to the low-risk group [1.7 (1.2, 2.8) g/L] (Z=-2.66, P=0.008).Complete follow-up data were obtained from 78 patients. The all-cause mortality rate (13.0% vs. 38.2%) and infection rate (6.7% vs. 57.1%) of the low-risk group were obviously lower than those of the intermediate-high-risk group. Within each risk stratum, there was no statistically significant difference in the incidence of severe infections among the four treatment regimens. Regimen A was associated with the highest survival rate among patients in the intermediate-high-risk group. Taking Regimen A as the reference, Regimens B, C and D were correlated with higher mortality risk, with corresponding OR values as follows: Regimen B OR=0.07 (95%CI 0.01-0.43, P=0.002), Regimen D OR=0.07 (95%CI 0.01-0.62, P=0.008), Regimen C OR=0.06 (95%CI 0.01-0.41, P=0.001).Multivariate logistic regression analysis revealed that rapidly progressive interstitial lung disease (RP-ILD) and dysphagia were independently correlated with mortality outcomes: RP-ILD (OR=24.95, 95%CI 1.47-422.55, P=0.026), dysphagia (OR=11.62, 95%CI 1.23-110.15, P=0.033). Positive anti-Ro52 antibody (OR=6.40, 95%CI 0.90-45.47, P=0.063) and elevated C-reactive protein (OR=1.04, 95%CI 0.99-1.10, P=0.091) showed a trend toward higher mortality risk. Conclusions: In patients with intermediate-high-risk anti-MDA5⁺DM, inflammatory biomarkers and clinical symptoms were positively associated with adverse mortality outcomes. RP-ILD and dysphagia were independently correlated with all-cause mortality. Among intermediate-high-risk patients, the regimen combining glucocorticoids, calcineurin inhibitors and cyclophosphamide was associated with higher survival probability, without an increased incidence of severe infections.