Nomogram to predict dermatomyositis prognosis: a population-based study of 457 cases

Abstract

ObjectiveDermatomyositis (DM) is a systemic autoimmune disease, which typically affects the striated muscle with a variable involvement of the skin and other organs. Clinically amyopathic DM (CADM) is a combination of hypomyopathic DM (HDM) and amyopathic DM (ADM), with a characteristic of skin-predominant lesions. To date, large-scale studies on the prognostic factors of DM/CADM have been limited. The aim of this study is to evaluate the prognostic values of clinical manifestations in DM/CADM and to develop a prognostic nomogram for DM/CADM. Methods A development cohort (n =239), an internal validation cohort (n=128) and an external validation cohort (n=90) were included in this study. Overall survival (OS) was estimated by the Kaplan-Meier method. Univariate and multivariate Cox proportional hazards regression analyses were performed. Cox proportional hazards model and forward stepwise selection with the Akaike information criterion were used for multivariate analysis of prognostic factors. The concordance index(C-index) and calibration curve were calculated to evaluate the predictive accuracy of the proposed nomogram. Results Rapidly progressive interstitial lung disease (RP-ILD) and erythrocyte sedimentation rate (ESR) were identified as risk independent prognostic factors, with antinuclear antibodies (ANA) was identified as protective independent prognosticfactors, for DM/CADM. A prognostic nomogram was formulated based on these three predictors. The C-index of the proposed nomogram in the development cohort was 0.874 (95%CI, 0.819-0.929). The predictive accuracy of the proposed nomogram was further validated in the internal validation cohort, with a C-index of 0.799 (95%CI,. Furthermore, the C-index was 0.864 (95%CI, 0.699-1.000) in the external validation cohort, indicating a good calibration ability. This proposed nomogram showed a promising predictive accuracy on the prognosis of DM/CADM. ConclusionRP-ILD, ANA and ESR are prognostic factors for DM/CADM. The proposed nomogram based on these three factors could accurately predict the 10-year OS probabilities of patients with DM/CADM.

An externally validated clinical-laboratory nomogram for myocardial involvement in adult idiopathic-inflammatory-myopathy patients

“…Compared with merely identifying risk factors, establishment of a predictive model would not only assist in deepening our comprehensive understanding of IIMs and its complications, but also provide an efficient and instant tool for clinical practice. In preceding studies concerning adult or juvenile IIMs, various predictive models for prognosis, positivity of anti-MDA5 antibody, complication of hypertension, RP-ILD, sHLH and malignancy [ 19 , 42 – 45 ] have been constructed. To date, this is the first study seeking to construct a comprehensive clinical model for prediction and early identification of MI in IIMs patients.…”

Clinical significance of serum antinuclear antibodies in patients with autoimmune hepatitis and comorbidity

“…4-5), which is rarely reported. Some studies reported that ANA was related to the prognosis of immune diseases, such as systemic lupus erythematosus 33 , dermatomyositis 34 . Notably, whether or not ANA could predict the prognosis outcome of AIH remains to be further studied.…”

Clinical significance of serum antinuclear antibodies in patients with autoimmune hepatitis and comorbidity

“…4 , 5 ), which is rarely reported. Furthermore, some studies also reported that ANA was related to the prognosis of immune diseases [ 35 , 36 ], such as systemic lupus erythematosus, dermatomyositis. However, whether or not ANA could predict the prognosis outcome of AIH remains to be further studied.…”