Peripheral lymphocyte count defines the clinical phenotypes and prognosis in patients with anti‐MDA5‐positive dermatomyositis

Abstract

Z, et al. Peripheral lymphocyte count defines the clinical phenotypes and prognosis in patients with anti-MDA5-positive dermatomyositis.

Triple Positive for Anti-MDA5, Anti-EJ and Anti-Ro52 Antibodies Dermatomyositis Associated Interstitial Lung Disease：a case report

“…In a large Chinese cohort study of patients with MDA5-DM, patients were classi ed into three subgroups according to peripheral lymphocyte count. The results proved severe lymphopenia is strongly associated with high mortality, and a normal peripheral lymphocyte number at baseline predicts a better prognosis [4] . However, a single indicator fails to accurately predict a particular outcome, a prediction model incorporating several relevant risk factors may enhance predictive power.…”

Proteomic profiling identifies SPP1 associated with rapidly progressive interstitial lung disease in anti-MDA5-positive dermatomyositis

“…Anti-MDA5 antibody-positive dermatomyositis (MDA5 + DM), a distinct subtype of idiopathic inflammatory myopathies (IIM), is significantly associated with interstitial lung disease (ILD), particularly the rapidly progressive interstitial lung disease (RP-ILD). MDA5 + DM with RP-ILD is often resistant to traditional treatment, and despite aggressive interventions, the mortality rate is still as high as 50-70% [ 1 , 2 ]. Hence, early identification of high-risk RP-ILD patients is a vital aspect of improving prognosis.…”

“…MDA5 + DM is a heterogeneous disease, with substantial variability in clinical presentation, treatment response, and patient outcomes. About 30 ~ 50% of patients exhibited the life-threatening RP-ILD [ 1 , 3 ], conversely, while about 60% of patients MDA5 + DM displayed mild symptoms with good prognosis. Therefore, accurate prediction of patients at high risk of RP-ILD may aid in decision-making on therapeutic strategies and assist in preventing overtreatment of “low-risk” patients.…”

“…Male, old age, short disease duration, skin ulceration, and forced vital capacity are suggested as risk factors for RP-ILD [ 2 , 4 ]. Some serum markers like the co-existence of anti-MDA5 antibody and anti-RO-52 antibody, elevated serum C-reactive protein (CRP), ferritin level, and Krebs von den Lungen-6 (KL-6) levels have been linked to poor outcome in RP-ILD patients [ 1 , 2 , 5 ]. However, due to the exact mechanisms underlying RP-ILD development have not yet been determined, most prognostic risk research mainly focused on the epidemiological and clinical characteristics of MDA5 + DM.…”

From machine learning to clinical practice: phenotypic clusters of anti-MDA5 antibody-positive dermatomyositis

“…Rather than describing phenotypes, clusters and decision trees provide better guidance for clinical practice if they follow commonly accepted definitions of MDA5-DM and RP-ILD, use a few easily and objectively measured clinical characteristics known at the time of diagnosis, predict a patient’s prognosis reliably and are validated by replication. As a striking illustration of this viewpoint, one of the four studies demonstrated that phenotypic clusters performed equally well if they were based on lymphocyte count only instead of a multitude of characteristics [ 5 ]. Another study, not included in the review, showed similar accuracy of phenotypic clusters based on immune cell phenotypes [ 7 ].…”