One year in review 2020: idiopathic inflammatory myopathies

ObjectiveThe clinical features of myositis specific antibody negative dermatomyositis (MSA negative DM) varied greatly, and there were few reports in the literatures. This study aimed to describe and expand the clinical phenotypes and prognoses of MSA negative DM patients.Methods MSA negative DM patients were identified from January 2010 to June 2020. We retrospectively reviewed the clinical features and laboratory data. The survival status was followed up until July 31. 2020 SPSS version 21.0 and R version 3.6.1 software were used for the statistical analyses. ResultsA total of 97 MSA negative DM patients were enrolled. The most common type of rashes was heliotrope rash (80.4%). More than half of the patients (55.7%) had interstitial lung disease (ILD), and seven of them developed rapid progressive ILD. There were eleven patients with tumours. During the follow-up, twelve patients died, of whom 5 (41.7%) died due to infection. Two phenotypes of MSA negative DM patients were identified by cluster analysis. Patients in cluster 1 developed muscle weakness, mechanic's hands, arthritis, and ILD more frequently. Patients in cluster 2 had a higher incidence of heliotrope rashes. Patients in cluster 1 tended to have worse prognoses, wherein the 1-year and 5-year survival rates (81.1% and 78.4%, respectively) were lower than those in cluster 2 (97.6% and 95.2%, respectively), with p-value 0.04 and 0.056, respectively. ConclusionThrough cluster analysis, different clinical phenotypes of MSA negative DM patients were determined. The prognoses of the two subgroups were different in terms of survival rate and cause of death.

Section: Discussionmentioning

confidence: 99%

Clinical heterogeneities and prognoses of patients with myositis specific antibody negative dermatomyositis: a retrospective study in China

Gao

Chen

et al. 2022

“…Recently, the 2017 EULAR/ACR classification criteria for IIM and their subgroups have been proposed (22). The differences in the pathogenesis and in the clinical phenotype indicated the heterogeneity and complexity of IIM, which should be considered as a group of different diseases and not as a single disease (49). Lately, machine learning is being utilised to unravel the complexity of IIM (21,50,51).…”

Section: Discussionmentioning

confidence: 99%

A retrospective cohort study in Chinese patients with adult polymyositis and dermatomyositis: risk of comorbidities and subclassification using machine learning

Zhu

Zhou

et al. 2022

ObjectiveTo identify the risk factors in Chinese patients with adult polymyositis and dermatomyositis for their comorbidities and explore a subclassification system. Methods Clinical records of 397 patients with idiopathic inflammatory myopathies were retrospectively reviewed. Logistic regression was used to identify potential risk factors for interstitial lung disease (ILD), other rheumatic diseases, and malignancy after bivariate analysis. Hierarchical clustering and decisional tree were utilised to identify subgroups and explore a subclassification system. ResultsA total of 119 polymyositis and 191 dermatomyositis patients were included. Anti-PM/Scl, anti-Ro52, anti-aminoacyl-tRNA synthetase and anti-MDA5 (adjusted odds ratios (AOR)=4.779, 1.917, 5.092 and 7.714 respectively) antibodies were risks (p<0.05), whereas overlapping malignancy was protective (AOR=0.107; p=0.002) for ILD across polymyositis, dermatomyositis and the total group. In subgroup models, Raynaud's phenomenon, arthralgia and semi-quantitative anti-nuclear antibody (AOR=51. 233, 4.261, 3.047 respectively) were risks for other overlapping rheumatic diseases (p<0.05). For overlapping malignancy, male and anti-TIF1γ antibodies (AOR=2. 533, 16.949) were risks (p<0.05), whereas disease duration and combination of ILD (AOR=0.954, 0.106) were protective in the total group (p<0.05); while anti-NXP2 antibodies were identified as risk factors (AOR=73.152; p=0.038) in polymyositis. Hierarchical clustering suggested a subclassification with 6 subgroups: malignancy overlapping dermatomyositis, classical dermatomyositis, polymyositis with severe muscle involvement, dermatomyositis with ILD, polymyositis with ILD, and overlapping of myositis with other rheumatic diseases. ConclusionAccompanying ILD, other rheumatic diseases and malignancy are strongly associated with clinical manifestation and myositis-specific or myositis-associated autoantibodies among Chinese polymyositis and dermatomyositis patients.The subclassification system proposed a more precise phenotype defining toward stratified treatments.

“…Following our regular annual reviews on different aspects of rheumatology (5)(6)(7)(8)(9)(10)(11)(12) we will here provide a critical digest of the recent literature on Ax-SpA of 2019 and 2020 (Medline search of articles published from 1st January 2019 to 31st December 2020).…”

Section: Methodsmentioning

confidence: 99%

One year in review 2021: axial spondyloarthritis

Cardelli

Monti

Terenzi

et al. 2021

Axial spondyloarthritides (axSpA) are a group of systemic inflammatory rheumatic diseases with a broad spectrum of clinical manifestations and typical imaging features, rarely accompanied by laboratory abnormalities. They can be classified into a so-called non-radiographic form (nr-axSpA), unlike the radiographic one, because magnetic resonance imaging may show specific inflammatory lesions when conventional radiology is not able to highlight them. Inflammatory involvement of the axial skeleton tends to associate typically with new bone formation and peripheral joints may also be affected. Patients with axSpA are at higher risk of developing some typical extraarticular manifestations, particularly, acute anterior uveitis, psoriasis and inflammatory bowel disease. In this paper we review the literature on axSpA of 2019 and 2020 (Medline search of articles published from 1st January 2019 to 31st December 2020).