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The idiopathic inflammatory myopathies are a heterogeneous group of connective tissue diseases of unknown aetiology characterized by chronic inflammation of skeletal muscle, or myositis. The main types of idiopathic inflammatory myopathies are polymyositis, dermatomyositis and inclusion-body myositis.
In the phase 1/2 CASTLE basket trial, autologous CD19 CAR-T cell therapy in patients with treatment-refractory systemic lupus erythematosus, systemic sclerosis or idiopathic inflammatory myopathy was safe, with improved disease activity and patient-reported global health in most patients.
In a patient with treatment-refractory idiopathic inflammatory myositis, reinfusion of CD19 CAR T cells following disease relapse led to the development of T cells targeting the CD19 CAR and no clinical improvement, with drug-free remission induced again after treatment with BCMA CAR T cells.
This Review provides an update on autoantibodies associated with idiopathic inflammatory myopathies in both adults and children. The authors also discuss methods of autoantibody detection and the advantages and limitations of each technique.
Comparing dermatomyositis with cutaneous lupus erythematosus, single-cell analysis identifies monocyte-mediated endothelial injury as a dermatomyositis-specific feature and highlights JAK1 inhibition as a potential therapeutic approach.
In a phase III clinical trial of abatacept for idiopathic inflammatory myopathy, the primary endpoint was not met but the results suggest abatacept could have benefits in some subtypes of the disease.
The pathogenesis of juvenile dermatomyositis (JDM) is complex and various evidence implicate a role for type I interferons. Could the use of a bioengineered paediatric skeletal muscle model provide insight into this disease and have potential for high throughput testing of therapeutic agents?
Results indicate that prophylactic use of co-trimoxazole can reduce infection-related mortality in patients with new-onset anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis.
Using a combination of various single-cell and immune profiling approaches, researchers have characterized the unique adaptive immune landscape of anti-melanoma differentiation-associated gene 5 antibody-positive (MDA5+) dermatomyositis.
