Anti-MDA5 antibody testing takes two to eight weeks and fails to detect 17% of cases — so in rapidly progressive ILD, the skin exam is the faster test. This review covers the pathognomonic and characteristic DM rashes, the specific findings that flag anti-MDA5, TIF1γ and NXP2, why DM-specific rashes appear in antisynthetase and overlap myositis, presentations in skin of colour, and the treatments for refractory cutaneous disease.
There are no PJP prophylaxis guidelines for rheumatic disease, so practice varies widely between clinicians. This review assembles what the evidence supports: prophylaxis clearly works, the harms are real enough to matter, and the number needed to treat swings from about 4 in VEXAS to over 900 in rheumatoid arthritis — which is why the underlying disease, not the steroid dose alone, should drive the decision.
A 2026 Personal View arguing that ‘seronegative’ usually describes the assay rather than the patient — and that separating genuinely seronegative disease (myeloid/CD8-driven, MHC class I-associated, rituximab-unresponsive) from merely undetected seropositivity is the field’s necessary next step. Includes a disease-by-disease audit of seronegativity rates and why classification criteria naming the antibody but not the method matters.
The VALOR phase 3 trial shows that brepocitinib 30 mg daily — a selective TYK2–JAK1 inhibitor — significantly improves composite disease activity, skin involvement, and muscle strength while enabling steroid discontinuation in adults with treatment-refractory dermatomyositis, marking the first successful phase 3 RCT of an oral targeted agent in this disease.
A contemporary NEJM review of idiopathic inflammatory myopathies — the five-subtype framework (IBM, IMNM, antisynthetase syndrome, overlap, dermatomyositis), the autoantibody revolution that now anchors classification, and the shift toward subtype-specific therapy with biologics, JAK/TYK2 inhibitors, and CAR-T.
CD19 CAR-T cellular therapy delivers drug-free remission in 80–85% of refractory SLE patients in early-phase trials, with similar signals in IIM and SSc — the first credible attempt at true immunological reset in autoimmune rheumatic disease.