FEA Medicina Interna. Hospital General Universitario de Elche. Enfermedades Autoinmunes Sistémicas/Autoinflamatorias/ Minoritarias. Profesor asociado en UMH.
Delighted to contribute to the 2025 EULAR recommendations for the management of PMR, GCA and TAK, now published in Annals of the Rheumatic Diseases. Honoured to work alongside outstanding colleagues from around the world. Special thanks to Prof. C Mukhtyar
https://t.co/CQXcY9Cp0Y
Should we classify AAV by clinical phenotype or ANCA specificity?
PR3-ANCA is associated with granulomatous disease (ENT, lungs), more relapses, and typically seen with GPA.
MPO-ANCA is linked to capillaritis (GN, alveolar hemorrhage, ILD), higher mortality, and most commonly MPA.
EGPA spans both ANCA-positive and ANCA-negative phenotypes, reflecting its biological heterogeneity.
ANCA specificity complements clinical phenotype, improving prognostication, patient stratification, and therapeutic decision-making.
The future of AAV classification isn’t syndrome vs serology - it’s the integration of both.
📖 Image Source: Nature Reviews Rheumatology
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The 2026 British Society for Rheumatology guideline for the management of children, young people and adults with systemic lupus erythematosus #Rheumatology#MedTwitter Full Ref Link below 👇
Presented at #EULAR2026:
Among patients with relapsed polymyalgia rheumatica, treatment with secukinumab plus a 24-week glucocorticoid taper resulted in a higher percentage of patients with remission than a glucocorticoid taper alone. Full phase 3 REPLENISH trial results: https://t.co/w1oD5ug1kJ
Editorial: Emerging Era for Polymyalgia Rheumatica and GCA — Interleukin-17A Targeting https://t.co/I13JtK01EA
Original Article in @NEJMEvidence: Secukinumab for Giant Cell Arteritis (phase 3 GCAptAIN trial) https://t.co/0dWjwDaEHs
@eular_org
Presented at #EULAR2026:
In a phase 3 trial, obexelimab led to a lower risk of disease flares than placebo among patients with IgG4-related disease. Glucocorticoid use was lower and the incidence of remission was higher with obexelimab than with placebo. Full INDIGO trial results: https://t.co/Lynf6nmA9N
Editorial: Obexelimab and the Promise of Nondepleting B-Cell Therapy in IgG4-Related Disease https://t.co/BUQ7r8jVWz
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🚨 Infliximab vs Cyclophosphamide in Severe Behçet’s Syndrome
This multicenter phase 2 Bayesian randomized controlled trial compared infliximab with cyclophosphamide in patients with severe Behçet’s syndrome.
🧪 Study Design
• 52 patients with severe Behçet’s syndrome
• Major vascular involvement: 71%
• Neuro-Behçet’s syndrome: 29%
Patients were randomized to receive:
🔹 Infliximab 5 mg/kg at weeks 0, 2, 6, 12, and 18
OR
🔹 Cyclophosphamide IV pulses every 4 weeks
All patients received glucocorticoids.
🎯 Primary Outcome
Complete response at week 22 defined as:
✅ Clinical remission
✅ Biological remission
✅ Radiological remission
✅ Prednisone dose ≤0.1 mg/kg/day
📊 Results
✅ Complete response achieved in:
• 81% with infliximab
• 56% with cyclophosphamide
📉 Overall adverse events:
• 29.6% with infliximab
• 64% with cyclophosphamide
⚠️ Serious adverse events were similar between groups.
💡 Key Takeaway
Infliximab demonstrated superior efficacy with fewer adverse events compared with cyclophosphamide, supporting TNF inhibition as a preferred induction strategy in severe Behçet’s syndrome with vascular or CNS involvement.
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VEXAS syndrome- Nature Reviews
⏩autoinflammatory syndrome caused by somatic UBA1 mutations ⏩mainly affecting older men with systemic inflammation, cytopenias & hematologic features
⏩Steroids help, but stem cell transplant may be curative.
https://t.co/opJTzCp9K5
Clinical Indications for ANCA Testing
ANCA testing should be considered when there is suspicion of ANCA-associated vasculitis (AAV), particularly in the following scenarios:
🫘 Renal
•Glomerulonephritis (especially rapidly progressive GN)
🫁 Pulmonary
•Pulmonary hemorrhage
•Pulmonary–renal syndrome
•Multiple lung nodules
🧴 Cutaneous
•Cutaneous vasculitis with systemic features
👃 ENT / Upper airway
•Chronic destructive disease of upper airways
•Long-standing sinusitis or otitis
•Subglottic tracheal stenosis
🧠 Neurological
•Mononeuritis multiplex
•Peripheral neuropathy
👁️ Ocular / Orbital
•Retro-orbital mass
•Scleritis
Order ANCA only when there is strong clinical suspicion of AAV-not as a screening test.
Source: Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis
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