FEA Medicina Interna. Hospital General Universitario de Elche. Enfermedades Autoinmunes Sistémicas/Autoinflamatorias/ Minoritarias. Profesor asociado en UMH.
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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The 2025 update of EULAR recommendations for the management of #Behçet’s syndrome, recently published in Annals of the Rheumatic Diseases, include 5 overarching principles and 12 recommendations organised by organ involvement.
◦ Overarching principles stress the relapsing-remitting nature of the disease, individualised treatment based on activity and prognostic factors, a multidisciplinary approach, patient education, and shared decision-making.
◦ Key recommendations:
- Mucocutaneous and joint involvement: Colchicine is first-line; apremilast or TNFα inhibitors for refractory cases.
- Organ-threatening disease: Early aggressive therapy with glucocorticoids + immunosuppressives, with prompt use of monoclonal TNFα inhibitors encouraged for severe or life-threatening manifestations.
Of the 12 recommendations, 1 is entirely new, 7 were modified, and 4 had only wording changes.
*Hatemi G, Ramiro S, Ozguler Y, et al. EULAR recommendations for the management of Behçet’s syndrome: 2025 update. Ann Rheum Dis. Published online March 23, 2026.
🔗https://t.co/dVblStDVGn