Strands

Strands

Autoimmune – adult vasculitis

Primary systemic small vessel vasculitis (PSV) refers to a group of rare autoimmune multi-system disorders with an annual incidence of 15/million and prevalence of approximately 2/10,000 and occurs primarily in adults with a median age of onset of 62. It presents acutely to a wide range of medical and surgical specialities and frequently presents a diagnostic dilemma as many clinicians will be unfamiliar with the condition, with delays in diagnosis of 6-12 months typical. Consequently, organ destruction progresses such that 30% end up with end-stage kidney disease and intensive care unit level care is often required due to multi-organ dysfunction.

However, early diagnosis of PSV and initiation of appropriate immunosuppressive therapy can substantially improve the associated morbidity and mortality, leading to a significant reduction in associated healthcare utilization costs. While effective treatments are available, PSV is a chronic relapsing condition with no cure that requires coordinated long-term multi-disciplinary input. In the “EULAR recommendations for management of primary small and medium vessel vasculitis” the first recommendation is to manage this condition in conjunction with centres of expertise with integrated expertise to manage relapses and complications of therapy.

To address this unmet need in Ireland and improve patient care, five centres across the Republic of Ireland developed a network of specialist units in collaboration with Vasculitis Ireland Awareness, the national vasculitis patient organisation. This network is comprised of dedicated multi-disciplinary centres that provide a coordinated care path from diagnosis to relapse and on to long-term remission, and provide access to clinical trials for patients with PSV. The five centres maintain close ties and difficult cases are often discussed in a cross-centre forum. RITA-Ireland's clinical guidance is aligned where appropriate with that of the UK and Ireland Vasculitis Rare Disease Group (UKIVAS). Please visit https://ukivas.ndorms.ox.ac.uk/ for further information.

• Primary Immunodeficiencies (PID)
• Autoinflammatory Disorders (AID)
• Autoimmune Diseases (AI)
  • Adult autoimmune vasculitis
  • Neuroinflammation
• Paediatric Rheumatology (PR)