Management of Vasculitis

Initial Assessment and Disease Categorization

The European League Against Rheumatism recommends referring patients to centers of expertise for management of primary small, medium, and large vessel vasculitis 1, 2
The American College of Rheumatology suggests performing ANCA testing (indirect immunofluorescence and ELISA) in appropriate clinical context for suspected ANCA-associated vasculitis 1
Obtaining tissue biopsy is recommended to confirm diagnosis—temporal artery biopsy for giant cell arteritis, skin/kidney/other affected organ biopsy for small/medium vessel vasculitis 1, 2
Categorizing ANCA-associated vasculitis by severity is crucial to guide treatment intensity, with categories including localized, early systemic, generalized, severe, and refractory 1, 3

The European League Against Rheumatism recommends using cyclophosphamide (2 mg/kg/day oral, max 200 mg/day, OR intravenous pulses) plus prednisolone (1 mg/kg/day, max 60 mg/day) for Wegener granulomatosis and microscopic polyangiitis 1, 3
Rituximab 375 mg/m² weekly for 4 weeks is equally effective as cyclophosphamide and is preferred for patients with fertility concerns or relapsing disease, achieving 64% complete remission at 6 months 1, 3
Providing Pneumocystis jiroveci prophylaxis (co-trimoxazole) during cyclophosphamide therapy is recommended 1
Using Mesna as uroprotective agent with cyclophosphamide to reduce bladder toxicity risk is suggested, though it does not eliminate risk entirely 1, 4

Initiating high-dose glucocorticoids immediately (prednisolone 1 mg/kg/day, typically 40-60 mg/day) for giant cell arteritis or Takayasu arteritis is recommended 2, 5
Adding methotrexate (10-15 mg/week for giant cell arteritis, 20-25 mg/week for Takayasu arteritis) to reduce relapse rate and cumulative glucocorticoid dose is suggested 2, 5
Prescribing low-dose aspirin (75-150 mg/day) for all giant cell arteritis patients to prevent cardiovascular and cerebrovascular events is recommended 2, 5

Performing structured clinical assessment, urinalysis, and basic laboratory tests at each visit is recommended 1, 6
Monitoring with clinical assessment and inflammatory markers (ESR, CRP) for large vessel vasculitis is suggested 2, 5
Considering periodic MRI or PET imaging for Takayasu arteritis disease activity assessment is recommended 2, 5
Investigating persistent unexplained hematuria in all patients with prior cyclophosphamide exposure due to bladder cancer risk is crucial 1, 4

The American College of Rheumatology recommends cyclophosphamide or rituximab combined with glucocorticoids as the standard treatment for severe ANCA-associated vasculitis, with approximately 90% of patients achieving remission 7, 8, 9
The European League Against Rheumatism suggests methotrexate plus glucocorticoids as a less toxic alternative for non-organ threatening or non-life threatening disease 7, 8
Induction therapy consists of cyclophosphamide and glucocorticoids for generalized and severe disease, with rituximab recommended as an alternative first-line agent for patients with fertility concerns or relapsing disease 7, 8, 10

The American College of Rheumatology recommends intravenous immunoglobulin (IVIG) at doses of 2 gm/kg as adjunctive therapy for short-term control in refractory disease, with prior measurement of serum immunoglobulin levels due to risk of anaphylaxis in IgA deficiency 7, 8, 11

Delaying proven immunosuppressive therapy in favor of unproven vitamin infusions risks permanent organ damage or death, with untreated systemic vasculitis carrying high mortality and a five-year mortality of 40-46% in patients with poor prognostic factors 12

The European Vasculitis Study Group recommends categorizing ANCA-associated vasculitis by severity using the EUVAS classification system, which includes localized, early systemic, generalized, severe, and refractory disease, to guide treatment intensity 13
The EUVAS classification system defines localized disease as upper/lower respiratory tract disease without systemic involvement, early systemic disease as any manifestations without organ-threatening disease, generalized disease as renal or organ-threatening disease, severe disease as renal or vital organ failure, and refractory disease as progressive disease unresponsive to standard therapy 13

The European Vasculitis Study Group recommends cyclophosphamide plus glucocorticoids as the cornerstone for severe disease, with cyclophosphamide dosed at 2 mg/kg/day oral and prednisolone dosed at 1 mg/kg/day 13
The European Vasculitis Study Group recommends IV methylprednisolone pulses for rapid effect in severe disease 13

The European Vasculitis Study Group recommends pneumocystis jiroveci prophylaxis with trimethoprim/sulfamethoxazole for all patients receiving cyclophosphamide 13
The European Vasculitis Study Group recommends mesna for bladder protection in patients receiving cyclophosphamide 13
The European Vasculitis Study Group recommends osteoporosis prophylaxis per local guidelines for all patients on long-term glucocorticoids 13

The European Vasculitis Study Group recommends adding plasma exchange for patients with serum creatinine >500 μmol/L, dialysis requirement, or diffuse alveolar hemorrhage with hypoxemia 13