From the Guidelines
The approach to diagnosing and managing vasculitis should involve prompt recognition, thorough evaluation, and targeted treatment based on the specific type and severity, with management in collaboration with, or at centres of expertise, as recommended by the most recent guidelines 1.
Key Principles of Management
- Initial assessment should include a comprehensive history, physical examination, laboratory tests (complete blood count, inflammatory markers like ESR and CRP, renal function, urinalysis, and autoantibodies such as ANCA), and appropriate imaging studies.
- Tissue biopsy of affected organs is often necessary for definitive diagnosis, with a positive biopsy being strongly supportive of vasculitis 1.
- Treatment typically follows a two-phase approach: induction of remission followed by maintenance therapy.
Induction of Remission
- For severe systemic vasculitis, induction commonly involves high-dose corticosteroids (prednisone 1 mg/kg/day, maximum 60-80 mg daily) combined with immunosuppressants like cyclophosphamide (15 mg/kg IV every 2-3 weeks) or rituximab (375 mg/m² weekly for 4 weeks) 1.
- The choice of immunosuppressant may depend on the specific type of vasculitis and the severity of organ involvement.
Maintenance Therapy
- Once remission is achieved (usually within 3-6 months), maintenance therapy with less toxic agents such as azathioprine (2 mg/kg/day), methotrexate (15-25 mg weekly), or mycophenolate mofetil (2-3 g daily) is initiated while gradually tapering steroids 1.
- Treatment duration typically ranges from 18-24 months, with monitoring for disease activity and medication toxicity.
Supportive Care
- Supportive care includes prophylaxis against infections and osteoporosis while on steroids.
- Patients should be monitored regularly for signs of disease activity, medication toxicity, and potential complications such as bladder cancer in those treated with cyclophosphamide 1.
Recent Guidelines
- The most recent guidelines from 2024 recommend a multidisciplinary approach to management, with consideration of referral to centres participating in clinical trials for patients with refractory disease 1.
- These guidelines emphasize the importance of accurate diagnosis, assessment of disease severity, and differentiation between active vasculitis, infection, and other complications or comorbidities.
From the FDA Drug Label
A total of 197 patients with active, severe GPA and MPA (two forms of ANCA Associated Vasculitides) were treated in a randomized, double-blind, active-controlled, multicenter, non-inferiority study, conducted in two phases – a 6 month remission induction phase and a 12 month remission maintenance phase. The main outcome measure for both GPA and MPA patients was achievement of complete remission at 6 months defined as a BVAS/GPA of 0, and off glucocorticoid therapy Patients received 1,000 mg of pulse intravenous methylprednisolone per day for 1 to 3 days within 14 days prior to initial infusion. Patients were randomized in a 1:1 ratio to receive either RITUXAN 375 mg/m2 once weekly for 4 weeks or oral cyclophosphamide 2 mg/kg daily for 3 to 6 months in the remission induction phase Following intravenous corticosteroid administration, all patients received oral prednisone (1 mg/kg/day, not exceeding 80 mg/day) with pre-specified tapering
The approach to diagnosing and managing vasculitis (inflammation of blood vessels) involves:
- Induction treatment: using medications such as rituximab or cyclophosphamide to achieve complete remission
- Remission maintenance: using medications such as azathioprine to maintain remission
- Glucocorticoid therapy: using medications such as prednisone to reduce inflammation
- Disease activity assessment: using measures such as the Birmingham Vasculitis Activity Score for Granulomatosis with Polyangiitis (BVAS/GPA) to assess disease activity
- Treatment tapering: gradually reducing the dose of medications to minimize side effects while maintaining remission 2 2
From the Research
Diagnostic Approach to Vasculitis
- The diagnosis of vasculitis involves a detailed history, careful physical examination, and appropriate laboratory tests to determine the type of onset, course of illness, organ systems affected, and extent of involvement 3.
- Biopsy examination of involved tissues is nearly always essential for diagnosis, except in certain cases such as Takayasu arteritis, when aortography is usually indicated 3.
- A rational approach is required during evaluation of patients with suspected vasculitis, considering the wide range of clinical manifestations, differential diagnosis, and potential for primary or secondary vasculitis 4.
Classification and Diagnosis
- Vasculitis can be classified based on the size of vessel involvement, which often correlates with specific clinical findings 5.
- The diagnostic work-up should be tailored to the clinical situation and geared toward a tissue or angiographic diagnosis, bearing in mind that the findings from these studies are not always pathognomonic 6.
- Determining the level of care the patient needs, ruling out secondary causes, and determining the size of vessel involvement and organ systems involved can help narrow down the differential diagnosis 7.
Treatment and Management
- Initial therapy for most types of systemic vasculitis consists of high-dose corticosteroids, with the addition of immunosuppressive therapy in certain patients 6.
- In most cases of vasculitis, corticosteroid therapy is necessary, and when the organ involvement is broader and more progressive, a combination of cytotoxic drug and corticosteroids is often needed 3.
- A comprehensive approach to diagnosing and treating the patient with suspected cutaneous vasculitis includes classification, diagnosis, laboratory workup, treatment, and careful follow-up 5.