The systemic vasculitis is a wide-ranging group of diseases that are characterized by the
presence of blood vessel inflammation (1). Despite this common feature, each type of
vasculitis has a unique variety of clinical manifestations that influences its degree of
disease severity and ultimately its management. Immunosuppressive therapy forms the
foundation of treatment for almost all forms of systemic vasculitis.
The systemic necrotizing vasculitis (SNV) are a subset of vasculitis with significant
morbidity and mortality (2). The SNV are Wegener's granulomatosis, allergic angiitis and
granulomatosis (AAG) (also known as Churg-Strauss syndrome), polyarteritis nodosum (PAN),
microscopic polyangiitis (MPA), and overlap syndrome. In spite of modern therapeutic immune
suppressive agents, there remains a not inconsequential morbidity and mortality associated
with SNV. The current standard therapy for SNV is chronic oral cyclophosphamide (1-3
mg/kg/day) and corticosteroids (3-6). Transplant doses of cyclophosphamide at 200 mg/kg
infused over 4 days is the most common worldwide transplant regimen for systemic lupus
erythematosus (SLE) (7). Like SLE, SNV are cyclophosphamide responsive disease. We,
therefore, propose a trial of high dose cyclophosphamide with anti-thymocyte globulin (ATG)
for patients with SNV.