Anti-neutrophil cytoplasmic antibody-associated vasculitis: prevalence, treatment, and outcomes

Abstract

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a primary small-vessel vasculitis group with three distinct clinical entities, including GPA, MPA, and EGPA. The incidence of AAV has increased since the 1980s and remained stable since the early 2000s. Distinct phenotypes of AAV may also differ in various geographical regions. Elderly people are susceptible to developing AAV, and AAV is used to be a fatal disease before the introduction of glucocorticoids and immunosuppressants. Different treatment protocols should be employed for patients with different disease severity levels. Several randomized controlled trials evaluated the efficacy and safety of treatment protocols for remission induction and maintenance. Glucocorticoid and cyclophosphamide therapies remain the mainstay for treating AAV. Rituximab is non-inferior to cyclophosphamide for inducing remission, and it is more effective for relapsing and refractory disease. The combination of low-dose glucocorticoids with less toxic immunosuppressants, such as azathioprine, rituximab, and methotrexate, is suggested in place of cyclophosphamide. The prognosis of patients with AAV is another crucial issue due to the accumulating damage caused by both the disease activity and treatment toxicity. This review focused on recent progress on the prevalence, treatment, and outcomes of patients with AAV.

ANCA associated vasculitis in patients from Saudi Arabia

“…Use of high dose corticosteroids and IV CYC for induction and azathioprine or methotrexate with steroid tapering for maintenance improved the outcome of our AAV patients. 10 , 19 , 20 Four of our GPA cases required rituximab therapy to control their symptoms which has been shown to be effective in relapsing and refractory AAV. 20 , 21 Since the introduction of treatment regimen involving IV CYC for induction therapy with glucocorticoids and azathioprine or methotrexate for maintenance therapy, five year survival has exceeded 80%.…”

The Protein Tyrosine Phosphatase Non-Receptor Type 22 (PTPN22) Gene Polymorphism and Susceptibility to Autoimmune Diseases

“…Though the manifestations in the lungs and kidneys are common, any organ or system can be affected. AAV refers to a group of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic GPA (EGPA, formerly Churg-Strauss syndrome) [256].…”

Actualización en el estudio de Granulomatosis con poliangeitis (Granulomatosis de Wegener)

“…El uso de rituximab también ha demostrado mantener remisión de la enfermedad con menor porcentaje de recaídas. Se recomienda el uso sistemático de cotrimoxazol para prevenir recaídas e infecciones por Pneumocystis jirovecii (2,3,40) .…”

“…Puede presentarse a cualquier edad con un peak a los 65-74 años. Su incidencia anual es de 5-10/millón con una prevalencia de 24-157 casos por millón, siendo similar entre ambos sexos (3,4) . En la etiopatogenia estarían involucrados gatillantes infecciosos, ambientales o farmacológicos que, en personas genéticamente predispuestas, generan una respuesta inflamatoria y producción de ANCA contra proteinasa-3 (PR3) y mieloperoxidasa (MPO) en el 80% y 10% de los pacientes respectivamente, desarrollando inflamación granulomatosa necrotizante (4) .…”