2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis (Part 1)
This week we will be starting a new topic. The new topic has something to do with the management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis (ANCA-AAV).
ANCA-AAV is a group of diseases that includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).
These diseases normally impact small and medium-sized vessels and may involve several organs in the body.
GPA is characterized by necrotizing granulomatous inflammation in on top of vasculitis.
Common signs of GPA include destructive sinonasal lesions, pulmonary nodules, and pauci-immune glomerulonephritis.
GPA is often linked with cytoplasmic ANCA and antibodies to proteinase 3 (PR3).
Most often the prevalence of this disease ranges from 24 to 157 cases per million.
MPA is characterized by vasculitis without granulomatous inflammation. Common clinical signs of MPA may include rapidly progressive pauci-immune glomerulonephritis and alveolar hemorrhage. MPA is linked with perinuclear ANCA and antibodies to myeloperoxidase. The prevalence of MPA is 0 to 66 cases per million among Europeans and 86 cases per million among Japanese people.
EGPA is characterized by eosinophilic tissue infiltration in addition to vasculitis.
The usual manifestations include asthma, peripheral eosinophilia, and peripheral neuropathy.
Records show 40% of patients may end with detectable ANCA.
The prevalence of EGPA among Europeans is estimated to range from 2 to 38 cases per million.
Before the prevalent use of alkylating agents, the survival of patients of the disease is quite poor. Modern treatment modalities have changed the poor prognosis or disease, yet treatments are known to be associated with toxicity.
The clinical trials, recently done, have probed the efficacy and toxicity of both biologic and nonbiologic immunosuppressive agents for the treatment of AAV.
There are studies showing additional insight regarding management strategies to help patients of these diseases.
As such, experts formulated a guideline to provide evidence-based recommendations for the treatment and management of GPA, MPA, and EGPA.
The guideline may be read by international audiences, but let it be known that the recommendations were formulated considering the experience, treatment and diagnostic modalities in the United States.
Next week we will be making a deep dive on the methods that were used by the American College of Rheumatology (ACR) in formulating the guidelines for the management of (ANCA-AAV).