Fast Five Quiz: Management of GPA

Medscape

03-07-2025

Granulomatosis with polyangiitis (GPA) is a variant of anti-neutrophil cytoplasmic antibody ( ANCA)-associated vasculitis that most commonly presents with proteinase 3 (PR3) serology and distinct clinical features that necessitates swift identification and therapeutic intervention. Prompt treatment plays a crucial role in minimizing permanent organ damage, particularly in the renal and pulmonary systems.
What do you know about the management of GPA? Check your knowledge with this quick quiz.
The latest guidelines from the Kidney Disease Improving Global Outcomes (KDIGO) and European Alliance of Associations for Rheumatology (EULAR) both recommend initiating immunosuppressive therapy in patients with suspected PR3 positive ANCA vasculitis, which is indicative of GPA, even if clinicians are still awaiting biopsy results. Further, EULAR specifically acknowledges that collecting biopsies might not be feasible for each patient with ANCA vasculitis and treatment should 'not be delayed while awaiting histological information.'
Learn more about biopsy for GPA.
KDIGO recommends maintaining therapeutic intervention for 18 months to 4 years following successful remission, which is similar to the EULAR guidelines recommending maintenance therapy for 24-48 months in patients with GPA. They also state, 'longer duration of therapy should be considered in relapsing patients or those with an increased risk of relapse, but should be balanced against patient preferences and risks of continuing immunosuppression.'
Learn more about remission maintenance in GPA.
Infection is a significant concern in ANCA vasculitis, especially regarding immunosuppressive therapies including glucocorticoids that are used to manage the clinical manifestations of the disease. Recent research has shown that chronic nasal carriage of S aureus is significantly higher in patients with the GPA subtype; it has also been shown to be associated with increased endonasal activity and risk for relapse.
Although M catarrhalis, H influenzae, and S pneumoniae do not appear to have a specific association with GPA, data have shown that infection is among the most common causes of death in GPA.
Learn more about infection in GPA.
Data indicate that the GPA subtype is an independent risk factor for disease recurrence. Additionally, KDIGO also considers lower serum creatinine, more extensive disease, PR3 histology (which is associated with higher relapse rates than MPO-positive disease commonly seen in MPA) and ear, nose, and throat involvement as 'baseline factors' for disease recurrence. Factors after diagnosis include history of relapse, ANCA positivity at the end of induction, and rise in ANCA. Treatment-related relapse factors include lower cyclophosphamide exposure, immunosuppressive withdrawal, and glucocorticoid withdrawal.
Learn more about prognosis in GPA.
To ensure disease does not recur into the transplanted tissue, KDIGO specifically recommends delaying kidney transplantation until patients have achieved complete clinical remission for at least 6 months. Further, ANCA positivity or negativity should not factor into this decision, with KDIGO stating, 'the persistence of ANCA should not delay transplantation.' Although a serum creatinine of > 4 mg/dL is a significant predictor of relapse, guidelines recommend considering combination of a monoclonal antibody and an alkylating agent for this indication. Similarly, induction and maintenance of dialysis should also be done under various separate conditions.
Learn more about kidney transplantation for GPA.
Editor's Note: This article was created using several editorial tools, including generative AI models, as part of the process. Human review and editing of this content were performed prior to publication.