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Granulomatosis with polyangiitis

What's new

Updated 2024 KDIGO guidelines for the diagnosis and management of granulomatosis with polyangiitis.

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of granulomatosis with polyangiitis are prepared by our editorial team based on guidelines from the Kidney Disease: Improving Global Outcomes Foundation (KDIGO 2024), the Canadian Vasculitis Research Network (CanVasc 2023,2021), the European League Against Rheumatism (EULAR 2023), the Vasculitis Foundation (VF/ACR 2021), and the Single Hub and Access Point for Pediatric ...
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Classification and risk stratification

Severity assessment: as per CanVasc 2021 guidelines, stratify patients with newly diagnosed or relapsing ANCA-associated vasculitides according to the extent and severity of the disease to tailor treatment accordingly.
B
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Diagnostic investigations

ANCA: as per EULAR 2023 guidelines, obtain proteinase 3-ANCA and myeloperoxidase-ANCA testing with a high-quality antigen-specific assay as the primary method in patients with signs and/or symptoms raising suspicion of an ANCA-associated vasculitis.
A

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  • Serum immunoglobulins

Diagnostic procedures

Biopsy: as per EULAR 2023 guidelines, perform a biopsy to establish a new diagnosis of ANCA-associated vasculitis and for further evaluation of suspected relapsing vasculitis.
B

Medical management

General principles
As per KDIGO 2024 guidelines:
Manage patients with ANCA-associated vasculitis at centers with experience in ANCA-associated vasculitis management.
E
Do not delay initiating immunosuppressive therapy while waiting for a kidney biopsy to be performed or reported in patients with a clinical presentation compatible with small-vessel vasculitis in combination with positive myeloperoxidase- or proteinase 3-ANCA serology, especially in rapidly deteriorating patients.
D

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  • Induction of remission (non-severe)

  • Induction of remission (severe)

  • Induction of remission (avacopan)

  • Induction of remission (IVIG and plasma exchange)

  • Maintenance of remission

  • Anticoagulant therapy

Therapeutic procedures

Plasma exchange
As per KDIGO 2024 guidelines:
Consider performing plasma exchange in patients with serum creatinine > 3.4 mg/dL (> 300 mcmol/L), rapidly increasing serum creatinine, or requiring dialysis, and in patients with diffuse alveolar hemorrhage having hypoxemia.
E
Perform plasma exchange in patients with an overlap syndrome of ANCA-associated vasculitis and anti-GBM.
E

Surgical interventions

Kidney transplantation: as per KDIGO 2024 guidelines, delay transplantation until patients are in complete clinical remission for ≥ 6 months. Do not delay transplantation because of the persistence of ANCA.
E

Specific circumstances

Pediatric patients, diagnosis and classification
As per SHARE initiative 2019 guidelines:
Recognize that there are no validated diagnostic criteria for GPA.
B
Recognize that there are two different classification criteria for GPA:
ACR criteria
EULAR/PRINTO/PReS endorsed Ankara 2008 criteria
B
Use the Ankara 2008 criteria for the classification of GPA in pediatric patients.
B

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  • Pediatric patients (ANCA testing)

  • Pediatric patients (diagnostic imaging)

  • Pediatric patients (biopsy)

  • Pediatric patients (treatment)

  • Pregnant patients

  • Patients with sinonasal involvement

  • Patients with respiratory tract involvement (systemic therapy)

  • Patients with respiratory tract involvement (topical therapy)

  • Patients with respiratory tract involvement (plasma exchange)

  • Patients with mass lesions

Patient education

General counseling: as per EULAR 2023 guidelines, provide patients with ANCA-associated vasculitis education focusing on the impact of ANCA-associated vasculitis and its prognosis, key warning symptoms, and treatment (including treatment-related complications).
B

Preventative measures

Prophylaxis for P. jirovecii pneumonia: as per EULAR 2023 guidelines, administer TMP/SMX for prophylaxis against P. jirovecii pneumonia and other infections in patients with ANCA-associated vasculitis receiving rituximab, cyclophosphamide, and/or high doses of corticosteroids.
B

Follow-up and surveillance

Serial clinical assessment
As per EULAR 2023 guidelines:
Obtain periodic assessment for treatment-related adverse effects and comorbidities in patients with ANCA-associated vasculitis. Offer prophylaxis and lifestyle advice to reduce treatment-related complications and other comorbidities.
B
Obtain structured clinical assessment, rather than ANCA and/or CD19-positive B cell testing alone, to inform decisions on changes in treatment in patients with ANCA-associated vasculitis.
B

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  • Serial laboratory assessment

  • Management of refractory disease

  • Management of relapse (immunosuppressants)

  • Management of relapse (avacopan)