From the Guidelines
P-ANCA diseases should be treated with immunosuppression, prioritizing corticosteroids and cyclophosphamide or rituximab for induction therapy, to reduce morbidity and mortality. The main P-ANCA diseases include microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), and some cases of glomerulonephritis, which typically affect small blood vessels in multiple organs, particularly the kidneys and lungs 1.
Key Considerations
- Treatment usually involves immunosuppression with corticosteroids like prednisone (starting at 1 mg/kg/day) combined with cyclophosphamide (2 mg/kg/day) or rituximab (375 mg/m² weekly for 4 weeks) for induction therapy.
- Maintenance therapy often includes azathioprine (2 mg/kg/day), methotrexate (15-25 mg weekly), or mycophenolate mofetil (1-2 g/day) for 18-24 months.
- Plasma exchange may be used in severe cases with pulmonary hemorrhage or rapidly progressive kidney disease, as supported by recent guidelines 1.
- Regular monitoring of kidney function, blood counts, and inflammatory markers is essential during treatment, to minimize the risk of adverse effects and optimize treatment outcomes.
Disease Characteristics
- Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare small-vessel vasculitis, characterized by asthma, eosinophilia, and granulomatous or vasculitic involvement of several organs 1.
- The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach.
- The clinical phenotype of EGPA is quite heterogeneous, and the diagnosis is not always straightforward, with anti-neutrophil cytoplasmic antibodies (ANCA) detectable in ~40% of cases 1.
Treatment Outcomes
- Early diagnosis and aggressive treatment are crucial, as these conditions can be life-threatening if left untreated, with five-year survival rates improving from 20% to over 80% with proper therapy.
- Recent studies have investigated the efficacy and toxicity of both biologic and nonbiologic immunosuppressive agents for the treatment of AAV, providing evidence-based recommendations for treatment and management 1.
From the Research
P-ANCA Diseases
P-ANCA (perinuclear anti-neutrophil cytoplasmic antibodies) are associated with several diseases, including:
- Ulcerative colitis (UC) 2, 3, 4, 5, 6
- Primary sclerosing cholangitis (PSC) 2, 3, 4, 5, 6
- Type 1 autoimmune hepatitis (AIH) 4
Prevalence of P-ANCA
The prevalence of P-ANCA in patients with UC and PSC is high, with studies reporting:
- 50-80% of UC patients testing positive for P-ANCA 4
- 70% of PSC patients testing positive for P-ANCA 4
- 92% of patients with type 1 AIH testing positive for P-ANCA 4
Clinical Significance of P-ANCA
P-ANCA have been associated with:
- Disease activity in UC patients 3
- Extensive disease in UC patients 5
- Backwash ileitis in UC patients 5
- Primary sclerosing cholangitis in UC patients 2, 3, 5, 6
- Autoimmune hepatitis in PSC patients 4
Diagnostic Markers
P-ANCA may be used as a diagnostic marker for:
- UC 3, 5, 6
- PSC 2, 3, 5, 6
- AIH 4 However, the sensitivity and specificity of P-ANCA as a diagnostic marker may vary depending on the disease and patient population 6.
Subtypes of P-ANCA
Different subtypes of P-ANCA may be associated with different diseases, including: