clinical findings
• The most common clinical finding is lung involvement (asthma, migratory pulmonary infiltrates).
• Renal involvement (glomerulonephritis) may be seen like other ANCA positive vasculitides.
• However, compared to other ANCA positive vasculitides;
o Mononeuritis multiplex is more common (2nd most common finding in EGPA).
o Cardiac involvement is expected more (most common cause of death in EGPA)
In the presence of constitutional symptoms (fever, weight loss, etc.) and signs of systemic organ involvement (hematuria, proteinuria, neuropathy, cardiac involvement, etc.) in a patient with a diagnosis of asthma and eosinophilia, eosinophilic granulomatous polyangiitis (EGPA) should be considered first.
Laboratory Findings
• Eosinophilia and IgE elevation are found in most of the patients.
• PANCA (anti-myeloperoxidase) positivity is expected.
Treatment
• Steroid alone is sufficient in most patients. In severe cases (cardiac etc.), cyclophosphamide should be added.
• Mepolizumab (anti-ILS monoclonal antibody) is approved for treatment.
ANCA positive diseases
PANCA
• Eosinophilic granulomatous polyangiitis
• Microscopic polyangiitis
• Ulcerative colitis
• Primary sclerosing cholangitis
CANCA
• Granulomatous polyangiitis