16th January 2024

Presentation

It has been proposed that EGPA presentations can be split into three different phases, depending on clinical symptoms and signs at the time of diagnosis. The three phases are summarised below:

• Phase 1: Prodrome – tends to begin between the ages of 20 and 30 and precedes the final phase by about a decade. The features of this phase are asthma symptoms, e.g., wheeze, with sinusitis and allergic rhinitis. 
• Phase 2: Eosinophilia – involves eosinophilic infiltration of the organs, especially the lungs.
• Phase 3: Organ-specific vasculitis – small to medium vessel vasculitis develops. Other symptoms like fatigue and weight loss can occur concurrently with this final phase, and asthma exacerbations often increase with greater severity. 

Patients typically present with a history of asthma and associated symptoms e.g., cough and wheeze. They may also have a number of other associated signs and symptoms depending on their stage of disease, including: 

• Purulent/bloody nasal discharge (due to sinusitis/allergic rhinitis)
• Haemoptysis
• Symptoms of PE or DVT – EGPA increases the risk of VTE.
• Orthopnoea, crackles and peripheral oedema may be present if heart failure secondary to vasculitis-induced cardiomyopathy has developed – cardiac involvement in EGPA has high mortality and poor prognosis.
• Abdominal pain and change in bowel habit 
• Renal insufficiency and glomerulonephritis – occur in about a quarter of patients with EGPA, also indicates poor prognosis.
• Peripheral neuropathy and weakness 
• New-onset motor deficits, e.g., foot drop 
• Arthralgia and myalgia 
• Fatigue
• Purpuric skin rash and nodules on extensor surfaces 
• Lymphadenopathy