Could a 48-year-old female with severe bronchial asthma (Bronchial Asthma) and frequent exacerbations, bronchiectasis on High-Resolution Computed Tomography (HRCT), and significant left ventricular dysfunction (Left Ventricular Ejection Fraction (LVEF) of 25%) without coronary artery disease be diagnosed with Eosinophilic Granulomatosis with Polyangiitis (EGPA)?

EGPA Should Be Strongly Considered and Urgently Investigated in This Patient

This clinical presentation is highly suspicious for EGPA and warrants immediate comprehensive evaluation including peripheral eosinophil count, ANCA testing, cardiac MRI, and nerve conduction studies. The combination of severe asthma with frequent exacerbations, bronchiectasis, and particularly the severe cardiomyopathy (LVEF 25%) with normal coronaries in a middle-aged woman creates a compelling constellation for EGPA 1.

Why EGPA Is the Leading Diagnosis

Cardinal Features Present

• Severe asthma with frequent exacerbations - Over 90% of EGPA patients have asthma, typically adult-onset, worsening over time, and rarely showing seasonal patterns 1
• Bronchiectasis on HRCT - Bronchial wall thickening and bronchiectasis occur in 66% of EGPA patients on chest CT 1
• Cardiomyopathy with preserved coronaries - This is the critical red flag. Cardiac involvement is the most important predictor of mortality in EGPA and occurs more frequently in ANCA-negative patients 1, 2. Myocarditis and cardiomyopathy are characteristic EGPA manifestations, and a significant proportion of patients remain asymptomatic despite severe cardiac dysfunction 2, 3

The Cardiac Presentation Is Particularly Telling

EGPA-associated cardiomyopathy with normal coronary arteries is a hallmark presentation that distinguishes this from typical ischemic heart disease 3. The constellation of peripheral eosinophilia, left ventricular dysfunction, and normal epicardial coronary arteries should immediately raise suspicion for eosinophilic myocarditis 3. Cardiac ECT (emission computed tomography) has higher sensitivity than routine tests like ECG, echocardiography, or troponin levels for detecting cardiac involvement 4.

Immediate Diagnostic Workup Required

Essential Laboratory Tests

• Peripheral blood eosinophil count and percentage - Active EGPA typically shows marked eosinophilia (>1,500 cells/μl or >10%), though this can be masked by systemic glucocorticoids 1
• ANCA testing with MPO-ANCA - Should be performed in all suspected cases. ANCA positivity occurs in 30-40% of EGPA patients, with MPO-ANCA being most common 1. However, ANCA-negative patients more frequently manifest cardiomyopathy and lung involvement, making a negative ANCA result consistent with this presentation 1
• Total IgE level - Usually elevated in EGPA if not on systemic steroids 1

Critical Cardiac Evaluation

Cardiac MRI is mandatory - It can reveal late gadolinium enhancement and assess ventricular function, and characteristic vasculitis findings on CMR strongly suggest EGPA-associated eosinophilic myocarditis 2, 3. The sensitivity of cardiac ECT is higher than routine tests, so timely screening is recommended for all suspected EGPA patients 4.

Additional Investigations to Complete the Picture

• Nerve conduction studies - Peripheral neuropathy (mononeuritis multiplex) occurs in 50-70% of EGPA patients and may be subclinical 1, 3. Even without clinical neurological symptoms, nerve conduction studies can confirm involvement 3
• Urinalysis and renal function - To assess for glomerulonephritis, proteinuria, or hematuria 1
• ENT examination - Chronic rhinosinusitis with nasal polyps occurs in 54-70% of cases 1

Critical Diagnostic Considerations

No Validated Diagnostic Criteria Exist

There are no diagnostic criteria for EGPA - classification criteria (ACR 1990, ACR-EULAR 2022) should not be used for diagnosis 1. Diagnosis must be based on highly suggestive clinical features, objective evidence of vasculitis (ideally from histology), and ANCA results 1.

The Lanham Criteria Framework

While not validated, the Lanham criteria (asthma, eosinophilia, vasculitis involving two or more organs) provide a useful framework 1. This patient potentially meets all three if eosinophilia and multi-organ vasculitis are confirmed.

ANCA Status Affects Phenotype

ANCA-positive patients more frequently show peripheral neuropathy, glomerulonephritis, and purpura, while ANCA-negative patients more commonly have myocardial, respiratory, and gastrointestinal involvement 1. This patient's cardiac-predominant presentation suggests she may be ANCA-negative.

Urgent Management Implications

This Is a Medical Emergency

Cardiac involvement in EGPA requires immediate evaluation and treatment 5. The LVEF of 25% represents severe organ-threatening disease that falls under the Five-Factor Score criteria for poor prognosis 1.

Treatment Should Not Be Delayed

If EGPA is confirmed with severe manifestations (cardiomyopathy qualifies), high-dose glucocorticoids plus cyclophosphamide or rituximab should be initiated immediately 1, 5. For organ-threatening manifestations including cardiomyopathy, glucocorticoids should be combined with cyclophosphamide or rituximab as remission-induction therapy 1.

Common Pitfalls to Avoid

• Do not dismiss the diagnosis based on ANCA negativity - 60-70% of EGPA patients are ANCA-negative, and these patients are more likely to have cardiac involvement 1
• Do not rely on troponin or routine ECG alone - Silent myocarditis is well-documented in EGPA, and patients can have severe cardiac dysfunction without symptoms or elevated troponin 2, 4
• Do not wait for histological confirmation to treat - If clinical suspicion is high and cardiac involvement is present, treatment should begin promptly given the mortality risk 2, 3
• Exclude helminthic infections before starting steroids - Particularly Strongyloides, which can cause fatal hyperinfection syndrome 5

The Diagnostic Timeline Matters

The mean delay from asthma diagnosis to EGPA diagnosis averages 11.8 years, but can be as short as 8 months 1, 6. EGPA can cause severe complications like cardiomyopathy as early manifestations of systemic vasculitis, even when the Five-Factor Score suggests good prognosis 6. This patient's presentation demands urgent rather than delayed evaluation.