Eosinophilic Granulomatosis with Polyangiitis (EGPA): Symptoms, Causes, Treatment
الورام الحبيبي اليوزيني مع التهاب الأوعية
Eosinophilic granulomatosis with polyangiitis is a rare disorder often associated with asthma and elevated eosinophils. Nerves, lungs, heart and other organs may be affected; asthma alone is not EGPA.
What is Eosinophilic Granulomatosis with Polyangiitis (EGPA)?
Previously called Churg-Strauss syndrome, its stages and severity vary. Important organ involvement can occur without every classic feature.
Related reading: Asthma and difficult symptom control.
Symptoms
Difficult asthma, sinus disease, rash, numbness or limb weakness may occur. New chest pain or breathlessness may signal organ involvement needing urgent assessment.
Causes
Eosinophilic inflammation and immune-mediated vessel injury overlap. The precise cause is unknown, and other explanations for eosinophilia must be excluded.
Risk factors
Asthma and eosinophilia accompanied by symptoms outside the airways increase suspicion. Most people with asthma do not have this rare disorder.
Diagnosis
Evaluation includes blood counts, organ function, urine testing, imaging and sometimes biopsy. ANCA antibodies may be negative; their absence does not exclude EGPA.
Treatment
Treatment depends on organ severity and may include steroids, selected immunosuppressants or biologics. Asthma-focused therapy alone may not adequately treat severe cardiac or nerve involvement.
Complications
Nerve, heart, kidney or intestinal injury can occur. Relapses are possible, so controlled asthma does not remove the need for wider organ surveillance.
Prevention
There is no established prevention. Follow asthma care, monitoring and appropriate vaccination plans; never abruptly stop long-term steroids.
When to see a doctor
Chest pain, fainting, severe breathlessness or new neurological weakness requires urgent care. Bloody urine or reduced urine output also needs prompt review.