From the Guidelines
Patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) should primarily see a rheumatologist who specializes in vasculitis and autoimmune conditions, as well as other specialists such as a pulmonologist, cardiologist, neurologist, nephrologist, dermatologist, and ENT specialist, depending on the organs involved, to ensure comprehensive care and management of this complex multisystem disorder. According to the most recent evidence-based guideline for the diagnosis and management of EGPA 1, patients with EGPA should be managed by interdisciplinary care, with decisions being shared by patients and physicians, and with consideration of safety, efficacy, and costs. The guideline also emphasizes the importance of improving the quality of life of patients with EGPA, together with clinical outcomes such as survival, long-term preservation of organ function, and prevention of disease flares.
The management of EGPA requires a coordinated approach, with a rheumatologist playing a central role in coordinating overall care and managing immunosuppressive treatments like corticosteroids, cyclophosphamide, azathioprine, methotrexate, or biologics such as mepolizumab. A pulmonologist is essential since EGPA commonly affects the lungs and often begins with asthma-like symptoms. Depending on organ involvement, patients may also need to see other specialists, such as:
- A cardiologist for heart complications, as major vascular events and cardiac involvement are frequent in EGPA and seem to be associated with a poorer survival 1
- A neurologist for nerve damage, as neuropathy is a severe complication of EGPA that can lead to disability due to muscle atrophy and neuropathic pain 1
- A nephrologist for kidney issues
- A dermatologist for skin manifestations
- An ENT specialist for sinus problems
Regular follow-ups with these specialists are crucial, as EGPA is a multisystem disorder that can affect various organs unpredictably. Treatment plans must be individualized based on disease severity, organ involvement, and patient-specific factors, with coordination between specialists to manage this complex condition effectively and prevent long-term damage 1. Additionally, patients with EGPA should be screened for treatment-related and cardiovascular comorbidities, and prophylaxis and lifestyle advice should be given to reduce cardiovascular risk and treatment-related complications 1.
From the Research
Specialists for Eosinophilic Granulomatosis with Polyangiitis (EGPA)
To manage EGPA effectively, it is crucial to consult specialists from various fields due to the disease's complex and multifaceted nature. The following specialists may be involved in the care of a patient with EGPA:
- Rheumatologists: They play a key role in diagnosing and treating EGPA, as it is classified as a form of vasculitis, which falls under the realm of rheumatology 2, 3, 4, 5, 6.
- Pulmonologists: Given that EGPA often presents with asthma and pulmonary involvement, pulmonologists are essential for managing respiratory symptoms and complications 2, 4, 6.
- Cardiologists: Cardiac involvement is a significant aspect of EGPA, associated with a poor prognosis. Therefore, cardiologists are crucial for monitoring and treating cardiac complications 2, 4, 6.
- Neurologists: Neurological involvement, such as mononeuritis multiplex, can occur in EGPA patients. Neurologists help in diagnosing and managing these neurological manifestations 5.
- Dermatologists: Skin involvement, including cutaneous vasculitis, may require the expertise of dermatologists for appropriate management 4, 5.
- Nephrologists: In cases where EGPA affects the kidneys, leading to glomerulonephritis, nephrologists are necessary for renal care and management 4.
- Immunologists: Given the immune system's role in EGPA, immunologists may be involved in understanding the disease's pathogenesis and in developing targeted therapies 3, 4.
- Pathologists: For confirming the diagnosis through biopsy and histopathological examination, pathologists play a vital role 5.