History
The 3 phases—allergic, eosinophilic, and vasculitic—of eosinophilic granulomatosis with polyangiitis (EGPA) do not necessarily follow one another in any particular order and frequently overlap. Symptoms depend on the phase and organ systems involved. A careful history should include medication usage, infectious symptoms, and/or preexisting disease. [15, 11]
The allergic phase (also called prodromal) may last months to years and is characterized by the following:
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Rhinitis, sinus pain, headache
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Cough
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Wheezing or asthma is one of the main manifestations, with an estimated 96-100% of patients affected
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Arthralgias, myalgias
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Malaise
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Fever
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Weight loss
The eosinophilic phase (or second phase) is characterized by the following:
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Peripheral eosinophilia (usually greater than 1,500/µL) and possible organ involvement
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Gastrointestinal - Abdominal pain, diarrhea, bleeding, nausea, vomiting, bleeding
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Pulmonary - Cough
The vasculitic phase (or third phase) is characterized by the following:
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General - Malaise, fever
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Cardiac - Chest pain, dyspnea
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Cutaneous - Purpura, papules
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Pulmonary - Cough, hemoptysis
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Rheumatologic - Arthralgia, arthritis, myalgia
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Neurologic - Weakness, numbness
Physical Examination
Clinical findings in eosinophilic granulomatosis with polyangiitis (EGPA) vary depending on the phase and organ systems involved. [2, 11, 16]
Allergic (prodromal) phase findings are as follows:
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Upper respiratory tract symptoms affect majority of patients
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Nasal polyps
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Wheezing
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Cough
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Rhinitis
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Recurrent or chronic sinusitis
Eosinophilic (second) phase findings are as follows:
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General - Weight loss, fever, sweats
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Pulmonary - Cough, hemoptysis, rales, rhonchi
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Gastrointestinal - Rebound, masses, obstruction, ascites, bleeding
Vasculitic (third) phase findings are as follows:
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Most commonly affects skin and peripheral nervous system [17]
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Constitutional symptoms - Fever, weight loss, adenopathy
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Cardiac - Gallop, pericardial friction rub, jugular venous distension, peripheral edema
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Pulmonary - Rales, rhonchi
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Renal - Mild proteinuria and hematuria (more common in those with serologically detectable ANCA)
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Genitourinary - Obstructive uropathy
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Ocular - Episcleritis, panuveitis, marginal corneal ulceration, conjunctival infiltration, retinal infarction
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Musculoskeletal - Joint swelling, muscle tenderness
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Cutaneous - Palpable purpura (50%), subcutaneous nodules (scalp and extensor surfaces on extremities), firm papules on fingertips (may resemble septic emboli), urticaria, livedo reticularis
See the images below.
Complications
Although many organ systems can be affected by eosinophilic granulomatosis with polyangiitis (EGPA), patients are most likely to have pulmonary or cardiac disease.
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Granuloma with a central core of eosinophilic debris surrounded by a peripheral palisade of epithelioid histiocytes and eosinophils.
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Bilateral papules and nodules with central necrosis.
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Magnified view of papules and nodules with central necrosis.
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Distal digital purpuric papule.
