History and exam
Key diagnostic factors
common
history of asthma, allergic rhinitis, or sinusitis
The first phase of EGPA consists of this triad.
Increases the risk of EGPA in patients with asthma by up to 12 times compared with the general population.[11]
Consistent with a disease with an allergic component, increased levels of serum IgE and immune complexes containing IgE have been noted, particularly during flares of EGPA.[22]
focal numbness or weakness
nasal discharge or stuffiness, or facial pain
Allergic rhinitis is a common feature in EGPA. Paranasal sinus involvement is frequent in EGPA, typically predates vasculitic disease, and may even predate asthma. Most common manifestations beyond allergic rhinitis include nasal polyps, nasal obstruction, and recurrent episodes of sinusitis. These may include purulent, bloody nasal discharge with facial pain.
palpable purpura and petechiae
Classic sign of small-vessel vasculitis; most commonly found on the lower extremities.
wheeze
Associated with asthma, which is present in 99% of patients with EGPA. Although generally indistinguishable from other forms of asthma, patients typically have asthma at least of moderate severity that typically predates the onset of vasculitis by several years.
uncommon
hemoptysis
May indicate alveolar hemorrhage, which is typically diagnosed at bronchoscopy by the presence of sequential blood aliquots of bronchoalveolar lavage fluid, with or without elevated hemosiderin-laden macrophages in the fluid. Alveolar hemorrhage is an uncommon manifestation of EGPA but warrants aggressive therapy with high-dose corticosteroids and cyclophosphamide.
Other diagnostic factors
common
use of certain medications
Various medications have been linked to EGPA. These include macrolide antibiotics and quinidine.
Whether leukotriene receptor antagonists confer any risk for EGPA is controversial. Evidence points toward these agents unmasking previously unrecognized underlying EGPA, either by facilitating systemic glucocorticoid withdrawal, thereby allowing the full syndrome to develop, or by preventing control of the disease as the patient's symptoms progress.[18][19][20]
fatigue, arthralgias, myalgias
Patients often have low-grade subacute or chronic constitutional symptoms.
shortness of breath or cough
May be due to cardiac or pulmonary involvement.
abdominal pain
About one third of patients have gastrointestinal manifestations. These may be secondary to esophagitis, gastritis, colitis, cholecystitis, or biliary tract vasculitis.
sensory or motor deficits
Isolated motor or sensory deficits may be noted in the case of mononeuritis multiplex.
tachypnea
Finding associated with pulmonary symptoms from asthma, thromboembolic disease, alveolar hemorrhage, heart failure, or other forms of pulmonary involvement.
uncommon
rales
Crackles are commonly associated with pulmonary edema. May be seen with alveolar hemorrhage as well.
peripheral edema
May be seen in heart failure as well as deep vein thrombosis, particularly if the edema is unilateral.
orthopnea
Associated with left-sided heart failure due to cardiomyopathy.
hepatojugular reflux
Commonly associated with right-sided heart failure.
Risk factors
strong
history of asthma, allergic rhinitis, or sinusitis
The first phase of EGPA consists of this combination.
Increases the risk of EGPA in patients with asthma by up to 12 times compared with that of the general population.[11]
Consistent with a disease with an allergic component, increased levels of serum immunoglobulin (Ig) E and immune complexes containing IgE have been noted, particularly during flares of EGPA.[22]
weak
use of certain medications
Various medications have been linked to EGPA. These include macrolide antibiotics and quinidine.
Whether leukotriene receptor antagonists confer any risk for EGPA is controversial. Evidence points toward these agents unmasking previously unrecognized underlying EGPA, either by facilitating systemic glucocorticoid withdrawal, thereby allowing the full syndrome to develop, or by preventing control of the disease as the patient's symptoms progress.[18][19][20]
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