What is the clinical presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)?
The clinical picture of EPGA typically consists of three partially overlapping phases, which may or may not be sequential:
- Prodromal/allergic phase:
a. Main manifestation is asthma, present in 96% to 100% of patients.
b. Upper respiratory symptoms are common.
c. May precede development of systemic vasculitis by several yrs.
- Eosinophilic/tissue infiltration phase:
a. Characterized by peripheral eosinophilia and eosinophilic tissue infiltration leading to organ-specific signs and symptoms, most commonly with pulmonary, cardiac, and/or GI involvement.
- Systemic vasculitic phase:
a. Onset of necrotizing vasculitis leading to constitutional and organ-specific signs and symptoms, typically with cutaneous, renal, and nervous system manifestations.
b.Distinct phenotypes related to ANCA reactivity influence frequency of organ-specific involvement
|Organ-Specific Manifestations and Phenotypes of EGPA|
|Organ Involvement||Disease Manifestations||Phase||ANCA+ |
|ANCA – Phenotype||Pathology|
|Ear, nose and throat||• Allergic rhinitis |
• Nasal polyposis
• Eosinophilic infiltrates
• Vasculitis rare
• Pulmonary infiltrates (transient) or nodules
• Pleural effusions
• Alveolar hemorrhage (rare)
|Prodromal, Eosinophilic, Vasculitis||x**||x#||• Eosinophilic infiltration|
• Necrotizing vasculitis
|Cardiac||• Congestive heart failure|
• Pericarditis/ Pericardial effusion
• Myocardial infarction
• Valvular defects
|Eosinophilic, Vasculitis||x||• Endomyocardial and pericardial eosinophilic infiltration|
• Coronary vasculitis (rare)
|Gastrointestinal||• Abdominal pain|
• Mesenteric vasculitis
|Eosinophilic, Vasculitis||• Eosinophilic infiltration; vasculitis less common|
|Renal||• Microscopic hematuria|
• Elevated creatinine
• Renal failure uncommon
|Vasculitis||x||• Pauci-immune focal segmental necrotizing glomerulonephritis|
• Crescents may or may not be present
• Eosinophils and granulomas uncommon
|Nervous System||Peripheral Nervous System:|
• Mononeuritis multiplex
Central Nervous System (rare):
• Cerebral hemorrhage or infarct
|Vasculitis||x||• Necrotizing vasculitis|
• Eosinophilic infiltration
• Subcutaneous nodules
• Livedo reticularis
|Vasculitis||x||• Leukocytoclastic vasculitis|
• Extravascular granulomas
• Dermal eosinophilic infiltration
*Spectrum of signs, symptoms, and syndromes described in patients with EGPA. **Alveolar hemorrhage more frequently present in ANCA+ patients
#Pulmonary infiltrates more frequently present in ANCA – patients
ANCA, Antineutrophil cytoplasmic antibodies.