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+ Phenotype | ANCA – Phenotype | Pathology |
| Ear, nose and throat | • Allergic rhinitis • Sinusitis • Nasal polyposis | Prodromal | • Granulomas • Eosinophilic infiltrates • Vasculitis rare | ||
| Pulmonary | • Asthma • Pulmonary infiltrates (transient) or nodules • Pleural effusions • Alveolar hemorrhage (rare) | Prodromal, Eosinophilic, Vasculitis | x** | x# | • Eosinophilic infiltration •Extravascular granulomas • Necrotizing vasculitis |
| Cardiac | • Congestive heart failure • Pericarditis/ Pericardial effusion • Myocarditis • Myocardial infarction • Arrhythmia • Valvular defects | Eosinophilic, Vasculitis | x | • Endomyocardial and pericardial eosinophilic infiltration • Coronary vasculitis (rare) | |
| Gastrointestinal | • Abdominal pain • Diarrhea • Bleeding • 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 | |
| Skin | • Purpura • Subcutaneous nodules • Livedo reticularis • Urticaria | 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.