Eosinophilic Granulomatosis with Polyangiitis (EGPA) (Churg-Strauss Syndrome, Allergic Angiitis and Granulomatosis)


Epidemiology


Physiologic and Pathologic Features


Diagnosis


Clinical Criteria

1990 Am Coll of Rheumatology Criteria

(with confirmation of necrotizing vasculitis on biopsy, sensitivity of criteria is 85% and specificity is 99.7%)

Three Phases of Disease

(although these phases may develop sequentially over years, features may present in other sequences)


Clinical Manifestations

Cardiovascular Manifestations (15-50% of cases)

(cardiac lesions are the cause of death in about 50% of cases)

Dermatologic Manifestations (50-70% of cases)

Gastrointestinal Manifestations (15-60% of cases)

Hematologic Manifestations

Hepatic Manifestations

Neurologic Manifestations

Otolaryngologic Manifestations

Pulmonary Manifestations (50% of cases)

(pulmonary symptoms occur in almost all patients and may precede the development of other manifestations by years)

Renal Manifestations (25-60% of cases)

Rheumatologic Manifestations

Prostate/Lower Urinary Tract Manifestations

Ocular Manifestations

Vascular Manifestations

Constitutional Manifestations (common)


Treatment

European Vasculitis Study Group Recommendations for Induction Therapy

(induction therapy is typically given until remission has been achieved or for the first 1 year)

Maintenance Therapy

Side Effects of Therapy

Relapse


Prognosis


References

Diagnosis

Clinical

Treatment