Churg-Strauss Syndrome

Abstract

Eosinophilic granulomatosis with polyangiitis, EGPA (formerly called as Churg Strauss syndrome) is the rarest of the three ANCA associated small vessel vasculitidies (AAVs). The clinical presentation has three prototypical phases, the first phase manifests as asthma or with allergic manifestations. The second phase is characterized by blood and/or tissue eosinophilia and the third phase presents as vasculitis manifesting with skin rash/peripheral nervous system involvement. Constitutional symptoms like fever, myalgias, arthralgias, and weight loss are common at the time of diagnosis. Rhinitis, polyposis, and/or asthma are present in all patients. Skin manifestations may vary from macules, papules, nodules, urticaria to livedo reticularis, ulcerations, and erythema multiforme. Mononeuritis multiplex is the most common peripheral nervous system involvement. Cardiac involvement, though less common, is associated with increased mortality. Kidney involvement is mostly focal segmental and pauciimmune and is not as common as in other AAVs like granulomatosis with polyangiitis (previously Wegener’s) and microscopic polyangiitis. Other organs can also be involved, but eye involvement is uncommon with case reports of conjunctivitis, scleritis, uveitis, corneal ulceration, and exophthalmos. The ANCA positivity is seen in less than half of these patients.