Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): state of the art
- 18 January 2013
- Vol. 68 (3), 261-273
- https://doi.org/10.1111/all.12088
Abstract
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) is a systemic small-vessel vasculitis associated with asthma and eosinophilia. Histology of EGPA shows tissue eosinophilia, necrotizing vasculitis, and eosinophil-rich granulomatous inflammation. EGPA commonly presents with upper airway tract and lung involvement, peripheral neuropathy, cardiac and skin lesions. Antineutrophil cytoplasmic antibodies (ANCA) are positive in ~40% of the cases and more often in patients with clinical manifestations due to small-vessel vasculitis. The pathogenesis of EGPA is multifactorial: the disease can be triggered by exposure to allergens or drugs, but a genetic background has also been recognized, particularly an association with HLA-DRB4. Th2 responses are prominent, with up-regulation of IL-4, IL-13, and IL-5; however, Th1 and Th17 responses are not negligible. Eosinophils are activated, have a prolonged lifespan and probably cause tissue damage by releasing their granule proteins; their tissue recruitment can be regulated by chemokines such as eotaxin-3 and CCL17. Humoral immunity is also dysregulated, as demonstrated by prominent IgG4 and IgE responses. EGPA promptly responds to glucocorticoid therapy, although combinations of glucocorticoids and immunosuppressants (e.g., cyclophosphamide, azathioprine) are eventually required in most cases. Newer therapeutic options include the anti-IL5 antibody mepolizumab, whose efficacy has been described in small clinical trials, and the B-cell-depleting agent rituximab, reported in several case series.Keywords
This publication has 78 references indexed in Scilit:
- A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patientsAnnals Of The Rheumatic Diseases, 2012
- IgG4 immune response in Churg–Strauss syndromeAnnals Of The Rheumatic Diseases, 2011
- Rituximab for the treatment of Churg-Strauss syndrome with renal involvementNephrology Dialysis Transplantation, 2011
- Diagnosis of Churg-Strauss syndrome: eotaxin-3 makes it easierRheumatology, 2011
- Eotaxin-3 in Churg-Strauss syndrome: a clinical and immunogenetic studyRheumatology, 2011
- Refining the definition of hypereosinophilic syndromeJournal of Allergy and Clinical Immunology, 2010
- Churg-Strauss syndrome manifested by appendicitis, cholecystitis and superficial micronodular liver lesions--an unusual clinicopathological presentationJournal of Clinical Pathology, 2010
- Cardiac involvement in Churg‐Strauss syndromeArthritis & Rheumatism, 2010
- Cardiac Involvement in Churg-Strauss SyndromeMedicine, 2009
- Peripheral neuropathy in Wegener's granulomatosis, Churg Strauss syndrome and microscopic polyangiitisJournal of Neurology, Neurosurgery & Psychiatry, 2007