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Churg-Strauss Syndrome
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- 2. Outline: Introduction Definition Epidemiology Pathogenesis Clinical manifestation Diagnosis Treatment Prognosis Summary
- 3. Introduction Churg andStrauss, 1951 Eosinophilic Granulomatosis with polyangiitis (EGPA) Uncommon Vasculitis disease Churg J, Strauss L (1951) Allergic granulomatosis, allergic angiitis and periarteriitis nodosa. Am J Pathol 27:277–301 Umezawa N, Kohsaka H, Nanki T et al (2014) Successful treatment of eosinophilic granulomatosis with polyangiitis (EPGA; formerly Churg-Strauss syndrome) with rituximab in a case refractory to glucocorticoids, cyclophosphamide, and IVIG. Mod Rheumatol 24:685–687
- 4. Definition A small-vesselvasculitis Eosinophil-rich and granulomatous inflammation involving the respiratory tract, with necrotizing vasculitis affecting small- to medium-sized vessels, associated with asthma and eosinophilia Which combines clinical and pathologic features of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) Association with p-ANCA. Jenette JC, Falk RJ, Andrasy K et al (1994) Nomenclature of systemic vasculitides. Proposal of an international Consensus Conference. Arthritis Rheum 37:187–192
- 5. Epidemiology Prevalence:1–2/100,000 Male:femaleis 2–3:1. Mean age of disease onset is 40 years of age. Stübiger, N., & Zierhut, M. (2016). Churg-Strauss Syndrome. In Intraocular Inflammation (pp. 761-765). Springer Berlin Heidelberg.
- 6. Pathogenesis Eosinophilic granulocytes Strong Th2-type immune response Th2- associated cytokines such as IL-4, IL-13, and IL-5 may induce the severe eosinophilia Prolonged survival of eosinophils due to inhibition of CD95-mediated apoptosis by soluble CD95 In < 50 %, autoantibodies, such as ANCA, are found. These ANCA are usually directed against myeloperoxidase (MPO-ANCA) and associated with a perinuclear (p-ANCA) pattern of immunofluorescence. Hellmich B, Ehlers S, Csernok E (2003) Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol 21:69–77 Stone JH (2007) Vasculitis: a collection of pearls and myths. Rheum Dis Clin North Am 33:691–739 Vitali C, Genovesi-Ebert F, Romani R (1996) Ophthalmological and neuroophthalmological involve-ment in Churg-Strauss syndrome. Arch Clin Exp Ophthalmol 234:404–408 Zwerina J, Axmann R, Jatzwauk M et al (2009) Pathogenesis of Churg- Strauss syndrome: recent insights. Autoimmunity 42:376–379
- 7. Clinical Manifestation pulmonaryand systemic vasculitis, extravascular granulomas, and eosinophilia, occurring almost exclusively in patients with asthma or a history of allergy. Angiitis and extravascular necrotizing granulomas usually with eosinophilic infiltrates Retinal vasculitis amaurosis fugax or CRAO Lie JT (1986) The classification of vasculitis and a reappraisal of allergicgranulomatosis and angiitis (Churg-Strauss syndrome). Mt Sinai J Med (NY)53:429–439 Lie JT, Members and Consultants of the American College of Rheumatology Subcommittee on classification of vasculitis (1990) Illustrated histopathologic classification criteria for selected vasculitis syndromes. Arthritis Rheum 33:1074–1087 Ooi KGJ, Drummond SR, Thompson KJet al (2004) Churg-Strauss syndrome presenting with conjunctival nodules in association with Candida albicans and ankylosing spondylitis. Clin Experiment Ophthalmol 32:441–442 (letter) Vitali C, Genovesi-Ebert F, Romani R (1996) Ophthalmological and neuroophthalmological involve-ment in Churg-Strauss syndrome. Arch Clin Exp Ophthalmol 234:404–408
- 8. Diagnosis (American College ofRheumatology, 1990) Asthma Eosinophilia greater than 10 % on differential white blood cell count Mononeuropathy (including multiplex) or polyneuropathy Non-fixed pulmonary infiltrates on X-ray Paranasal sinus abnormality Biopsy containing a blood vessel with extravascular eosinophils sensitivity of 85 % and a specificity of 99.7 % If a patient has hypereosinophilia and a documented history of either asthma or allergy, then the diagnosis of CSS can also be established Masi AT, Hunder GG, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33(8):1094 1100
- 9. Treatment High doseof corticosteroids (1–2 mg prednisolone/kg of body weight). Taper after 3-4 weeks. In most cases, monotherapy with corticosteroids is efficient, and maintenance treatment is not longer necessary as 1 year Azatiophrine for patients with steroid-resistant In case of active vasculitis and neuropathy pulsed doses of 500–1,000 mg/m2 of cyclophosphamide intravenously could be given The FAUCI-Schema (cyclophosphamide 1–2 mg/kg bw daily) is indicated if cardiac or CNS involvement is present A single dose of 500 mg/m2 of cyclophosphamide for life-threatening condition Stübiger, N., & Zierhut, M. (2016). Churg-Strauss Syndrome. In Intraocular Inflammation (pp. 761-765). Springer Berlin Heidelberg.
- 10. Treatment IVIG Interferon-alfafor therapy-resistant patients The chimeric anti-CD20 monoclonal antibody rituximab (RTX) (for 4 weeks, 375 mg/m2/week) was effective Stübiger, N., & Zierhut, M. (2016). Churg-Strauss Syndrome. In Intraocular Inflammation (pp. 761-765). Springer Berlin Heidelberg.
- 11. Prognosis Milder thanof GPA and MPA The 1-year survival rate is 90 % and the 5-year survival rate is 62 % The mortality correlates primarily with the severity of cardiac involvement, followed by CNS and renal manifestations Stübiger, N., & Zierhut, M. (2016). Churg-Strauss Syndrome. In Intraocular Inflammation (pp. 761-765). Springer Berlin Heidelberg.
- 12. Summary Uncommon eosinophilicvasculitis disease ACR 1990 diagnostic criteria CSS often overlaps with GPA and MPA; all three are ANCA-associated vasculitides. Treatment: corticosteroids (or immunosuppresants)
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