Churg-Strauss syndrome classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [3]
Overview
Overview
Revised international chapel hill consensus conference 2012 on nomenclature of vasculitides, defines eosinophilic granulomatosis with polyangiitis (formerly known as Churg – Strauss syndrome) as an eosinophilic, granulomatous inflammatory disease affecting most commonly the conducting pulmonary airways, and leading to a necrosis of the small and/or medium sized vessels. Eosinophilic granulomatosis with polyangiitis is often synonymous with adult-onset asthma. According to revised CHCC 2012, eosinophilic granulomatosis with polyangiitis is considered as a variant of the ANCA – associated vasculitides.
Classification
Classification
- According to the revised international chapel hill consensus conference 2012, eosinophilic granulomatosis with polyangiitis is described as an eosinophilic, granulomatous inflammatory disease affecting most commonly the conducting pulmonary airways, and leading to a necrosis of the small and/or medium sized vessels. Eosinophilic granulomatosis with polyangiitis is often present with adult-onset asthma.[1]
- The name Churg–Strauss syndrome, has been replaced by the 2012 revised international chapel hill consensus conference, by focusing on the histopathology of the disease. Eosinophilic granulomatosis with polyangiitis can be considered a variant of the ANCA associated vasculitis of the small vessels.[2]
- The revised CHCC 2012 nomenclature of vasculitides has stated that anti-neutrophil cytoplasmic antibodies (ANCA) are found in eosinophilic granulomatosis with polyangiitis, particularly in patients with glomerulonephritis. This evidence may support to describe subsets of of eosinophilic granulomatosis with polyangiitis, wheather the presence or the absence of ANCA.
- The chapel hill consensus conference, however, does not propose any diagnostic or classification criteria.
- Therefore, the diagnostic criteria proposed by the american college of rheumatology (ACR) in 1990 on eosinophilic granulomatosis with polyangiitis is still being used to distinguish the disease. The presence of 4 out of the 6 diagnostic criteria has a reported 85% sensitivity and 99.7% specificity for the diagnosis of eosinophilic granulomatosis with polyangiitis.[3][4]
| American College of Rheumatology (ACR) Classification of Eosinophilic granulomatosis with polyangiitis |
|---|
| Asthma |
| Eosinophilia (>10% of total WBC) |
| Neuropathy (mononeuropathy or polyneuropathy) |
| Pulmonary infiltrate |
| Paranasal sinus abnormality |
| Biopsy that shows extravascular eosinophil infiltration |
References
References
- ↑ Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA (January 2013). “2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides”. Arthritis Rheum. 65 (1): 1–11. doi:10.1002/art.37715. PMID 23045170.
- ↑ Mouthon L, Dunogue B, Guillevin L (2014). “Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome)”. J. Autoimmun. 48-49: 99–103. doi:10.1016/j.jaut.2014.01.018. PMID 24530234.
- ↑ Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP; 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): 1094–100. PMID 2202307.
- ↑ Gioffredi A, Maritati F, Oliva E, Buzio C (2014). “Eosinophilic granulomatosis with polyangiitis: an overview”. Front Immunol. 5: 549. doi:10.3389/fimmu.2014.00549. PMC 4217511. PMID 25404930.
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