Churg-Strauss syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]

Overview

According to the American College of Rheumatology (ACR), Eosinophilic granulomatosis with polyangiitis is classified using 6 criteria.

Classification

According to the 2012 Revised International Chapel Hill Consensus Conference on Nomenclature of Vasculitides (DOI 10.1002/art.37715), Eosinophilic Granulomatosis with Polyangitis (formerly known as Churg-Strauss' syndrome) can be considered a variant of the ANCA associated vasculitides of the small vessels. 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 use.

The presence of 4 out of the 6 diagnostic criteria has a reported sensitivity of 85% and a specificity of 99.7% for the diagnosis of Eosinophilic granulomatosis with polyangiitis[1] (doi: 10.3389/fimmu.2014.00549). They are:

American College of Rheumatology (ACR) Classification of Eosinophilic granulomatosis with polyangiitis
Asthma
Eosinophilia > 10%
Neuropathy (mononeuropathy or polyneuropathy)
Pulmonary infiltrate, non fixed
Paranasal sinus abnormality
Biopsy that shows extravascular eosinophil infiltration

4 out of the 6 criteria are needed to distinguish Eosinophilic granulomatosis with polyangiitis from other vasculitides.

References

  1. 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.

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