Differentiating Churg-Strauss syndrome from other diseases

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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

Eosinophilic granulomatosis with polyangiitis must be differentiated from other diseases that can cause eosinophilia, purpura, alveolar hemorrhage, necrotizing extra-capillary glomerulonephritis, such as granulomatosis with polyangiitis and microscopic polyangiitis.

Differentiating Eosinophilic granulomatosis with polyangiitis from other Diseases

Eosinophilic granulomatosis with polyangiitis must be differentiated from other diseases that cause purpura, alveolar hemorrhage, necrotizing extra-capillary glomerulonephritis, such as granulomatosis with polyangiitis and Microscopic polyangiitis.[1]

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis serological findings
Eosinophilic granulomatosis with polyangiitis Granulomatosis with polyangiitis Microscopic polyangiitis
Cytoplasmic ANCA (cANCA) 90% positive
Perinuclear ANCA (pANCA) 30 to 40% positive 60 to 80% positive
Myeloperoxidase antigen 40% sensitivity 10% sensitivity 30% sensitivity
Proteinase 3 antigen <5% sensitivity 70-80% sensitivity 60% sensitivity

Differentiating Eosinophilic granulomatosis with polyangiitis from other Diseases

Eosinophilic granulomatosis with polyangiitis must be differentiated from other diseases that cause pulmonary eosinophilia and perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) such as:[2]

Pulmonary eosinophilia

Perinuclear ANCA

Diagnosis of Eosinophilic granulomatosis with polyangiitis

In order to make a diagnosis of Eosinophilic granulomatosis with polyangiitis the following criteria must be present:

According to the American College of Rheumatology classification criteria [3]

Asthma

Eosinophilia

Polyneuropathy or Mononeuropathy

Non fixed pulmonary infiltrates

Paranasal sinus that is abnormal

Eosinophils that are extravascular

Patients must express 4 out the 6 criteria to be diagnosed with eosinophilic granulomatosis with polyangiitis.

According to Lanham diagnostic criteria [4]

Asthma

Eosinophilia peak of >1.5x109 cell/L or >10% of the total WBC

Systemic vasculitis, two or greater extra pulmonary sites

All 3 criteria’s need to be present

References

  1. Pagnoux C (2016). "Updates in ANCA-associated vasculitis". Eur J Rheumatol. 3 (3): 122–133. doi:10.5152/eurjrheum.2015.0043. PMID 27733943.
  2. Conron M, Beynon HL (2000). "Churg-Strauss syndrome". Thorax. 55 (10): 870–7. PMC 1745623. PMID 10992542.
  3. 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.
  4. Lanham JG, Elkon KB, Pusey CD, Hughes GR (1984). "Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome". Medicine (Baltimore). 63 (2): 65–81. PMID 6366453.

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