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 polyangiits must be differentiated from other diseases that can cause purpura, alveolar hemorrhage, fever, arthralgia, myalgia, necrotizing extra-capillary glomerulonephritis, such as Granulomatosis with polyangiitis and Microscopic polyangiitis.[1]

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
Proteinase 3 antigen

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

  • Parasitic infections
  • Drugs
  • Allergic bronchopulmonary aspergillosis

Perinuclear ANCA

  • Cystic Fibrosis
  • Bronchogenic carcinoma
  • Inflammatory bowel disease
  • Sclerosing cholangitis
  • Myeloproliferative disorders
  • Systemic lupus erythematosis
  • Rheumatoid arthritis

Diagnosis of Eosinophilic granulomatosis with polyangiitis

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

  • Asthma
  • Eosinophilia
  • Polyneuropathy or Mononeuropathy
  • Non fixed pulmonary infiltrates
  • Paranasal sinus that is abnormal
  • Eosinophil's that are extravascular

Patients must express 4 out the 6 criteria to be diagnosed with Eosinophilic granulomatosis with polyangiitis. [3]


References

  1. 1.0 1.1 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.

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