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 | 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
- 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:
According to the American College of Rheumatology classification criteria [3]
| 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. |
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
- ↑ 1.0 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.
- ↑ Conron M, Beynon HL (2000). "Churg-Strauss syndrome". Thorax. 55 (10): 870–7. PMC 1745623. PMID 10992542.
- ↑ 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.
- ↑ 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.