Differentiating Churg-Strauss syndrome from other diseases
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
- 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
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
- ↑ 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.