Churg-Strauss syndrome natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
OR
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
OR
Natural History, Complications, and Prognosis
Natural History
- The symptoms of (disease name) usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
- The symptoms of (disease name) typically develop ___ years after exposure to ___.
- If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
Complications
- Complications of eosinophilic granulomatosis with polyangiitis depend on the specific organ involved in the disease process.
- Heart:[1]
- Lung:[2]
- Pleural effusion
- Pulmonary hypertension
- Alveolar hemorrhage
- Status asthamaticus
- Kidney:[3]
- GI tract:[4]
- Gastrointestinal bleeding
- Acute abdominal pain or intestinal angina
- Neurologic:[5]
- Skin:[6]
Prognosis
- Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated.
- Treatment with glucocorticoids has been shown to improve prognosis.[7]
- Prognosis of Eosinophilic granulomatosis with polyangiitis is most likely dependent on stage at which the disease was diagnosed and organ involvement.
- The following are favorable prognostic factors:[8]
- Early diagnosis of the disease
- Less cutaneous involvement
- Elevated C-reactive protein
- The following are poor prognostic factors:
- Five-Factor Score Assessment (FFS) :[9]
- Cardiac involvement(cardiomyopathy)
- Severe GI manifestations(ulcers, perforations, bleeding)
- Proteinuria > 1g/d
- Renal involvement(Creatinine > 1.4 mg/dl)
- Cns involvement
- Five-Factor Score Assessment (FFS) :[9]
- In 2011, two more criteria are added to to the FFS, include age of the patient >65 years, ear, nose, and throat involvement. They removed CNS involvement from earlier criteria.[10]
- Revised FFS assessment criteria:
- Cardiomyopathy
- Severe GI manifestations(ulcers, perforations, bleeding)
- Proteinuria > 1g/d
- Renal involvement(Creatinine > 1.6 mg/dl)
- Age > 65 yrs
- Revised FFS assessment criteria:
- The FFS is a good predictor of death. It can be used to choose the appropriate treatment. Renal and gastrointestinal involvement are the most common serious prognostic factors.[9]
| Five factor score | 5 year mortality rate |
|---|---|
| 0 | 11.9% |
| 1 | 25.9% |
| >2 | 45.95% |
References
- ↑ Aakerøy L, Amundsen BH, Skomsvoll JF, Haugen BO, Soma J (March 2011). "A 50-year-old man with eosinophilia and cardiomyopathy: need for endomyocardial biopsy?". Eur J Echocardiogr. 12 (3): 257–9. doi:10.1093/ejechocard/jeq167. PMID 21138993.
- ↑ Lai RS, Lin SL, Lai NS, Lee PC (1998). "Churg-Strauss syndrome presenting with pulmonary capillaritis and diffuse alveolar hemorrhage". Scand. J. Rheumatol. 27 (3): 230–2. PMID 9645420.
- ↑ Clutterbuck EJ, Evans DJ, Pusey CD (1990). "Renal involvement in Churg-Strauss syndrome". Nephrol. Dial. Transplant. 5 (3): 161–7. PMID 2113641.
- ↑ Kaneki T, Kawashima A, Hayano T, Honda T, Kubo K, Koizumi T, Sekiguchi M, Ichikawa H, Matsuzawa K, Katsuyama T (February 1998). "Churg-Strauss syndrome (allergic granulomatous angitis) presenting with ileus caused by ischemic ileal ulcer". J. Gastroenterol. 33 (1): 112–6. PMID 9497232.
- ↑ Wolf J, Bergner R, Mutallib S, Buggle F, Grau AJ (April 2010). "Neurologic complications of Churg-Strauss syndrome--a prospective monocentric study". Eur. J. Neurol. 17 (4): 582–8. doi:10.1111/j.1468-1331.2009.02902.x. PMID 20050889.
- ↑ Lestre S, Serrão V, João A, Pinheiro S, Lobo L (2009). "[Churg-Strauss syndrome presenting with cutaneous vasculitis]". Acta Reumatol Port (in Portuguese). 34 (2A): 281–7. PMID 19569284.
- ↑ Guillevin L (October 2008). "Advances in the treatments of systemic vasculitides". Clin Rev Allergy Immunol. 35 (1–2): 72–8. doi:10.1007/s12016-007-8068-4. PMID 18181034.
- ↑ Kim MY, Sohn KH, Song WJ, Park HW, Cho SH, Min KU, Kang HR (January 2014). "Clinical features and prognostic factors of Churg-Strauss syndrome". Korean J. Intern. Med. 29 (1): 85–95. doi:10.3904/kjim.2014.29.1.85. PMC 3932399. PMID 24574837.
- ↑ 9.0 9.1 Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P (January 1996). "Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients". Medicine (Baltimore). 75 (1): 17–28. PMID 8569467.
- ↑ Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (January 2011). "The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort". Medicine (Baltimore). 90 (1): 19–27. doi:10.1097/MD.0b013e318205a4c6. PMID 21200183.