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: Chandrakala Yannam, MD [2]
Overview
Eosinophilic granulomatosis with polyangiitis develops through three phases, include prodromal phase, eosinophilic phase, vasculitic phase. Most complications result from the vasculitic phase. Most common complications include cardiomyopathy, myocardial infarction, perimyocarditis, rapidly progressive renal failure, GI bleeding, neuropathy and status asthmaticus. Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated. Prognosis is most likely dependent on stage at which the disease was diagnosed and organ involvement. The five-factor score assessment (FFS) is a good predictor of survival rate. It can be used to choose the appropriate treatment.
Natural History, Complications, and Prognosis
Natural History
- Classically, three phases of Eosinophilic granulomatosis with polyangiitis are seen. They are distributed as follows:[1]
- Prodromal phase: This stage occurs in the teenage years through the 20’s.
- Eosinophilic Phase: Peripheral blood eosinophilia is present. The second stage involves the onset of acute asthma.
- People can live for many years in the first two stages before progressing to stage three.
- The Vasculitic Phase: The third phase occurs in the 40’s-50’s and involves multiple organ systems in a life-threatening systemic vasculitis of small and medium-sized vessels.
- If eosinophilic granulomatosis with polyangiitis left untreated, death occurs most commonly from the following complications:
- Cardiac failure
- Myocardial infarction
- Rapidly progressive renal failure
- Cerebral hemorrhage
- Gastrointestinal bleeding/perforation
- Status asthmaticus
Complications
- Complications of eosinophilic granulomatosis with polyangiitis depend on the specific organ involved in the disease process.
- Heart:[2]
- Lung:[3]
- Pleural effusion
- Pulmonary hypertension
- Alveolar hemorrhage
- Status asthmaticus
- Kidney:[4]
- GI tract:[5]
- Gastrointestinal bleeding
- Acute abdominal pain or intestinal angina
- Neurologic:[6]
- Skin:[7]
Prognosis
- Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated.
- Treatment with glucocorticoids has been shown to improve prognosis.[8]
- 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:[9]
- Early diagnosis of the disease
- Less cutaneous involvement
- Elevated C-reactive protein
- The following are poor prognostic factors:
- Five-Factor Score Assessment (FFS) :[10]
- 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) :[10]
- 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.[11]
- 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 survival rate. It can be used to choose the appropriate treatment. Renal and gastrointestinal involvement are the most common serious prognostic factors.[10][12]
| Five factor score | 5 year mortality rate |
|---|---|
| 0 | 11.9% |
| 1 | 25.9% |
| >2 | 45.95% |
References
- ↑ Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M (April 2015). "Churg-Strauss syndrome". Autoimmun Rev. 14 (4): 341–8. doi:10.1016/j.autrev.2014.12.004. PMID 25500434.
- ↑ 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.
- ↑ 10.0 10.1 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.
- ↑ Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, Matthis C, Metzler C, Nölle B, Richardt G, Gross WL (June 2013). "A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients". Ann. Rheum. Dis. 72 (6): 1011–7. doi:10.1136/annrheumdis-2012-201531. PMID 22887848.