Churg-Strauss syndrome natural history, complications and prognosis: Difference between revisions
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===Prognosis=== | ===Prognosis=== | ||
*Prognosis is | *Prognosis of eosinophilic granulomatosis with polyangiitis is poor when untreated. | ||
* | *Treatment with glucocorticoids has been shown to improve prognosis.<ref name="pmid18181034">{{cite journal |vauthors=Guillevin L |title=Advances in the treatments of systemic vasculitides |journal=Clin Rev Allergy Immunol |volume=35 |issue=1-2 |pages=72–8 |date=October 2008 |pmid=18181034 |doi=10.1007/s12016-007-8068-4 |url=}}</ref> | ||
*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: | |||
**Early diagnosis of the disease | |||
**Decreased cutaneous involvement | |||
**Elevated C-reactive protein | |||
**The following are poor prognostic factors: | |||
**Five-Factor Score Assessment | |||
***Proteinuria > 1g/d | |||
Creatinine > 140mm/l | |||
Cardiomyopathy | |||
Severe GI manifestations | |||
CNS involvement | |||
In the past the prognosis of Eosinophilic granulomatosis with polyangiitis was fatal when untreated. Patients who were not treated had an estimated 5 year survival rate of 20 to 30%. Today, the prognosis of Eosinophilic granulomatosis with polyangiitis is heavily dependent on starting treatment with a glucocorticoid. | |||
The following are favorable prognostic factors:[2] | |||
Early diagnosis of the disease | |||
Decreased cutaneous involvement | |||
Elevated C-reactive protein | |||
The following are poor prognostic factors: | |||
Five-Factor Score Assessment | |||
Proteinuria > 1g/d | |||
Creatinine > 140mm/l | |||
Cardiomyopathy | |||
Severe GI manifestations | |||
CNS involvement | |||
According to the Five-Factor Score, a score of 0, 1, or greater than 2 has the following mortality rate: | |||
Five-Factor Score | |||
Score Mortality Rate | |||
0 12% | |||
1 26% | |||
>2 46% | |||
[3] It has also been concluded that age greater than 65 is also a risk factor for mortality.[4] | |||
==References== | ==References== | ||
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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
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
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
- 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 when 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:
- Early diagnosis of the disease
- Decreased cutaneous involvement
- Elevated C-reactive protein
- The following are poor prognostic factors:
- Five-Factor Score Assessment
- Proteinuria > 1g/d
Creatinine > 140mm/l Cardiomyopathy Severe GI manifestations CNS involvement
In the past the prognosis of Eosinophilic granulomatosis with polyangiitis was fatal when untreated. Patients who were not treated had an estimated 5 year survival rate of 20 to 30%. Today, the prognosis of Eosinophilic granulomatosis with polyangiitis is heavily dependent on starting treatment with a glucocorticoid.
The following are favorable prognostic factors:[2]
Early diagnosis of the disease Decreased cutaneous involvement Elevated C-reactive protein The following are poor prognostic factors:
Five-Factor Score Assessment Proteinuria > 1g/d Creatinine > 140mm/l Cardiomyopathy Severe GI manifestations CNS involvement According to the Five-Factor Score, a score of 0, 1, or greater than 2 has the following mortality rate: Five-Factor Score Score Mortality Rate 0 12% 1 26% >2 46% [3] It has also been concluded that age greater than 65 is also a risk factor for mortality.[4]
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.