Churg-Strauss syndrome natural history, complications and prognosis
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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
- Classically, three phases of eosinophilic granulomatosis with polyangiitis are seen. They are distributed as follows:
- 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:
- Complications of eosinophilic granulomatosis with polyangiitis depend on the specific organ involved in the disease process.
- GI tract:
- Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated.
- Treatment with glucocorticoids has been shown to improve prognosis.
- Prognosis is most likely dependent on stage at which the disease was diagnosed and organ involvement.
- The following are favorable prognostic factors:
- The following are poor prognostic factors:
- 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.
- 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.
|Five factor score||5 year mortality rate|
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