Churg-Strauss syndrome natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [2]

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.

• 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 of eosinophilic granulomatosis with polyangiitis depend on the specific organ involved in the disease process.
  • Heart:^[2]
    • Pericarditis
    • Myocarditis
    • Pericardial effusion
    • Myocardial infarction
    • Cardiac tamponade
  • Lung:^[3]
    • Pleural effusion
    • Pulmonary hypertension
    • Alveolar hemorrhage
    • Status asthmaticus
  • Kidney:^[4]
    • Glomerulonephritis
    • Renal failure
  • GI tract:^[5]
    • Gastrointestinal bleeding
    • Acute abdominal pain or intestinal angina
  • Neurologic:^[6]
    • peripheral neuropathy
    • Cerebral hemorrhage
  • Skin:^[7]
    • Purpura
    • Petichiae
    • Noduluar lesions on extensor surfaces

• Prognosis of eosinophilic granulomatosis with polyangiitis is poor if left untreated.
• Treatment with glucocorticoids has been shown to improve prognosis.^[8]
• Prognosis 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
• 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
• 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]

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