Churg-Strauss syndrome or Eosinophilic Granulomatosis with Polyangiitis

Churg-Strauss syndrome or Eosinophilic Granulomatosis with Polyangiitis


Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA), has peripheral eosinophilia and pulmonary eosinophilia with infiltrates on CXR. The characteristic feature of this disease is Eosinophilic vasculitis which is not seen in other diseases of lungs which have eosinophilia.

A mnemonic to remember Churg-Strauss syndrome is: “CHARM”:

CCardiac manifestation of myocarditis

HHigh eosinophil count with as much 75% of TLC being eosinophils

A Asthma and Allergic rhinitis history

R – Renal involvement with Glomerulonephritis and Rashes with Purpura

M Mononeuritis multiplex (peripheral neuropathy)

Usually, the patient will start having features of asthma and allergic rhinitis by 20-30 years of age. Lateral Peripheral eosinophilia is seen with as much as 75% of TLC being eosinophils. Eosinophils then infiltrate tissues producing neuropathy, myocarditis, eosinophilic gastroenteritis. In late forties or fifties these patients will develop fever, weight loss and malaise with myalgia.


Laboratory Findings

  1. Eosinophilia: Elevated eosinophil count in peripheral blood.
  2. Elevated Acute Phase Reactants: Such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP).
  3. Positive ANCA (Antineutrophil Cytoplasmic Antibodies): Although not always present, ANCA positivity, particularly perinuclear ANCA (p-ANCA), can be seen in a subset of patients.
  4. Chest X-ray: May reveal pulmonary infiltrates, nodules, or cavities
  5. CT Scan: High-resolution CT may show ground-glass opacities, interstitial fibrosis, or other pulmonary abnormalities.
  6. Biopsy Findings: Histopathological examination of affected tissues may reveal eosinophilic infiltration, vasculitis, and granulomatous inflammation.

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