eosinophilic granulomatosis with polyangitis aka churg strauss

Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis that can affect multiple organs. Key to its diagnosis are the following features: history of asthma, greater than 10% eosinophils in the peripheral blood cell count, mononeuropathy, migratory or transient pulmonary infiltrates, paranasal sinus involvement, and biopsy results demonstrating a blood vessel with accumulation of eosinophils in extravascular areas. Peripheral blood eosinophil counts are typically greater than 1,500/μL (1.5 × 109/L), (ie, significantly higher than in this patient). Disease involvement should be shown to include 2 or more organs. Cardiac manifestations, including pericarditis and myocarditis, may occur. Renal involvement is relatively common. This patient shows no evidence of vasculitis, and his IgE levels are lower than expected for EGPA (choice B is incorrect).2

Demographics

  • young adult

Pathogenesis

  • small to medium vessel vasculitis

Symptoms

  • adult onset asthma with palpable purpura refractory to medical treatment
  • atopy history, sinusitis
  • less renal involvement
  • eosinophilia
  • p-ANCA positive
  • Atopy and vasculitis
  • asthma
  • Asymmetric multifocal neuropathy (mononeuritis multiplex) is particularly common due to the vasculitis affecting the epineural vessels (eg, wrist drop due to radial nerve involvement).
  • Other common manifestations include skin nodules, migratory/transient pulmonary infiltrates, and paranasal sinus abnormalities. In addition to peripheral eosinophilia, a frequent laboratory finding is antibodies against neutrophil myeloperoxidase, which most commonly have a pattern of perinuclear staining (p-ANCA).1

Footnotes

  1. Uworld Questionnaires ↩ ↩2

  2. SEEK Questionnaires ↩