Eosinophilic granulomatosis with polyantiitis

Background

• Small-vessel ANCA-associated vasculitis
• Also known as Churg-Strauss disease

Clinical Manifestations

• Thought to follow separate stages, though there is overlap:
  • Prodromal phase, with atopy, allergic rhinitis, asthma, chronic rhinosinusitis
  • Eosinophilic phase, with peripheral eosinophilia (greater than 0.5), with a mean eosinophil count of 7.5
  • Vasculitic phase, with the development of end-organ damage from vasculitis, often with fevers, malaise, and weight loss
• Asthma (90%), which precedes vasculitis by around 10 years
• Allergic rhinitis, recurrent sinusitis, nasal polyposis, nasal obstruction, characterized by nasal discharge (90%), congestion (85%), facial pain/pressure (85%)
• Skin involvement during the vasculitic phase, with tender subcutaneous nodules particularly on the elbows, hands, and legs
• Peripheral neuropathy (mononeuritis multiplex) (75%)
• Kidney injury (25%)
• Eosinophilic gastritis or enteritis, with abdominal pain (605), diarrhea (30%), GI bleed (20%)
• Myalgias and arthralgias and even arthritis (40-50%)
• Lymphadenopathy (30-40%)