What are the signs and symptoms of EGPA (Churg-Strauss syndrome)?

Updated: Dec 24, 2018
  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

The following symptoms and signs of the disease were reported by Guillevin et al in their case series [3] :

  • Constitutional symptoms - Malaise, fatigue, flulike symptoms, weight loss (70%), fever (57%), myalgias (52%)

  • Asthma symptoms - Asthma is a central feature of EGPA, occurring in 97% of patients. Asthma may precede vasculitis by up to 10 years or, less frequently, may coincide with the appearance of vasculitis. Asthma symptoms are usually persistent; therefore, patients are usually treated with steroids. This, in turn, might mask other features of the syndrome. [21]

  • Paranasal sinusitis (61%) - Usually responds to oral steroids

  • Allergic rhinitis - This is a common symptom. Additionally, recurrent sinusitis and polyposis are seen, but unlike in granulomatosis with polyangiitis [Wegener granulomatosis], necrotizing lesions of the upper airway are unusual.

  • Pulmonary symptoms (37%), including cough and hemoptysis

  • Arthralgias (40%)

  • Skin manifestations (49%)

  • Purpura - Skin nodules, urticarial rash, necrotic bulla, digital ischemia

  • Cardiac manifestations - Symptoms related to heart failure, myocarditis, pericarditis, constrictive pericarditis, and myocardial infarction

  • Gastrointestinal (GI) symptoms (31%) - Symptoms related to GI vasculitis, eosinophilic gastritis, or colitis; these include abdominal pain (59%), diarrhea (33%), and GI bleeding (18%).

  • Peripheral neuropathy - Mononeuritis multiplex (most frequent form, occurring in as many as 77% of patients)

  • Less frequent symptoms - Symptoms related to stroke, ophthalmologic involvement, and other rare symptoms


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