Introduction: Granulomatosis with polyangiitis (Wegener’s) (GPA) is a necrotizing-type of granulomatous inflammation that usually involves the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) presents as a necrotizing granulomatous inflammation often involving the respiratory tract, associated with asthma, eosinophilia, and often nasal polyps.
Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis or overlaps several vasculitides categories. Several polyangiitis overlap syndromes have been identified, however, to our knowledge, there are very few case reports of an overlap syndrome involving both GPA and EGPA.
Case Presentation: A 50-year-old woman with a past medical history of childhood allergic rhinitis and asthma with recurring upper respiratory tract infections, sinusitis, and bronchitis initially presented with an episode of hemoptysis. She exhibited oral ulcers and peripheral neuropathy.
Laboratory findings included a complete blood count with a total white cell count of 11,000 cells per microliter, and differential showing markedly increased eosinophils at 5200 cells per microliter. Quantitative serum immunoglobulin E was elevated at 520 international units/mL.
Based on these findings, she fit criteria by the American College of Rheumatology for GPA and EGPA and was subsequently treated with corticosteroids in combination mepolizumab and azathioprine.
Discussion: To our knowledge, there have been very few cases reported of polyangiitis overlap syndrome of GPA and EGPA. Identification of patients with polyangiitis overlap syndrome of GPA and EGPA is imperative because treatment options differ. The majority of patients with EGPA are able to achieve remission with corticosteroids and now anti-IL5 therapy,
Full conference title:
- ATS 2018