A Rare Case of Polyangiitis Overlap Syndrome: Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis

M. Quan1, S. K. Frankel2, M. Maleki3, L. Tan4;

Author address: 

1Internal Medicine, Arrowhead Regional Medical Center, Colton, CA, United States, 2Division of Pulmonary, Critical Care and Sleep Medicine, Natl Jewish Med & Research Ctr, Denver, CO, United States, 3Division of Rheumatology, National Jewish Health, Denver, CO, United States, 4Department of Pulmonary, Critical Care, Hyperbaric and Sleep Medicine, Loma Linda University Medical Center, Loma Linda, CA, United States.

Abstract: 

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. Patient was negative for ANCA, hepatitis B or C, PR3 and MPO antibodies. Chest computed tomography imaging revealed left upper lobe consolidation with a central cavitary lesion, scattered consolidations, ground-glass opacities and tree-in-bud markings. Previous bronchoscopy were culture positive for mycobacteriumavium as well as Aspergillus and despite aggressive multidrug and fungal therapy patient did not exhibit significant clinical improvement. Repeat bronchoscopy with left lower lobe transbronchial biopsy showed acute and chronic inflammation, with infiltration of scattered eosinophils, and fresh hemorrhage.
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, however patients with GPA or polyangiitis overlap syndrome often require a combination of corticosteroids, biologic therapy and immunosuppression.

2018

abstract No: 

A3004 / P636

Full conference title: 

The American Thoracic Society Conference 2018
    • ATS 2018