A rare case report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

Abstract

Background: Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature.

Case presentation: We conducted a PUBMED literature review using key words 'granulomatosis with polyangiitis,' 'Wegener's,' 'GPA,' 'eosinophilic granulomatosis with polyangiitis,' 'Churg-Strauss,' 'EGPA,' 'overlap syndrome,' 'Wegener's with eosinophilia,' and 'GPA with eosinophilia' in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described. Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. Patients ranged in age between 21 and 78. Of those whose gender was identified, 80 % of the patients were female. All cases described involved the lungs, 60 % reported sinus involvement, and more than 50 % displayed renal involvement. An overwhelming majority of patients were positive for c-ANCA and demonstrated eosinophilia (peripheral blood or tissue eosinophilia). A preponderance of the cases described were treated with systemic corticosteroids combined with an immunosuppressive/cytotoxic agents.

Conclusion: To our knowledge, there have been very few cases reported of an overlap syndrome of GPA and EGPA. Identification of patients with a polyangiitis overlap syndrome of GPA and EGPA is imperative as prognosis, longitudinal management and treatment modalities may differ between these entities.

Keywords: Churg-Strauss; EGPA; Eosinophilic granulomatosis with polyangiitis; GPA; GPA with eosinophilia; Granulomatosis with polyangiitis; Overlap syndrome; Wegener’s; Wegener’s with eosinophilia.

Fig. 1

CT imaging revealing left upper lobe consolidation with central cavitary lesion, adjacent scattered consolidation, ground-glass opacities and tree-in-bud markings

Fig. 2

20X field of an H&E stain (hematoxylin and eosin stain) with perivascular eosinophils and a neighboring airspace with a plug of organizing pneumonia

Fig. 3

20X field of H&E stain with perivascular eosinophils

Fig. 4

20X field of H&E stain with plugs of organizing pneumonia and fibrin

Fig. 5

Follow up CT imaging with resolving left upper lobe cavitary lesion after initiation of corticosteroids, azathioprine and anti-IL-5