An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

Surendran, Sujit; Gundappa, Chandramohan; Gandhi, Arun; Kurien, Abraham; Fernando, Edwin

doi:10.4103/1319-2442.206450

Cited by 4 publications

(2 citation statements)

References 2 publications

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“…In her case, distinguishing between the vasculitides was increasingly difficult given conflicting supporting evidence including polyarthritis, pan-sinusitis, positive C-ANCA, rapidly progressive glomerulonephritis and mononeuritis multiplex. By meeting the criteria for both GPA and EGPA, she was diagnosed with polyangiitis overlapping syndrome [ 15 ]. Although previously reported, polyangiitis overlapping syndrome is not commonly recognized.…”

Section: Discussionmentioning

confidence: 99%

A Rare Presentation of Polyangiitis Overlapping Syndrome

Subramanian¹,

Hanchosky²,

Vuyyuru³

et al. 2023

Cureus

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This case follows a 38-year-old Caucasian male with no known medical history who presented to the emergency department for syncope. He also endorsed a two-month history of fevers, weight loss, oral ulcers, rashes, joint swelling and arthralgias. After extensive workup, he was given a working diagnosis of granulomatosis with polyangiitis (GPA). Conflicting diagnostic evidence made it increasingly difficult to distinguish between GPA and eosinophilic granulomatosis with polyangiitis. In conclusion, we believe the patient may be better diagnosed with polyangiitis overlapping syndrome.

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Section: Discussionmentioning

confidence: 99%

A Rare Presentation of Polyangiitis Overlapping Syndrome

Subramanian¹,

Hanchosky²,

Vuyyuru³

et al. 2023

Cureus

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“…Surendran et al identified overlap syndrome in a patient with similar features including the presence of polyarthritis, pan-sinusitis, c-ANCA positivity, rapidly progressive glomerulonephritis, and mononeuritis multiplex arguing for both EGPA and GPA [ 9 ]. The presence of a history of asthma and tissue eosinophilia favored EGPA, while the presence of nasal septal perforation and eye proptosis favored GPA.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2018

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BackgroundGranulomatosis 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 presentationWe 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.ConclusionTo 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.

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Polyangiitis overlap syndrome: a rare clinical entity

et al. 2023

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Polyangiitis overlap syndrome is a rare clinical entity comprising patients with overlapping features of more than one vasculitis, usually eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA). Few cases of polyangiitis overlap syndrome have been described in the literature, mostly associated with c-ANCA, anti-proteinase (PR)-3 positivity, a protean clinical picture characterized by vasculitis, eosinophilia and eosinophilic infiltrates in tissues and a favorable response to steroids and immunosuppressant treatments. Herein, we present a case of a 66-year-old woman with nasal obstruction, external nose deformity, sensorineural hearing loss, peripheral blood eosinophilia, high titer anti-PR3 antibodies and lung involvement. Nasal septum biopsies showed inflammatory infiltrate with eosinophilic component; histopathology of the lung demonstrated necrotizing granulomas associated with inflammatory infiltrate composed of numerous neutrophils and some eosinophils. The patient was diagnosed with polyangiitis overlap syndrome and successfully treated with cyclophosphamide. Recognizing this entity is fundamental given the distinct clinical phenotype and outcomes to therapy in the complex scenario of ANCA-associated vasculitides.

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An overlap of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis

Cited by 4 publications

References 2 publications

A Rare Presentation of Polyangiitis Overlapping Syndrome

A Rare Presentation of Polyangiitis Overlapping Syndrome

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

Polyangiitis overlap syndrome: a rare clinical entity

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