Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Mar;43(3):537-543.
doi: 10.1007/s00296-023-05281-x. Epub 2023 Jan 31.

Polyangiitis overlap syndrome: a rare clinical entity

Lorenza Bruno  1 , Martina Mandarano  2 , Guido Bellezza  2 , Angelo Sidoni  2 , Roberto Gerli  1 , Elena Bartoloni  1 , Carlo Perricone  3
Affiliations
Review

Polyangiitis overlap syndrome: a rare clinical entity

Lorenza Bruno et al. Rheumatol Int. 2023 Mar.

Abstract

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.

Keywords: ANCA; Anti-PR3; EGPA; Eosinophilia; Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Vasculitis.

PubMed Disclaimer

Conflict of interest statement

No funds, grants, or other support was received. The authors have no relevant financial or non-financial interests to disclose. Patient signed informed consent regarding publishing her data and photographs. All authors contributed to the study conception and design. All authors read and approved the final manuscript.

Figures

Fig. 1
Fig. 1
The patient presenting with saddle nose
Fig. 2
Fig. 2
Microscopic view of nasal mucosa replaced by chronic inflammatory infiltrate with microabscesses and some eosinophils (arrow heads). A: February 2022, B: March 2022, Haematoxylin and Eosin stain, Original magnification: 200X
Fig. 3
Fig. 3
Microscopic view of lung parenchyma with altered architecture (A) due to the presence of necrotizing granulomatous inflammation (B), and areas of fibrosis (A, asterisks). Necrotizing vasculitis coexists (C), the inflammatory infiltrate which mainly consists of neutrophilic granulocytes, with eosinophils (arrows). Haematoxylin and eosin stain, original magnification: (A) 16X, (B) and (C) 200X
Fig. 4
Fig. 4
Flow-chart summarizing the main findings in this case
See this image and copyright information in PMC

References

    1. Leavitt RY, Fauci AS. Polyangiitis overlap syndrome. Classification and prospective clinical experience. Am J Med. 1986;81(1):79–85. doi: 10.1016/0002-9343(86)90186-5. PMID: 2873744 - PubMed
    1. Rasheed N, Ahuja R, Borneo H. Polyangiitis overlap syndrome: a novel presentation of microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. BMJ Case Rep. 2021;14(2):e239031. doi: 10.1136/bcr-2020-239031. PMID: 33526532; PMCID: PMC7852911 - PMC - PubMed
    1. Shibata A, Kondo T, Kurasawa T, Chino K, Okada Y, Amano K. A case of polyangiitis overlap syndrome of giant cell arteritis and granulomatosis with polyangiitis successfully treated with rituximab. Mod Rheumatol Case Rep. 2021;5(2):317–321. doi: 10.1080/24725625.2020.1780003. - DOI - PubMed
    1. Rosenberg CE, Khoury P. Approach to Eosinophilia Presenting With Pulmonary Symptoms. Chest. 2021;159(2):507–516. 10.1016/j.chest.2020.09.247. Epub 2020 Sep 28. PMID: 33002503; PMCID: PMC8039005 - PMC - PubMed
    1. Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022;81(3):309–314. 10.1136/annrheumdis-2021-221794. Epub 2022 Feb 2. PMID: 35110334 - PubMed

MeSH terms