Myeloperoxidase-Antineutrophil Cytoplasmic Antibody–Positive Otitis Media and Rhinosinusitis With Pathological Features of Immunoglobulin G4-Related Disease

Abstract

Background:

Immunoglobulin G4-related disease (IgG4-RD) and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have different clinical and pathological features. However, differentiation between these 2 disorders is sometimes difficult.

Objective:

To report a case involving a patient with characteristics of both IgG4-RD and AAV.

Methods:

Case report with literature review.

Results:

We report a case of myeloperoxidase-ANCA–positive otitis media and rhinosinusitis with pathological features of IgG4-RD in a 73-year-old man. The patient was first clinically suspected to have granulomatosis with polyangiitis. All of the main characteristic pathological features of IgG4-RD were present: dense lymphoplasmacytic infiltration, increased numbers of IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis.

Conclusions:

The simultaneous presence of the characteristics of both IgG4-RD and AAV makes diagnosis and treatment difficult.

Keywords

IgG4 ANCA IgG4-related disease ANCA-associated vasculitis fibrosis nasal manifestation

Get full access to this article

View all access options for this article.

References

Umehara

Okazaki

Masaki

Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012;22(1):21-30.

Jennette

Falk

Andrassy

Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187-192.

Yoshida

Iino

. Pathogenesis and diagnosis of otitis media with ANCA-associated vasculitis. Allergol Int. 2014;63(4):523-532.

Hoffman

Kerr

Leavitt

Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488-498.

Brouwer

Tervaert

Horst

Predominance of IgG1 and IgG4 subclasses of anti-neutrophil cytoplasmic autoantibodies (ANCA) in patients with Wegener’s granulomatosis and clinically related disorders. Clin Exp Immunol. 1991;83(3):379-386.

Yamamoto

Takahashi

Suzuki

Analysis of serum IgG subclasses in Churg-Strauss syndrome—the meaning of elevated serum levels of IgG4. Intern Med. 2010;49(14):1365-1370.

Deshpande

Zen

Chan

Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181-1192.

Strehl

Hartmann

Agaimy

. Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol. 2011;64(3):237-243.

Kamisawa

Okazaki

Kawa

Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 III. Treatment and prognosis of autoimmune pancreatitis. J Gastroenterol. 2014;49(6):961-970.

10.

Ohno

Matsuda

Arai

Kimura

. Nasal manifestations of IgG4-related disease: a report of two cases. Auris Nasus Larynx. 2015;42(6):483-487.

11.

Ikeda

Awataguchi

Shoji

Oshima

. A case of paranasal sinus lesions in IgG4-related sclerosing disease. Otolaryngol Head Neck Surg. 2010;142(3):458-459.

12.

Moteki

Yasuo

Hamano

Uehara

Usami

. IgG4-related chronic rhinosinusitis: a new clinical entity of nasal disease. Acta Otolaryngol. 2011;131(5):518-526.

13.

Suzuki

Nakamaru

Akazawa

Nasal manifestations of immunoglobulin G4-related disease. Laryngoscope. 2013;123(4):829-834.

14.

Takagi

Nakamaru

Fukuda

. Otologic manifestations of immunoglobulin G4-related disease. Ann Otol Rhinol Laryngol. 2014;123(6):420-424.

15.

Cho

Lee

Chung

Koo

. Otologic manifestation in IgG4-related systemic disease. Clin Exp Otorhinolaryngol. 2011;4(1):52-54.

16.

Watts

Lane

Hanslik

Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66(2):222-227.

17.

Sada

Yamamura

Harigai

Classification and characteristics of Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis in a nationwide, prospective, inception cohort study. Arthritis Res Ther. 2014;16(2):R101.

18.

Harabuchi

Kishibe

Komabayashi

. Clinical manifestations of granulomatosis with polyangiitis (Wegener’s granulomatosis) in the upper respiratory tract seen by otolaryngologists in Japan. Clin Exp Nephrol. 2013;17(5):663-666.

19.

Khosroshahi

Wallace

Crowe

International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67(7):1688-1699.

20.

Kihara

Sugihara

Hosoya

Miyasaka

. Clinical significance of complement as a biomarker of disease activity in 4 cases of IgG4-related disease with retroperitoneal fibrosis. Clin Exp Rheumatol. 2013;31(6):947-949.