IgG4-Related Disease of the Oral Cavity. Case Series from a Large Single-Center Cohort of Italian Patients
Andrea Rampi,
Marco Lanzillotta,
Gaia Mancuso,
Alessandro Vinciguerra and
Lorenzo Dagna
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Andrea Rampi: Otorhinolaryngology Unit, Division of Head and Neck Department, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy
Marco Lanzillotta: School of Medicine, Vita-Salute San Raffaele University, 20132 Milan, Italy
Gaia Mancuso: School of Medicine, Vita-Salute San Raffaele University, 20132 Milan, Italy
Alessandro Vinciguerra: Otorhinolaryngology Unit, Division of Head and Neck Department, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy
Lorenzo Dagna: School of Medicine, Vita-Salute San Raffaele University, 20132 Milan, Italy
IJERPH, 2020, vol. 17, issue 21, 1-11
Abstract:
A series of destructive and tumefactive lesions of the oral cavity are increasingly recognized as part of the IgG4-related disease (IgG4-RD) spectrum. We herein examined the clinical, serological, radiological, and histological features of a series of patients referred to our clinic because of oral cavity lesions ultimately attributed to IgG4-RD. In particular, we studied 6 consecutive patients out of 200 patients referred to the immunology outpatient unit who presented with erosive and/or tumefactive lesions of the oral cavity. All patients underwent serum IgG4 measurement, nasal endoscopy, radiological studies, and histological evaluation of tissue specimens. The histological studies included immunostaining studies to assess the number of IgG4+ plasma cells/High-Power Field (HPF) for calculation of the IgG4+/IgG+ plasma cell ratio. Six patients (3% of the entire cohort) were diagnosed with IgG4-RD of the oral cavity based on histological evaluation. A major complaint at presentation was oral discomfort due to bulging mass. A mild to no increase in serum IgG4 was observed. Different patterns of organ involvement were associated with oral lesions. Five patients were treated with immunosuppressive therapy and two patients promptly responded to B-cell depletion with rituximab. Watchful waiting was decided in one patient with no major clinical symptoms. Involvement of the oral cavity is an infrequent manifestation of IgG4-RD but should be taken into consideration as a possible differential diagnosis of tumefactive or erosive lesions once neoplastic conditions are excluded. A histological examination of biopsy samples from the oral cavity represents the mainstay for diagnosis of IgG4-RD.
Keywords: IgG4-related disease; oral lesions; differential diagnosis; histology; biopsy (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2020
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Persistent link: https://EconPapers.repec.org/RePEc:gam:jijerp:v:17:y:2020:i:21:p:8179-:d:440480
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