Findings that shed new light on the possible pathogenesis of a disease or an adverse effect: A new category for chronic sclerosing sialadenitis as an IgG4 related syndrome

doi:10.1136/bcr.10.2009.2412

BMJ Case Rep. 2010; 2010: bcr10.2009.2412.

Published online 2010 March 26. doi: 10.1136/bcr.10.2009.2412

PMCID: PMC3028012

Findings that shed new light on the possible pathogenesis of a disease or an adverse effect

A new category for chronic sclerosing sialadenitis as an IgG4 related syndrome

Kazuki Nagai,¹ Kazuo Andoh,² Akira Ogata,² Noriaki Aoki,³ Noriko Nakamura,⁴ Hiroo Hosaka,⁵ and Rika Kurihara⁶

¹Nagai Clinic, Internal Medicine, 1-7-25, Yokodi, Isogo-ku,, Yokohama City, Kanagawa, 2350045, Japan

²Saiseikai Yokohama City Nanbu Hospital, Radiology, 3-2-10, Konandai, Konan-ku, Yokohama City, Kanagawa, 233-8503, Japan

³Saiseikai Yokohamashi Nanbu Hospital, Oral and Maxillofacial Surgery, 3-2-10, Konandai, Konan-ku, Yokohama City, 233-8503, Japan

⁴Saiseikai Yokohamashi Nanbu Hospital, Pathology, 3-2-10, Konandai, Konan-ku, Yokohama City, 233-8503, Japan

⁵Saiseikai Yokohamashi Nanbu Hospital, Internal Medicine, 3-2-10 Kohnandai, kohnan-ku, Yokohama, 234-8503, Japan

⁶Kanagawa Dental College, Otolaryngology, 82, Inaokacho, Yokosuka, 238-8580, Japan

Correspondence to Kazuki Nagai, Email: k-nagai/at/mtj.biglobe.ne.jp

Abstract

This report presents three cases of IgG4 related sclerosing disease complicated with chronic sclerosing sialadenitis. The clinical, radiographic and pathologic findings later suggested that the patients had chronic sclerosing sialadenitis. The submandibular gland tumour regressed in two patients. These patients were suspected to have Mikulicz’s disease or Sjögren’s syndrome, but the diagnostic criteria were not satisfied. The workup for chronic sclerosing sialadenitis revealed a significantly elevated value of serum IgG4. The patient was then diagnosed with IgG4 related sclerosing disease complicated with chronic sclerosing sialadenitis. After administering prednisolone to these patients, the serum IgG4 values decreased after 4 weeks. The current cases may represent a new category for Mikulicz’s disease or Sjögren’s syndrome as an IgG4 related sclerosing disease.

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