J Korean Med Sci.  2014 Apr;29(4):599-603. 10.3346/jkms.2014.29.4.599.

A Case of IgG4-Related Disease with Bronchial Asthma and Chronic Rhinosinusitis in Korea

• Lee YS
• Cho HJ
• Yoo HS
• Shin YS
• Park HS

• KMID: 1774469
• DOI: http://doi.org/10.3346/jkms.2014.29.4.599

Abstract

IgG4-related disease (IgG4-RD) is characterized by a systemic involvement of tumor-like lesions with IgG4-positive plasmacytes. We experienced a case of IgG4-RD developed in a patient with bronchial asthma (BA) and chronic rhinosinusitis (CRS). A 55-yr-old female patient with BA and CRS complained of both eyes and neck swelling as well as a recurrent upper respiratory infection in recent 1 yr. The serum levels of IgG4, creatinine, and pancreatic enzymes were elevated. A biopsy of the submandibular gland showed an abundant infiltration of IgG4-positive plasmacytes. Her symptoms remarkably improved after the treatment of a systemic steroid that has been maintained without recurrence. We report a rare case of IgG4-RD developed in a patient with BA and CRS.

Keyword

IgG4-Related Disease; Asthma; Bronchitis, Chronic

MeSH Terms

Asthma/complications/*diagnosis
Chronic Disease
Creatinine/blood
Female
Humans
Immunoglobulin G/*blood
Middle Aged
Pancreas/enzymology
Plasma Cells/physiology
Prednisolone/therapeutic use
Republic of Korea
Rhinitis/complications/*diagnosis/drug therapy
Sinusitis/complications/*diagnosis/drug therapy
Submandibular Gland/pathology
Tomography, X-Ray Computed
Creatinine
Immunoglobulin G
Prednisolone

Figure

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  Fig. 1 Clinical manifestations of IgG4-related disease in the patient. (A) Facial photograph of the patient shows bilateral periorbital swelling. (B) Facial photograph of the patient shows bilateral enlargement of the submandibular glands and neck.

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  Fig. 2 Computed tomography (CT) images of the patient. (A) Chest CT image shows bilateral hilar lymphadenopathy (arrowheads). (B) Chest CT image shows peribronchovascular bundle thickening, centrilobular opacities and interlobular septal thickening.

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  Fig. 3 Computed tomography (CT) images of the patient. (A) Neck CT image shows diffuse enlargement of the sublingual (arrows) and submandibular (arrowheads) glands. (B) Abdominal CT image shows multifocal strictures and dilatations of the pancreatic duct in the pancreas body (arrows) and multifocal low attenuation of both kidneys (arrowheads).

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  Fig. 4 Histopathological features in a biopsy of the submandibular gland of the patient. The tissue specimen from the right submandibular gland shows dense lymphoplasma cell infiltration and typical storiform fibrosis (arrowheads) (A, H&E, ×20) and IgG4-positive plasmacytes (B, immunohistostain, ×40).

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