Kosin Med J.  2017 Dec;32(2):233-239. 10.7180/kmj.2017.32.2.233.

IgG4-Related Disease with lymphadenopathy Presenting as a Cervical lymph node enlargement

• Choi YS
• Kim S
• Sohn JS
• Hwang J
• Chang T
• Cho DY

• KMID: 2400267
• DOI: http://doi.org/10.7180/kmj.2017.32.2.233

Abstract

During the course of evaluation and management of neck masses, consideration for Immunoglobulin G4-related disease (IgG4-RD) should be given. IgG4-RD is relatively a new growing entity of immune-mediated origin, characterized by a mass-forming lesion, the infiltration of IgG4-positive plasma cells and occasionally elevated serum IgG4. The most common manifestations are parotid and lacrimal swelling, lymphadenopathy and autoimmune pancreatitis. A previously healthy 72-year-old man was referred to our clinic with a 2-month history of left cervical lymph node enlargement without systemic manifestations . A cervical lymph node biopsy was planned because of elevated serum IgG4 levels. Pathological findings showed prominent infiltration of IgG4-postive plasma cells in the lymph node. After steroid therapy, a computed tomography scan revealed a decrease in the cervical lymph node size. This case illustrates the importance of including IgG4-RD in the differential diagnosis of a cervical lymph node enlargement.

Keyword

Immunoglobulin G4; Lymphadeopathy

MeSH Terms

Aged
Biopsy
Diagnosis, Differential
Humans
Immunoglobulin G
Immunoglobulins
Lymph Nodes*
Lymphatic Diseases*
Neck
Pancreatitis
Plasma Cells
Immunoglobulin G
Immunoglobulins

Figure

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  Fig. 1 Fig. 1A. Initial neck CT(Computed Tomography) showed multiple cervical lymph node enlargement, Fig. 1B. Post-steroid treatment image (The size of the cervical lymph node decreased after 8 month steroid trial)

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  Fig. 2 Histological findings in Lt. cervical LN biopsy specimen, H&E staining ×200

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  Fig. 3 Histological findings in Lt. cervical LN biopsy specimen, Interfollicular and perisinusoidal infiltration of small and large lymphocytes, admixed atrophic and hyperplastic germinal centers, large numbers of plasma cells showing IgG4 immunoreactivities (>100/HPF), Immunohistochemicalstaining × 200

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