49 Citations (Scopus)

Abstract

Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized by both elevated serum IgG4 (135mg/dL) and histopathological features, including a dense lymphoplasmacytic infiltrate rich in IgG4 + plasma cells (IgG4 +/IgG + plasma cell ratio 40%). However, patients with hyper-interleukin (IL-) 6 syndromes such as multicentric Castlemans disease, rheumatoid arthritis, and other immune-mediated conditions frequently show lymph node involvement and often fulfill the diagnostic criteria for IgG4-RD. Owing to these factors, IgG4-RD cannot be differentiated from hyper-IL-6 syndromes on the basis of histological findings alone. Laboratory analyses are crucial to differentiate between the 2 diseases. Hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia. In contrast, IgG4-RD does not share any of these characteristics. Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

Original languageEnglish
Article number572539
JournalInternational Journal of Rheumatology
Volume2012
DOIs
Publication statusPublished - 2012

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Immunoglobulin G
Immunoglobulins
Interleukin-6
Plasma Cells
Lymph Nodes
Thrombocytosis
Hypoalbuminemia
Lymphoproliferative Disorders
Lymphadenopathy
Serum
C-Reactive Protein
Immunoglobulin A
Immunoglobulin M
Anemia
Lymphoma
Rheumatoid Arthritis
Biopsy

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

IgG4-related lymphadenopathy. / Sato, Yasuharu; Yoshino, Tadashi.

In: International Journal of Rheumatology, Vol. 2012, 572539, 2012.

Research output: Contribution to journalArticle

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AB - Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized by both elevated serum IgG4 (135mg/dL) and histopathological features, including a dense lymphoplasmacytic infiltrate rich in IgG4 + plasma cells (IgG4 +/IgG + plasma cell ratio 40%). However, patients with hyper-interleukin (IL-) 6 syndromes such as multicentric Castlemans disease, rheumatoid arthritis, and other immune-mediated conditions frequently show lymph node involvement and often fulfill the diagnostic criteria for IgG4-RD. Owing to these factors, IgG4-RD cannot be differentiated from hyper-IL-6 syndromes on the basis of histological findings alone. Laboratory analyses are crucial to differentiate between the 2 diseases. Hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia. In contrast, IgG4-RD does not share any of these characteristics. Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

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