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IgG4-Related Lymphadenopathy.

Sato Y, Yoshino T - Int J Rheumatol (2012)

Bottom Line: Laboratory analyses are crucial to differentiate between the 2 diseases.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.

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 (>135 mg/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 Castleman's 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.

No MeSH data available.


Related in: MedlinePlus

Rheumatic lymphadenopathy with abundant IgG4+ cells. (a), (b) The lymph node shows marked follicular hyperplasia and interfollicular plasmacytosis with small lymphocytes; eosinophil infiltration is absent. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >60%).
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fig7: Rheumatic lymphadenopathy with abundant IgG4+ cells. (a), (b) The lymph node shows marked follicular hyperplasia and interfollicular plasmacytosis with small lymphocytes; eosinophil infiltration is absent. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >60%).

Mentions: Hyper-IL-6 syndromes such as MCD, RA, and other immune-mediated conditions are characterized by elevated serum IL-6 levels [16, 17]. Moreover, IL-6 itself functions to raise the serum levels of IgG4 and other IgG subclasses [18, 19]. In fact, MCD, RA, and other immune-mediated conditions sometimes fulfill the histological diagnostic criteria for IgG4-RD (Figures 6 and 7) and are characterized by elevated serum IgG4 levels [8, 20–23]. This complicates diagnosis, owing to the fact that hyper-IL-6 syndromes frequently involve lymph nodes. Because of this, laboratory analyses are crucial to differentiate between the 2 diseases [8]. Unlike IgG4-RD, hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia (Table 2). These abnormalities are closely related to high IL-6 levels [8, 17, 20]. On the other hand, elevated serum IgE is often typical of IgG4-RD [1, 3, 5]. However, IL-6 plays a critical role in IL-4-driven IgE synthesis [24]. As such, hyper-IL-6 syndromes may also be characterized by elevated serum IgE levels, rendering serum IgE level less useful as a biomarker for a differential diagnosis of the 2 diseases [8, 22].


IgG4-Related Lymphadenopathy.

Sato Y, Yoshino T - Int J Rheumatol (2012)

Rheumatic lymphadenopathy with abundant IgG4+ cells. (a), (b) The lymph node shows marked follicular hyperplasia and interfollicular plasmacytosis with small lymphocytes; eosinophil infiltration is absent. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >60%).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3376648&req=5

fig7: Rheumatic lymphadenopathy with abundant IgG4+ cells. (a), (b) The lymph node shows marked follicular hyperplasia and interfollicular plasmacytosis with small lymphocytes; eosinophil infiltration is absent. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >60%).
Mentions: Hyper-IL-6 syndromes such as MCD, RA, and other immune-mediated conditions are characterized by elevated serum IL-6 levels [16, 17]. Moreover, IL-6 itself functions to raise the serum levels of IgG4 and other IgG subclasses [18, 19]. In fact, MCD, RA, and other immune-mediated conditions sometimes fulfill the histological diagnostic criteria for IgG4-RD (Figures 6 and 7) and are characterized by elevated serum IgG4 levels [8, 20–23]. This complicates diagnosis, owing to the fact that hyper-IL-6 syndromes frequently involve lymph nodes. Because of this, laboratory analyses are crucial to differentiate between the 2 diseases [8]. Unlike IgG4-RD, hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia (Table 2). These abnormalities are closely related to high IL-6 levels [8, 17, 20]. On the other hand, elevated serum IgE is often typical of IgG4-RD [1, 3, 5]. However, IL-6 plays a critical role in IL-4-driven IgE synthesis [24]. As such, hyper-IL-6 syndromes may also be characterized by elevated serum IgE levels, rendering serum IgE level less useful as a biomarker for a differential diagnosis of the 2 diseases [8, 22].

Bottom Line: Laboratory analyses are crucial to differentiate between the 2 diseases.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan.

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 (>135 mg/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 Castleman's 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.

No MeSH data available.


Related in: MedlinePlus