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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

Multicentric Castleman's disease with abundant IgG4+ cells. (a) Atrophic germinal centers and interfollicular expansion are seen. (b) Sheets of proliferating mature plasma cells are present in the interfollicular zone. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >70%).
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fig6: Multicentric Castleman's disease with abundant IgG4+ cells. (a) Atrophic germinal centers and interfollicular expansion are seen. (b) Sheets of proliferating mature plasma cells are present in the interfollicular zone. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >70%).

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)

Multicentric Castleman's disease with abundant IgG4+ cells. (a) Atrophic germinal centers and interfollicular expansion are seen. (b) Sheets of proliferating mature plasma cells are present in the interfollicular zone. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >70%).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig6: Multicentric Castleman's disease with abundant IgG4+ cells. (a) Atrophic germinal centers and interfollicular expansion are seen. (b) Sheets of proliferating mature plasma cells are present in the interfollicular zone. (c) The majority of mature plasma cells are positive for IgG4 (IgG4+/IgG+ plasma cell ratio >70%).
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