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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

IgG4-related lymphadenopathy (type III). (a) The lymph node shows interfollicular expansion with normal to small germinal centers. (b) Hypervascular proliferation is seen in the interfollicular zone. (c) A mixed infiltrate of small lymphocytes, immunoblasts, immature plasma cells, mature plasma cells, and scattered eosinophils is observed (d) Numerous IgG4+ cells are present in the interfollicular zone.
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fig3: IgG4-related lymphadenopathy (type III). (a) The lymph node shows interfollicular expansion with normal to small germinal centers. (b) Hypervascular proliferation is seen in the interfollicular zone. (c) A mixed infiltrate of small lymphocytes, immunoblasts, immature plasma cells, mature plasma cells, and scattered eosinophils is observed (d) Numerous IgG4+ cells are present in the interfollicular zone.

Mentions: This type is also frequently characterized by systemic lymphadenopathy [1–3]. Histologically, the lymph nodes show marked interfollicular expansion with prominent high endothelial venules and patent sinuses. The lymphoid follicles are usually normal to atrophic. A mixed infiltrate of small lymphocytes, immunoblasts, immature plasma cells, mature plasma cells, and scattered eosinophils is observed (Figure 3). The morphological features overlap with those of atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP), which is a characteristic lymphadenopathy observed in patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and other autoimmune diseases [9].


IgG4-Related Lymphadenopathy.

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

IgG4-related lymphadenopathy (type III). (a) The lymph node shows interfollicular expansion with normal to small germinal centers. (b) Hypervascular proliferation is seen in the interfollicular zone. (c) A mixed infiltrate of small lymphocytes, immunoblasts, immature plasma cells, mature plasma cells, and scattered eosinophils is observed (d) Numerous IgG4+ cells are present in the interfollicular zone.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig3: IgG4-related lymphadenopathy (type III). (a) The lymph node shows interfollicular expansion with normal to small germinal centers. (b) Hypervascular proliferation is seen in the interfollicular zone. (c) A mixed infiltrate of small lymphocytes, immunoblasts, immature plasma cells, mature plasma cells, and scattered eosinophils is observed (d) Numerous IgG4+ cells are present in the interfollicular zone.
Mentions: This type is also frequently characterized by systemic lymphadenopathy [1–3]. Histologically, the lymph nodes show marked interfollicular expansion with prominent high endothelial venules and patent sinuses. The lymphoid follicles are usually normal to atrophic. A mixed infiltrate of small lymphocytes, immunoblasts, immature plasma cells, mature plasma cells, and scattered eosinophils is observed (Figure 3). The morphological features overlap with those of atypical lymphoplasmacytic and immunoblastic proliferation (ALPIBP), which is a characteristic lymphadenopathy observed in patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and other autoimmune diseases [9].

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