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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 V). This is a regional lymph node with IgG4-related cholangitis. (a) The majority of the lymph node is replaced by hyalinized fibrous tissue. (b) Mature plasma cells infiltrate the hyalinized fibrous tissue. (c) The mature plasma cells are IgG4+.
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fig5: IgG4-related lymphadenopathy (type V). This is a regional lymph node with IgG4-related cholangitis. (a) The majority of the lymph node is replaced by hyalinized fibrous tissue. (b) Mature plasma cells infiltrate the hyalinized fibrous tissue. (c) The mature plasma cells are IgG4+.

Mentions: In this type, the lymph nodes show asymptomatic localized lymphadenopathy [13]. Histologically, most of the lymph node is occupied by hyalinized fibrous tissue. A few residual lymphoid follicles with hyperplastic germinal centers and a focally dense lymphoid infiltrate are observed in the lymph node. Small lymphocytes, plasma cells, and eosinophils infiltrate the dense sclerotic tissue (Figure 5). This type is rare; we have encountered only 2 cases, and no other cases have been reported thus far [1, 13].


IgG4-Related Lymphadenopathy.

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

IgG4-related lymphadenopathy (type V). This is a regional lymph node with IgG4-related cholangitis. (a) The majority of the lymph node is replaced by hyalinized fibrous tissue. (b) Mature plasma cells infiltrate the hyalinized fibrous tissue. (c) The mature plasma cells are IgG4+.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig5: IgG4-related lymphadenopathy (type V). This is a regional lymph node with IgG4-related cholangitis. (a) The majority of the lymph node is replaced by hyalinized fibrous tissue. (b) Mature plasma cells infiltrate the hyalinized fibrous tissue. (c) The mature plasma cells are IgG4+.
Mentions: In this type, the lymph nodes show asymptomatic localized lymphadenopathy [13]. Histologically, most of the lymph node is occupied by hyalinized fibrous tissue. A few residual lymphoid follicles with hyperplastic germinal centers and a focally dense lymphoid infiltrate are observed in the lymph node. Small lymphocytes, plasma cells, and eosinophils infiltrate the dense sclerotic tissue (Figure 5). This type is rare; we have encountered only 2 cases, and no other cases have been reported thus far [1, 13].

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