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L.Y.C. Chen et al.
Figure 1. Manifestations of IgG4-related disease by organ system. The most common primary disease features are indicated in bold.
ized (the latter typically contiguous with involved organs such as pancreas and lungs). Parallel enlargement of sali- vary, lacrimal and parotid glands is common. Five mor- phological subtypes, all of which display increased IgG4+ plasma cells, have been described (see Figure 2 for exam- ples):18-20
(i) Multi-centric Castleman disease-like: preserved nodal architecture with patent sinusoids and hyperplastic folli- cles; abundant mature plasma cells in interfollicular areas with some eosinophils, similar to lymphadenopathy in multicentric Castleman disease or autoimmune disease.
(ii) Reactive follicular hyperplasia: increased IgG4+ plas- ma cells in germinal centers and often in the interfollicular zones with some eosinophils present.
(iii) Interfollicular expansion pattern: expanded interfol- licular zones with small lymphocytes, plasmablasts and mature plasma cells and eosinophils which may resemble lymphoma (e.g. angioimmunoblastic lymphoma). Examples are given in Figure 2A-C.
Table 1. Clinical characteristics of patients from two large published cohorts.
(iv) Progressive transformation of germinal center-like:
Mean age at diagnosis
Male sex
Ethnicity
Elevated serum IgG4
Mean number of organs involved (range)
Affected organs Salivary glands
Lacrimal glands/orbit Lymph nodes Pancreas Retroperitoneal/ aorta
Kidney
Lung
Japan (n=334)17
63.8 years
61.4% 100% Japanese >95%
3.2 (1-11)
72.3%
57.1% 56.5% 25.5 24.9
23.7%
23.4%
Boston (125)16
55.2 years
60.8% 76% White 51% 2.3 (1-7)
28% (submandibular) + 16.8% (parotid) 22.4%
27.2%
19.2%
18.4% (retroperitoneal) + 11.2% (aorta) 12%
17.6%
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