Table 1.

The different characteristics of Orbital Lymphoma, EGPA and IgG4-RD with Kimura's disease

Diseases	Kimura's disease	Orbital Lymphoma	EGPA	IgG4-RD
Clinical features	An immune-mediated inflammatory disorder; lymphadenopathy; subcutaneous nodules; painless; swelling in the head and neck is common	A malignant tumor arising as clonal expansions of B-lymphocytes, T-lymphocytes, or NK-cells; Lymphadenopathy; B symptoms (fever, night sweats, or weight loss, etc.)	An ANCA associated vasculitis; eosinophil-rich granulomatous; asthma; recurrent pneumonia; paranasal sinus infections; skin lesions (most commonly purpura); Mono- or poly-neuropathy	A fibroinflammatory condition; diffuse/localized swelling in single or multiple organs; non-specific, and overlap with various inflammatory and neoplastic conditions
Laboratory analysis	Peripheral blood eosinophil and IgE levels ↑↑	Depending on histopathological subtype and the clinical stage; anemia, WBC↑are common	Peripheral blood eosinophilia > 10% total WBC; CRP level is median 2.5 ~ 6.6 mg/dl; 30 ~ 47% of patients ANCA ( +); serum IgE and IgG4 level↑	Serum IgG4 > 135 mg/dl
Histopathological features	Hyperplasia of lymphoid follicular; enlargement of germinal center; infiltration or accumulation of eosinophils, even to form “eosinophilic abscess”; hyperplasia of postcapillary and venular with intermingled fibrotic changes	Effacement of lymph node architecture; rich inflammatory background, eosinophils and EBERs may be positive; immunohistochemical features to differentiate between B- and T-cell	Necrotizing small vasculitis accompanied by eosinophil infiltrates; perivascular and extravascular granulomas; palisading granuloma; fibrinoid necrosis in the wall of the vessels; rupture of internal elastic lamina	IgG4 + plasma cell infiltration; IgG4 + /IgG + of > 0.40, or a total of > 50 IgG4 + plasma cells/ high power field; storiform fibrosis; obliterative phlebitis; increased numbers of eosinophils, without eosinophilic granulomas
Available treatments	Glucocorticoids; immunosuppressants; surgery; local low-dose radiotherapy; anti-IgE monoclonal antibody(omalizumab)	Radiotherapy; chemotherapy; Glucocorticoids; surgery; Monoclonal antibodies (rituximab); Stem cell transplant	Glucocorticoids; immunosuppressants; monoclonal antibody: against interleukin-5 (mepolizumab); anti-CD20 (rituximab); Anti-IgE (omalizumab)	Glucocorticoids; immunosuppressants; anti-CD20 monoclonal antibody (rituximab)
References	[11, 22, 23]	[24, 25]	[26, 27]	[28, 29]

EGPA eosinophilic granulomatosis with polyangiitis, IgG4-RD IgG4-related disease, ANCA antineutrophil cytoplasmic antibody, WBC white blood cells, CRP c-reactive protein, EBERs EBV-encoded RNAs