A comparative study of Kimura’s disease and IgG4‐related disease: similarities, differences and overlapping features

Abstract

A comparative study of Kimura's disease and IgG4-related disease: similarities, differences and overlapping features Aims: To compare the fibroinflammatory diseases Kimura's disease (KD) and immunoglobulin (Ig)G4related disease (IgG4RD) and to explore their possible relationship. Methods and results: Forty-six cases of KD and 29 IgG4RD from our institution diagnosed from 2011 to 2020 were studied. They were compared with each other on clinical, pathological and immunohistological features. There were similar clinical features, except that IgG4RD affected an older patient population, with more frequent salivary gland involvement and KD affected head and neck lymph nodes, and showed blood eosinophilia more frequently than IgG4RD. IgG4RD exhibited frequent storiform fibrosis and obliterative phlebitis, while KD showed more frequent tissue eosinophilia, eosinophilic abscess, germinal centre eosinophilic deposit and vascularisation. Twenty to 30% of KD had more than 50 IgG4 + plasma cells (PC) per high-power field (HPF) and IgG4/IgG + PC ratio exceeding 40%. These parameters, however, occurred in 100% of IgG4RD. Significantly more KD had >10 IgE + PC/HPF and lymphoid germinal centre IgE reticular staining compared to IgG4RD. All these histological and immunohistological features are overlapping in the two diseases, although they differed with statistical significance. Conclusion: Our study confirmed that there is significant overlap in clinical, pathological and immunohistological features between KD and IgG4RD. It is important to recognise these overlapping features, and correlation with a clinicopathological picture is required in differential diagnoses. The overlapping features also suggest a possible close relationship between KD and IgG4RD, which could represent different facets of a continuous fibroinflammatory disease spectrum.

Successful treatment of dupilumab in Kimura disease independent of IgE: A case report with literature review

“…A Th2 cell immune response from the very beginning was suggested due to its impressive characteristics of elevated serum IgE, peripheral blood eosinophilia, and infiltration of eosinophils in tissues. Recent findings of its correlation to IgG4related diseases also enhance this viewpoint (10)(11)(12). Ohta et al…”

A young woman with facial erythematous induration and eyelid swelling

“…In recent years, an increasing number of cases have been reported of KD with features of IgG4-related disease (IgG4-RD), as well as IgG4-RD with features of KD. Studies have shown a significant overlap in clinical, pathologic, and immunohistological features between KD and IgG4-RD, with the latter, frequently occurring in the elderly, characterized by obliterative phlebitis, storiform fibrosis, and IgG4-positive cells [8]. With only limited data are currently available, it is difficult to distinguish KD from IgG4-RD, and the relationship between them needs further study.…”

Kimura’s disease: A clinicopathological study of 23 cases

“…T cell-derived IL-4 and IL-13 are separately known to induce B cell synthesis of IgE and IgG4 (19,20). Infiltration of IgG4 + plasma cells has been variably reported in previous KD case series (5,10,21,22). It has been postulated that as KD lesions progress to chronic stage, Th2 inflammation would be suppressed with resolution of type I hypersensitivity, resulting in an apparent increased proportion of IgG4 compared with IgE (22).…”

“…However, high endothelial venules are atypical. The replacement of normal germinal centers with IgE or eosinophilic deposits, necrosis, and polykaryocytes (Warthin-Finkeldey-type giant cells) can be present (4,10). In some patients, inflammatory infiltration of nerve fibers results in skin irritation and pruritus (11).…”