Differential diagnosis of immunoglobulin G4-related sialadenitis and Kimura’s disease of the salivary gland: a comparative case series

Abstract

The aim of this study was to investigate key points for the differential diagnosis of immunoglobulin G4-related sialadenitis (IgG4-RS) and Kimura’s disease (KD) involving the salivary glands. The clinical, serological, radiological, histological, and immunohistochemical features of 85 IgG4-RS cases and 52 KD cases were evaluated comparatively. Seventy-two IgG4-RS cases had enlargement of multiple salivary and/or lacrimal glands; 67 patients had bilateral submandibular gland (SMG) involvement. Unilateral parotid gland involvement (59.6%) and comorbid skin lesions (61.5%) were common in KD. Serum IgG4 was elevated in 94.1% of IgG4-RS cases versus 19.0% of KD cases (cut-off value=266.5mg/dl). KD was more commonly associated with elevated eosinophil counts (86% vs 23.1%) and elevated IgE concentrations (95.5% vs 76.6%). Storiform fibrosis, irregular lymphoid follicles, and increased IgG4-positive cells (112.9±37.6/high-power field (HPF)) were common in IgG4-RS. Acellular fibrosis, regular lymphoid follicles, IgE-positive reticular networks, increased IgE-positive cells (43.4±26.7/HPF), and tryptase-positive mast cells (29.7±13.3/HPF) were usually detected in KD. Computed tomography showed that 85.7% of KD cases involved subcutaneous fat tissue. A superficial hypoechoic and reticular pattern with multiple hypoechoic foci were the sonographic features of the SMG in IgG4-RS. Despite numerous overlapping manifestations, histopathological examination showed meaningful differences in the types of fibrosis, eosinophils, and IgG4-positive cell counts. Comprehensive evaluation of clinical, serological, radiological, and histopathological features are crucial for the differential diagnosis.