IgG4-Related Disease: Pathological Features and Differential Diagnosis



The broad recognition of IgG4-related disease has led to a consensus statement regarding its pathology. This widely accepted multi-organ condition must be diagnosed based on morphological features and immunohistochemical findings. The changes that occur are basically similar in any organ manifestation, with several site-specific findings being recognized. This newly formed systemic condition has a broad range of differential diagnoses, including multicentric Castleman’s disease, granulomatosis with polyangiitis, and malignant lymphoma, for which tissue examinations play a critical role. Since the demand to diagnose this condition by biopsy is increasing, more knowledge and experience need to be accumulated on this approach. Two aspects need to be considered when collecting biopsy samples from patients with suspected IgG4-related disease; one is to obtain additional evidence to support the diagnosis, while the other is to exclude the possibility of this condition. Unlike other inflammatory conditions, which are difficult to rule out using small tissue samples, IgG4-related disease may be excluded on the basis of biopsy findings because there are known histological findings (e.g., necrosis, granuloma, and abscess) that are unlikely to be found in this condition and strongly suggest other diagnoses. Although follow-up biopsies are currently not required, on-treatment biopsies may contribute to a clinical decision being reached, particularly in terms of steroid withdrawal.


IgG4 Pathology Sialadenitis Pancreatitis Cholangitis 


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Copyright information

© Springer Japan 2016

Authors and Affiliations

  1. 1.Department of Diagnostic PathologyKobe University Graduate School of MedicineKobeJapan

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