Inflammatory pseudotumour of the alveolar process of the maxilla as clinical manifestation of IgG4-related disease: a case report and literature review

Inflammatory pseudotumour of the alveolar process of the maxilla as clinical manifestation of IgG4-related disease: a case report and literature review:



Publication date: Available online 19 December 2019

Source: International Journal of Oral and Maxillofacial Surgery

Author(s): W.L. Kouwenberg, F.J. Dieleman, S.M. Willems, A.J.W.P. Rosenberg

Abstract

IgG4-related disease (IgG4-RD) is an uncommon immune-mediated condition considered to be a systemic disease, described in multiple organ systems. IgG4-RD that involves the maxillary and sinonasal region is rare. This report presents a very rare presentation of IgG4-RD in the maxillary alveolar process. The patient presented with left-sided facial pain, headache, and mobility and loss of teeth. The first biopsy and resection specimen reports were inconclusive and showed a non-specific chronic inflammatory process. After the third resection, the diagnosis was finally established through findings that satisfied the 2012 consensus criteria for IgG4-RD. Consequently high doses of oral corticosteroids and azathioprine were given, tapered over a total period of 36 months. Weaning is still in progress, but no recurrence was observed after 34 months. A review of the English-language literature was performed, which identified seven cases of IgG4-RD with maxillary and sinonasal involvement. Cases were excluded from the review if there was any doubt that they met the consensus statement on the pathology.