Primary Sjögren’s syndrome is a chronic autoimmune disorder of unknown etiology and is characterized by progressive focal lymphocytic infiltration of the lacrimal and salivary glands. Comparison of B cell subsets from the peripheral blood and salivary glands of patients with primary Sjögren’s syndrome and those from healthy individuals shows dysregulation and derangement of B cell subsets in both peripheral circulation and in inflamed glandular tissues. This dysregulation is expressed as a decrease in the percentage of CD27+ memory B cells in peripheral blood and an increase in the CD27+ memory B cells in the affected glands. Further, the overall percentage of long-lived autoantibodies-producing plasma cells within the affected glands is increased. In the last two decades, several studies have shown growing evidences that B cells play multiple roles in primary Sjögren’s syndrome pathophysiology, and that dysregulation of these cells may actually play a central role in the disease development.

Background/purpose Hyperparathyroidism-jaw tumor syndrome is a rare autosomal dominant disease characterized by parathyroid tumors and ossifying fibroma of the jaw. Disease-causing mutations have been localized in the tumor suppressor gene CDC73. This study is designed to highlight the importance of genetic testing in the diagnosis of ossifying fibroma related to this syndrome. Materials and methods The Clinical, histopathological, radiographical, familial and genetic features of 24 patients with Hyperparathyroidism-jaw tumor syndrome were collected by searching the electronic literature PubMed, Medline, Embase, and Science Direct databases combining the MeSH heading terms “Hyperparathyroidism jaw tumor syndrome”, with the words “Ossifying fibroma” and “CDC73”. The collected features were simply assessed and analyzed. Results The average age was 28.68 years old (age range 10–66), with 12 male and 12 female patients (1:1 M/F ratio). Hyperparathyroidism results from parathyroid adenoma in 16/24 cases (66.666%) and parathyroid carcinome in 5/24 (20.833%). Bone pathology occurred most often in the mandible (16/24 cases; 66.666%), while 5/24 cases were in the maxilla (20.883%) and 3 cases in both (12.5%). In 5/24 cases, ossifying fibroma was the first to occur before hypercalcemia. Genetic mutation of CDC73 were positive in 19/24 cases (79.166%). Conclusion Since the jaw lesions in Hyperparathyroidism-jaw tumor syndrome could proceed the cardinal signs of hyperparathyroidism, its accurate diagnosis needs to depend on clinical, histological, radiographical, family history and most of all the genetic testing for CDC73 gene.

Parathyroid glands secrete the parathyroid hormone that plays an essential role in bone remodeling. Excessive production of parathyroid hormone causes a common metabolic bone disorder known as hyperparathyroidism that is classified into primary, secondary, or tertiary. In hyperparathyroidism, the late bony complication is manifested as a giant cell osteolytic lesion called “brown tumor.” Primary hyperparathyroidism is usually a sporadic disorder, but in minority of cases it occurs in inherited forms, and one of these forms is the hyperparathyroidism-jaw tumor syndrome, which is characterized by primary hyperparathyroidism and ossifying fibroma in the mandible and/or maxilla.

IgG4-related disease is a multiorgan immunological fibroinflammatory disorder characterized by lymphoplasmacytic infiltration and fibrosis in multiple organs accompanied by high serum IgG4 levels. The salivary glands are the most common organs involved in this disease. Recently, chronic sclerosing sialadenitis affecting salivary glands, formerly known as Küttner’s tumor, and Mikulicz’s disease have been classified as a class of IgG4-related diseases. The etiopathobiology of IgG4-related disease is not fully understood. It has recently been hypothesized that the inflammatory and fibrotic process and the increased serum IgG4+ levels in IgG4-related disease are the result of an interaction between B cells and T helper cells, suggesting that T cells may play a key role in the pathogenesis of this disease. The aim of this review is to discuss the proposed roles of different T cell subsets in the pathogenesis of IgG4-related disease focusing on their roles in immunopathogenesis of IgG4-related sialadenitis.