Kazuya Kurakami

Clinical and pathological characteristics of IgG4-related sclerosing sialadenitis†

A new concept of IgG4‐related sclerosing sialadenitis characterized by high serum IgG4 levels and tissue infiltration of IgG4‐expressing plasmacytes has recently been proposed. To determine appropriate serum levels of IgG4 for monitoring disease activity, a total of 36 serum samples and eight tissue samples from patients with IgG4‐related sclerosing sialadenitis were studied.

Roles of IL-17, Th1, and Tc1 Cells in Patients With IgG4-Related Sclerosing Sialadenitis

Immunoglobulin G4 (IgG4)‐related sclerosing sialadenitis is a recently recognized disease entity characterized by high serum IgG4 concentration and IgG4‐producing plasma cell expansion in affected organs, which show fibrotic or sclerotic changes. However, little is known about the roles of CD4+ and CD8+ T cells or interleukin (IL)‐17 in this disease. The purpose of this study was to evaluate the characteristics of CD4+ and CD8+ T cells and IL‐17 in patients with IgG4‐related sclerosing sialadenitis.

Expressions and Roles of Periostin in Otolaryngological Diseases

Periostin is a 90-kDa member of the fasciclin-containing family; it functions as part of matricellular proteins, and its production by airway epithelial cells is induced by IL-4 and IL-13. Periostin is secreted by fibroblasts and upregulated in the airway epithelia of patients with bronchial asthma; it is considered to contribute to remodeling under this pathological condition. However, despite many studies in diverse research areas, our overall understanding of this intriguing molecule is still inadequate. Here, we integrate the available evidence on periostin expression and its roles in otolaryngological diseases, including allergic rhinitis, chronic rhinosinusitis with nasal polyps, aspirin-induced asthma, organized hematoma, eosinophilic otitis media, and IgG4-related disease. Periostin might be involved as an important structural mediator in pathological processes such as insult and injury, Th2-driven inflammation, extracellular matrix restructuring, fibrosclerosis, tumor angiogenesis, and tissue remodeling.

Roles of TGF-beta and periostin in fibrosclerosis in patients with IgG4-related diseases.

Abstract Conclusion: These results suggest that transforming growth factor (TGF)-beta and periostin could be useful as novel biomarkers and therapeutic targets in IgG4-related disease. Objectives:IgG4-related disease is an uncommon fibrosclerosing and inflammatory mass-forming disease that can be systemic or can affect single organs. To clarify the roles of TGF-beta, periostin, and interleukin (IL)-13 in the pathogenesis of IgG4-related disease, we studied a total of 36 serum and 11 tissue samples from patients with IgG4-related disease. Methods: This was a retrospective clinical study. The patient group consisted of six females and seven males (average age 60 years, range 38–74 years). Serum IgG4 levels, the tissue density of IgG4-positive plasmacytes, and the expression of TGF-beta and periostin in the affected tissues were examined immunohistochemically. Results: Serum IgG4 levels were elevated in all patients (mean 776.6, range 185–2820 mg/dl), and IgG4-positive plasmacytes were observed in the affected salivary glands. Seven patients with prominent infiltration of the involved glands with IgG4-positive plasmacytes had fatal systemic complications, including pancreatitis, after swelling of the salivary glands. Overexpression of TGF-beta and periostin was observed in affected tissues obtained from these patients.

OK-432 treatment of ranula extending to the parapharyngeal space.

Abstract Conclusions: Our results confirmed that OK-432 therapy is simple, easy, safe, and effective and can be used as a substitute for surgery in the treatment of ranula extending to the parapharyngeal space. Objective: The aim of this study was to evaluate the outcome and complications of the OK-432 treatment of patients with ranula extending to the parapharyngeal space. Methods: This was a case series with planned data collection at Yamagata University and Fukase clinic. We tried this therapy in six patients with ranula extending to the parapharyngeal space, between January 2001 and February 2012. We injected OK-432 solution into the lesion with an 18 or 27 gauge needle depending on the patients condition (location and size of ranula and complications). This treatment was performed on an outpatient basis without hospitalization. Results: Disappearance or marked reduction of the lesion were observed in all patients who had this therapy, and local scarring and deformity of the injection sites did not occur in any patients. As side effects, local pain at the injection site and fever (37–39°C) were observed in 40% of the patients who had this therapy, but such problems resolved within a few days.

Aortic aneurysm rupture as a rare complication of granulomatosis with polyangiitis: a case report

IntroductionGranulomatosis with polyangiitis is characterized by systemic inflammation of medium and small blood vessels. Aortic involvement in granulomatosis with polyangiitis is extremely rare. As far as we know this is the first reported case of successful treatment in a patient with granulomatosis with polyangiitis complicated with aortic aneurysm rupture.Case presentationWe describe a case of granulomatosis with polyangiitis in a 38-year-old Japanese man who developed an aortic aneurysm rupture 22 years after disease onset. The patient was operated on and a J-graft was inserted. He recovered uneventfully.ConclusionRecommendations in regard to, and consideration of, aortic involvement should be kept in mind in the long-term careful follow up of granulomatosis with polyangiitis.

Clinical Investigation of Surgical Closure of Tracheoesophageal Shunt

The purpose of this study was to elucidate the appropriate time for closing a tracheoesophageal shunt for a safe and non-invasive surgical procedure, after acquiring another type of vocal rehabilitation. A tracheoesophageal shunt is globally considered to be the most useful tool for excellent vocal rehabilitation; nevertheless, it must be closed for several reasons. In some cases, surgical closure of a tracheoesophageal shunt is difficult due to poor histological conditions around the shunt. We herein propose a new strategy of vocal rehabilitation to utilize a tracheoesophageal shunt effectively. Materials and methods: Between 1995 and 2014, 46 patients underwent voice prosthesis insertion surgery. Nine (eight laryngeal cancer patients, one thyroid cancer patient) of these patients underwent surgical closure of a tracheoesophageal shunt. We investigated their cancer treatments, reasons for closing the tracheoesophageal shunt, period of voice prosthesis insertion, operative method, number of operations, and outcome. Results: The reasons for closing the tracheoesophageal shunt were aspiration pneumonia and acquisition of esophageal voice in 4 patients each. Regarding the period of voice prosthesis speech, 6 patients had used it for approximately 3 years and 3 patients for more than 7 years. Approximately all 3-year users underwent a non-invasive surgical procedure, such as triple-layered suture, and their operation succeeded the first time. Conversely, the more than 7-year users required an invasive surgical procedure, such as a pedicle flap, and had to undergo more than one operation. Conclusion: In the present study, tracheoesophageal shunt closure could be performed within 3 years via a safe and non-invasive surgical procedure. We recommend that the operation for a tracheoesophageal shunt be undertaken at a relatively early stage after total laryngectomy. Such patients should acquire esophageal voice within 3 years and undergo surgical closure of the tracheoesophageal shunt as soon as possible.

Epstein‐Barr virus in the enlarged salivary tissues of patients with IgG4‐related disease

Immunoglobulin G4‐related disease (IgG4‐RD) is a recently recognized disease entity characterized by high‐serum IgG4 concentration and IgG4‐producing plasma cell production with fibrotic or sclerotic changes in affected organs. We aimed to clarify the roles of Epstein‐Barr virus (EBV) in patients with IgG4‐RDs.

Exploring the Limits of the Endoscopic Approach to Frontal Sinus Osteoma

Objective: We present the case of a frontal sinus osteoma in a 71-year-old Japanese woman who presented with an approximately 2-year history of head dullness. Case report: CT showed a 45×39 mm radio dense mass extending from the left to the right frontal sinus. Under general anesthesia the mass was removed by endoscopic surgery. Although the tumor was large and extended into both frontal sinuses, the tumor was completely removed, without CSF leakage or orbital complications, by using a modified Lothrop procedure. Conclusion: A unique osteoma of the frontal sinus is described. The challenges related to surgical treatment of this particular case and similar lesions are addressed.

IgG4-Related Sialadenitis: Pearls and Pitfalls in Management

Objective: A new concept of IgG4-related sialadenitis characterized by high serum IgG4 levels and tissue infiltration of IgG4-expressing plasmacytes has recently been proposed. To determine appropriate serum levels of IgG4 for monitoring disease activity, a total of 36 serum samples and 8 tissue samples from patients with IgG4-related sialadenitis were studied. Method: The patient group consisted of 6 men and 4 women with an average age of 60 years (range, 47 to 74 years). Serum levels of IgG4 and the density of IgG4-positive plasmacytes in affected tissues were studied. Results: All patients had elevated serum IgG4 levels (>135 mg/dL), and IgG4-positive plasmacytes (IgG4+ plasma cells/IgG+ plasma cells >50%) were observed in the involved salivary glands. Six patients with IgG4-related sclerosing sialadenitis with high IgG4/IgG ratios and prominent infiltration of IgG4-positive plasmacytes in the involved salivary glands had systemic complications, including pancreatitis, retroperitoneal fibrosis, and/or inflammatory pseudotumor of the lung after swelling of the salivary glands. All 6 of these patients were successfully treated with systemic corticosteroids. Conclusion: In the 6 patients with systemic complications, treatment with systemic corticosteroids reduced the salivary gland enlargement and lowered serum IgG4 concentrations. These results suggest that IgG4 levels and IgG4/IgG ratios may be used as additional indicators of disease activity and as biomarkers for potential life-threatening complications.