Orbital IgG4 Disease: Imaging Findings on 68Ga-DOTANOC PET/CT / 대한핵의학회잡지

Immunoglobulin G4 (IgG4)–related diseases are a spectrum of systemic inflammatory conditions of unknown etiology, which are characterized by infiltration of tissues by IgG4 plasma cells and sclerosing inflammation (Cheuk and Chan Adv Anat Pathol 17:303-32, 2010). Although this condition was initially described in relation to autoimmune pancreatitis, now it has been reported in almost every organ system of body (Zen and Nakanuma Am J Surg Pathol 34:1812-9, 2010, Masaki et al. Ann Rheuma Dis 68:1310-5, 2009). Orbital involvement by IgG4 disease can involve extraocular muscles (EOM), lacrimal glands, conjunctiva, eyelids, infraorbital nerve, orbital fat, and nasolacrimal system (McNab and McKelvie. Ophthal Plast Reconstr Surg 31:167-78, 2015, Katsura et al. Neuroradiology 54:873-82, 2012). The basis of using ⁶⁸Ga-DOTANOC PET/CT in IgG4 orbital disease is the known expression of somatostatin receptors in chronic inflammatory cells (Cuccurullo et al. Indian J Radiol Imaging 27:509-16, 2017) and also avidity shown previously in other IgG4-related diseases (Cheng et al. Clin Nucl Med 43:773-6, 2018).

Eosinophilic angiocentric fibrosis of nasal septum: a case report.

Eosinophilic Angiocentric Fibrosis (EAF) is an uncommon benign condition involving the sinonasal tract and rarely the larynx and orbit. Its etiology still remains unclear, although some studies have hypothesized it as a form of IgG4-related systemic disease. Histologically EAF is characterised by the presence of mixed inflammatory cells with eosinophil predominance in the early stage, followed by concentric layers of sclerosis around the small caliber vessels in the late stage. The rarity of this condition, tumor like presentation and oblivious etiology, makes it an interesting entity. We hereby report one such rare case of EAF, since the awareness of this entity is essential to make an accurate diagnosis.

Waffle Procedure for a Constrictive Pericarditis as an Emerging Manifestation of Hyper-IgG4 Disease / 日本心臓血管外科学会雑誌

A 74-year-old man presenting with general fatigue and dyspnea was admitted to another hospital. He was transferred to our hospital because his symptoms deteriorated and pericardial fluid increased. The symptoms did not improve even after percutaneous pericardial drainage. On a diagnosis of heart failure due to pericardial constriction, he underwent pericardiectomy. No hemodynamics improvement was found despite subtotal pericardiectomy. Multiple longitudinal and transverse incisions like a waffle were made in the thickened epicardium and improved the hemodynamics. The symptoms improved after sugery. Steroid therapy was effective after pathological examination of the excised epicardium that confirmed an emerging manifestation of hyper-IgG4 disease. We report a waffle procedure with good results for a constrictive pericarditis with hyper-IgG4 disease.