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Optic Neuropathies Associated with Systemic Disorders And Radiation-Induced Optic Neuropathy
Published in Vivek Lal, A Clinical Approach to Neuro-Ophthalmic Disorders, 2023
IgG4-related disease is a multi-organ, fibro-inflammatory condition, which has recently been recognized as the cause of several systemic and neurological inflammatory syndromes previously regarded as idiopathic in nature (7, 8). This disorder has a predilection for involving anatomical structures in the head and neck, including the orbits, meninges, pituitary gland and peripheral nerves (7, 8). Less frequently, IgG4-related disease affects the brain parenchyma and vasculature (7, 8). Autoimmune pancreatitis, sclerosing cholangitis, chronic sclerosing sialadenitis (especially of the submandibular glands), dacryoadenitis and retroperitoneal fibrosis are common systemic features (7, 8). IgG4-related disease is most commonly reported among middle-aged to elderly men. Yet, when disease manifestations involve the head and neck, both sexes may be equally affected. Ocular involvement is relatively common among pediatric patients (7, 8). Since disease manifestations may wax and wane over time, the diagnosis of IgG4-related disease is often delayed. Yet, early identification of this condition is important, because treatment response is often favorable.
Non-Neoplastic Salivary Gland Diseases
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Stephen R. Porter, Stefano Fedele, Valeria Mercadante
The salivary glands are commonly affected, and in the past the features were termed ‘Mikuliz disease’ ‘Kuttner tumour’ or ‘chronic sclerosing sialadenitis’. The submandibular gland is commonly affected, although the parotids, sublingual glands and minor salivary glands can occasionally be affected. The salivary gland features manifest as long-standing and generally asymptomatic swelling, sometimes, but not always, with symptoms of xerostomia.75, 76
Salivary Glands
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Lesions that need to be distinguished from MZL also include cystic lymphoepithelial lesion (in HIV+ and HIV− patients; Figure 43.5), Warthin’s tumor (Figure 43.6), chronic sclerosing sialadenitis (Kuttner tumor), and reactive processes involving the lymph nodes within or around the salivary glands. IgG4-related process which may mimic MALT lymphoma, is characterized by prominent lymphoplasmacytic infiltrate, reactive follicles, variable degree of fibrosis and increased number of IgG4+ plasma cells within germinal centers. Plasma cells are polytypic and there is no clonal IGH rearrangement.
Involvement of two or more sets of lacrimal glands and/or major salivary glands is related to greater systemic disease activity due to multi-organ involvement in IgG4-related dacryoadenitis/sialadenitis
Published in Modern Rheumatology, 2021
Yoshinori Takahashi, Ichiro Mizushima, Masahiro Konishi, Hiroyuki Kawahara, Hajime Sanada, Kazuyuki Suzuki, Akari Takeji, Satoshi Hara, Kiyoaki Ito, Hiroshi Fujii, Mitsuhiro Kawano
Concerning the involvement of ≥2 sets of LGs and/or MSGs in IgG4-DS, although its diagnostic significance is generally recognized, its prevalence has not been well determined. Kitagawa et al. reported that, in 12 patients with IgG4-related chronic sclerosing sialadenitis (Küttner tumor), 6 had unilateral gland involvement [6]. Sakamoto et al. reported that 61.9% and 6.5% of patients with IgG4-DS had involvement of ≥2 sets of LGs and/or MSGs and unilateral gland involvement, respectively, in their analysis of 31 patients [15]. In the present study analyzing 97 patients with IgG4-DS, involvement of ≥2 sets of LGs and/or MSGs was seen in 45% of patients and unilateral gland involvement in 19%. Accordingly, it is estimated that the prevalence of the former is approximately 50% and that of the latter is 10–20%. Verification using data from more patients will be necessary to more accurately clarify the prevalence of each pattern.
Major salivary gland enlargement in IgG4-related disease is associated with multiorgan involvement and higher basal disease activity
Published in Modern Rheumatology, 2020
Eduardo Martín-Nares, Arturo Ángeles-Ángeles, Gabriela Hernandez-Molina
Although the traditional concept of Mikulicz disease and chronic sclerosing sialadenitis typically affected men, we did not find a male predominance among the group with major salivary gland involvement likewise the studies by Li and Hong [8,12]. In addition in our study, 63% of our patients auto-referred dry mouth. Conversely, the presence of xerostomia was not assessed in Li and cols study, but they reported that 35.7% of the patients had decreased salivary flow, 22.6% impaired submandibular uptake function and 77.4% impaired submandibular secretory function by scintigraphy [8]. Moreover in our study, salivary gland enlargement was identified both clinically and radiologically in the majority of patients (almost 70%), whereas Li and cols reported a higher prevalence of parotid enlargement when searched by computed tomography instead of palpation [8].
Diagnostic sensitivity of cutoff values of IgG4-positive plasma cell number and IgG4-positive/CD138-positive cell ratio in typical multiple lesions of patients with IgG4-related disease
Published in Modern Rheumatology, 2018
Ichiro Mizushima, Kazunori Yamada, Kenichi Harada, Shoko Matsui, Takako Saeki, Satoru Kondo, Masayuki Takahira, Yuko Waseda, Yasuhito Hamaguchi, Hiroshi Fujii, Masakazu Yamagishi, Mitsuhiro Kawano
Our data suggest that the cutoff value of IgG4 + PC number in IgG4-related submandibular gland disease needs to be lowered because only 50.0% of true IgG4-related sialoadenitis patients with typical other organ involvement exceed this cutoff value. Similarly, Strehl et al. analyzed seven patients with IgG4-related sclerosing sialadenitis/Küttner's tumor and showed that the absolute IgG4 counts ranged from 4 to 104/hpf with an average of 40/hpf, although exactly how many patients had absolute IgG4 counts exceeding 100/hpf in Strehl’s study is unclear [3]. In contrast, they showed that the absolute IgG4 counts in the submandibular glands were very low, in the range of 0–26.8/hpf, in chronic non-autoimmune sialoadenitis related to sialolithiasis. In addition, Kitagawa et al. showed that the absolute IgG4 counts ranged from 62.2 to 232.4/hpf with an average of 118.2/hpf in 16 submandibular gland lesions of 12 patients with chronic sclerosing sialadenitis (Küttner's tumor) [9]. Six of these 16 lesions had absolute IgG4 counts under 100/hpf. They also showed that the absolute IgG4 counts were under 10/hpf in the submandibular glands of eight patients with sialolithiasis and the minor salivary glands of 13 patients with Sjögren’s syndrome. These studies suggested that the cutoff value adopted in the ICS for the pathology of IgG4-RD is too high leading to low sensitivity in diagnosing IgG4-related sialadenitis, although the study of Geyer et al. reported the high average IgG4-positive PC number of 229/hpf (range 75–608/hpf) in 13 submandibular gland lesions of patients with chronic sclerosing sialadenitis [10]. In addition, even lowering the cutoff value would seem to have had only a minimal influence on the specificity because control disease showed a small number of IgG4 + PC infiltrates in the salivary glands.