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Fibroinflammatory lesions of the sinonasal tract include inflammatory polyps (chronic rhinosinusitis), various infectious, sarcoidosis, and NK/T-cell lymphoma as examples of the most commonly encountered lesions. However, the differential diagnosis includes several less frequently encountered entities, such as granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), eosinophilic angiocentric fibrosis considered part of IgG 4 -related disease, and Rosai-Dorfman disease. This review focuses on these latter entities providing an update on clinical, laboratory, imaging, histology, and ancillary testing employed to reach an actionable diagnosis., Competing Interests: Disclosure The author declares that he has no conflict of interest as it relates to this review. This review article was performed in accordance with ethical standards and did not require informed consent. Consent to participate or for publication was waived as it is a review article without any uniquely identifying personal data. The opinions or assertions contained herein are the private views of the author., (Published by Elsevier Inc.)

Lymphadenopathy occurring in patients with immunoglobulin G4 (IgG4)-related disease, termed IgG4-related lymphadenopathy, shows morphologic heterogeneity and overlap with other nonspecific causes of lymphadenopathy including infections, immune-related disorders, and neoplasms. This review describes the characteristic histopathologic features and diagnostic approach to IgG4-related disease and IgG4-related lymphadenopathy, with comparison to nonspecific causes of increased IgG4-positive plasma cells in lymph nodes, and with emphasis on distinction from IgG4-expressing lymphoproliferative disorders., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: IgG4-related disease (IgG4-RD) is an inflammatory process that uncommonly can present in the skull base and calvarium and mimic a tumor but the nature of this condition is not well summarized in the neurosurgical literature., Methods: A review was performed of 2 cases of IgG4-RD in the skull base highlighting the diagnostic challenges with assessment of these skull base lesions, and a systematic review of relevant literature was carried out., Results: A systematic review of the literature conducted in accordance with PRISMA guidelines identified 113 articles, with 184 cases of IgG4-RD in the skull base or calvarium. The most commonly affected locations include the meninges, cavernous sinus, base of the posterior fossa, clivus, and mastoid bone. Headache, visual and auditory disturbances, cranial nerve dysfunction, and seizures were the most common presenting symptoms. Medical treatment was highly successful and most commonly consisted of corticosteroids coadministered with immunosuppressive agents such as rituximab. Prevalence seemed to be equal between sexes, and serum IgG4 levels were increased in 61% of patients. Delayed diagnosis and a need for multiple biopsies were reported in numerous cases. Two cases of skull base IgG4-RD from the authors' institution show the variable presentations of this disease. More invasive surgical biopsies were required in both cases, and corticosteroid treatment led to significant clinical improvement., Conclusions: IgG4-RD is an uncommon condition with an increasing body of reported cases that can affect the skull base and calvarium and should be in the differential diagnosis, because delay in diagnosis and treatment may be common., (Copyright © 2021. Published by Elsevier Inc.)

Immunoglobulin-G4-related disease (IgG4-RD) is an autoimmune-mediated spectrum of diseases, characterized by infiltration of IgG4+ plasma cells into one or multiple organs, with the pancreas being the most commonly affected organ. The disease mostly affects middle-aged to elderly men. Diagnosis requires an integration of clinical, radiologic, pathologic, and serologic studies. Histologically, there is an increased infiltration of IgG4+ plasma cells, elevated ratio of IgG4+/IgG plasma cells of more than 40%, and a storiform pattern of fibrosis. There may be eosinophilia, along with elevated IgG4 levels. IgG4-RD can mimic several diseases and should be differentiated from inflammatory and neoplastic processes. Recently, there has been increased awareness of cutaneous involvement by IgG4-RD either as an isolated lesion or primary involvement or as a secondary involvement from a systemic disease. Clinically, cutaneous IgG4+-related disease presents as papules, plaques, and nodules involving the head and neck areas. We have provided a systematic review of the literature of this new and challenging entity of cutaneous IgG4-RD., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Spinal immunoglobulin G4-related hypertrophic pachymeningitis (IgG4-HP) is a rare disease. Little information is known regarding the diagnosis, management, and prognosis of patients with spinal IgG4-HP., Methods: The authors present a case of spinal IgG4-HP with a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Relevant studies (up to April 2020) that reported patients with spinal IgG4-HP, based on the criteria of Japan College of Rheumatology, were identified from the PubMed and Cochrane Library databases., Results: This systematic review identified 33 patients, including the present case, of whom 21 were male and 12 were female. The mean value of age was 51.2 (±12.6) years. Eight patients had systemic involvement. In addition, among 33 patients, 13 patients had an elevated serum IgG4. Surgery was performed in 31 patients. Steroid therapy alone and steroid therapy with immunosuppressants were effective in 94% and 100% of the cases, respectively. Furthermore, 31 of 33 patients reported improved outcomes, 1 patient died due to infection, and in 2 patients the data were not available., Conclusions: Spinal IgG4-HP is a rare entity. In addition, it should be considered in the differential diagnosis of space-occupying lesions around the spinal cord. Histopathology with immunohistochemistry results provides the most reliable evidence for diagnosis. Steroid therapy is the first line of treatment. Surgical decompression may be required in patients presenting with nerve root and/or spinal cord compression. Long-term follow-up is necessary for patients with spinal IgG4-HP., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Immunoglobulin G4 related disease (IgG4-RD) is a recently recognized immune-mediated disease which is far from understanding. A case of inflammatory demyelinating pseudotumor had been confirmed as IgG4-RD according to pathology features and clinical context. Combined with liver dysfunction, IgG4 related sclerotic cholangitis was suspected. However, primary biliary cholangitis was finally diagnosed by immune marks and histopathological findings. This is the first report in which mass lesions in the brain parenchyma were caused by IgG4-RD while liver dysfunction was due to primary biliary cholangitis. The clinical features of IgG4-RD are miscellaneous, and the accumulation of case reports might enrich clinicians experience and broaden their horizons about this condition., (Copyright © 2020 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.)

Sellar region lesions include a broad range of benign and malignant neoplastic as well as non-neoplastic entities, many of which are newly described or have recently revised nomenclature. In contrast to other intracranial sites, imaging features are relatively less specific, and the need for histopathological diagnosis is of paramount importance. This review will describe pituitary adenomas, inflammatory lesions, and tumors unique to the region (craniopharyngioma) as well as tumors which may occur in but are not exclusively localized to the sellar location (schwannoma, metastasis, etc.)., Competing Interests: Disclosure The authors have no relevant financial or commercial disclosures. No specific funding sources were used in the writing of this article., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Chronic pancreatitis (CP) is a common disease in the English cocker spaniel (ECS) and is characterized histologically by duct destruction, interlobular fibrosis and dense periductular and perivenous lymphocytic aggregates. These features are also found in human autoimmune pancreatitis type 1, part of a glucocorticoid-responsive, multiorgan syndrome, newly recognized as IgG4-related disease (IgG4-RD). Human IgG4-RD affects one or several organs, often showing a predominance of IgG4 + plasma cells histologically, with an IgG4 + :total IgG + plasma cell ratio of >40%. This study investigated whether ECSs with CP and/or inflammatory disease in several organs show an increase in IgG4 + plasma cells within affected tissues. Histological sections of pancreas, liver, kidney, salivary gland and conjunctiva were obtained from ECSs with idiopathic chronic inflammatory disease affecting those tissues. Tissue samples from age-matched dogs of other breeds with similar diseases were also sampled. Control diseased tissue samples, from dogs without a suspected immune-mediated disease, were included. A subset of ECSs and dogs of other breeds presented with disease in more than one organ. Immunohistochemistry was performed with primary reagents detecting total IgG and three of the four canine IgG subclasses (IgG2, IgG3 and IgG4). Normal sections of pancreas and liver showed an absence of labelled plasma cells of any subclass. Normal kidney and salivary gland sections showed the presence of a few labelled plasma cells (<10 plasma cells/high-power field). Fourteen tissue sections from 12 ECSs and seven sections from six dogs of other breeds showed elevated numbers of IgG4 + plasma cells and IgG4 + :IgG + ratios >40%. Individual dogs (ECSs and other breeds) showed marked increases in IgG4 + cells. There were no significant differences in the number of IgG4 + plasma cells between ECSs and dogs of other breeds for affected pancreas, liver, salivary glands and conjunctiva. Kidney sections had more IgG4 + cells, for both ECSs and dogs of other breeds, than did sections from other organs. Dogs of other breeds had significantly more IgG4 + plasma cells in affected kidneys than ECSs. In conclusion, several ECSs and dogs of other breeds fulfilled the histological criteria for the diagnosis of IgG4-RD, supporting the existence of a multiorgan immune-mediated disease in ECSs and some dogs of other breeds., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Immunoglobulin (Ig)G4-related disease (IgG4-RD) was defined only recently and can be found in many organs. As intracranial lesions, hypophysitis and pachymeningitis are well known, whereas intracranial pseudotumor is unusual. This case involved multiple intracranial pseudotumors without extracranial lesions, mimicking multiple meningioma., Case Description: A 72-year-old woman was referred to our hospital with an incidental mass lesion at the craniocervical junction on magnetic resonance imaging (MRI). MRI showed diffuse enhanced extra-axial nodules around the medulla and middle cranial fossa. Surgery was performed for the gradually enlarging tumor. Intraoperative findings showed hard nodules around the vertebral artery. We performed subtotal resection. Neuropathological findings showed diffuse lymphoplasmacytic infiltration with lymphoid follicles. Immunohistochemical studies for IgG4 and IgG showed the histological criteria for IgG4-RD were met. Given the high IgG4 serum level, we diagnosed IgG4-RD. Steroid was administered initially but was then tapered, and nodules have since remained small., Conclusions: This case demonstrates IgG4-RD mimicking meningioma with multiple masses but without extracranial lesions. This case and previous descriptions suggest the possibility of pseudotumor due to IgG4-RD in the presence of multiple extra-axial lesions at the dura and supplying artery, hard nodules with thickening of the artery, and frozen section findings of lymphoplasmacytes surrounded by rich collagen fibers. In such cases, the pseudotumor due to IgG4-RD should be resected, and total resection might not be warranted., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Many cases of immunoglobulin G4 (IgG4)-related disease involving different organ systems have been reported in the literature since the disorder was first described in patients with sclerosing pancreatitis. This report discusses an interesting case of IgG4-related cardiovascular disease that involved the pericardium and resulted in reoccurring chest pain in a 36-year-old man. The challenging diagnosis was made using established diagnostic criteria for other tissue sites and included the presence of elevated serum IgG4, pericardial infiltration by IgG4-positive plasma cells, focal phlebitis, and fibrosis. The patient's symptoms improved with immunosuppressive therapy., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)