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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.
Diseases of the Aorta
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Immunoglobulin G subclass 4 (IgG4)-related sclerosing disease can occur in the cardiovascular system and some inflammatory aortic aneurysms belong to IgG4-related sclerosing disease. Immunohistochemistry shows numerous IgG4-positive plasma cell infiltrates. Inflammatory aortic aneurysms are uncommon, but the lymphoplasmacytic subtype is probably associated with IgG4-related systemic disease. Immunoglobulin G subclass 4 (IgG4)-related aortitis is part of a systemic disease with high variability in diverse organ manifestations, including autoimmune pancreatitis, retroperitoneal fibrosis, IgG nephropathy, sclerosing cholangitis, mediastinal pseudotumours and inflammatory pericarditis. IgG4-related disease is usually characterized by numerous IgG4-positive plasma cells, raised IgG4 plasma levels and steroid sensitivity. It is commonly observed in elderly men with abdominal aortic aneurysm (AAA) and concomitant retroperitoneal fibrosis. Studies have identified significant differences between the abdominal and thoracic presentations, the latter appears more frequently in females and correlates less with serum IgG4, although the histopathological picture is similar in both. IgG4 aortitis can include both thoracic and abdominal aneurysms with intramural haematoma formation.13 There is frequently a good response of IgG4-related systemic disease to steroid treatment which may prevent progression of the IgG4-related systemic disease.14
Endocrinology
Published in Kristen Davies, Shadaba Ahmed, Core Conditions for Medical and Surgical Finals, 2020
Causes of primary hypothyroidism include: Primary atrophic hypothyroidism: Common, lymphocytic infiltrate of the gland – AutoimmuneHashimoto's thyroiditis: Goitre due to lymphocytic and plasma cell infiltration. Occasionally can have a period of hyperthyroidism prior to hypothyroidism – AutoimmuneIodine deficiency (most common cause worldwide)Post-thyroidectomy or radioiodine treatmentDrugs: Amiodarone (can cause hypothyroidism and hyperthyroidism as structurally like T4), lithiumSubacute thyroiditis (can also have period of hyperthyroidism prior to hypothyroidism)Riedel's thyroiditis: Replacement of thyroid tissue with fibrotic tissue. IgG4-related disease
Lacrimal Gland and Orbital Lesions in LatY136F Knock-in Mice, a Model for Human IgG4-Related Ophthalmic Disease
Published in Current Eye Research, 2022
Shoko Hamaoka, Masayuki Takahira, Mitsuhiro Kawano, Kazunori Yamada, Kiyoaki Ito, Tetsuhiko Okuda, Sachiyo Hatake, Marie Malissen, Bernard Malissen, Kazuhisa Sugiyama
Immunoglobulin G4 (IgG4)-related disease is a clinical entity characterized by elevated serum IgG4 concentrations and IgG4-positive lymphoproliferative lesions in multiple organs such as the pancreas, lacrimal and salivary glands (also known as Mikulicz disease), kidney, aorta, and lung.1–4 As ophthalmic lesions, lacrimal gland swelling is the most frequent, but extraocular muscle swelling and trigeminal nerves enlargement may also occur.5 A less frequent but more severe outcome of IgG4-related ophthalmic disease is optic neuropathy, resulting in visual field and visual acuity deterioration.6 Although the majority of patients with IgG4-related ophthalmic disease respond well to corticosteroid treatment,7 relapse is common during the tapering or discontinuation of steroid therapy.8 Treatment with rituximab, an anti-CD20 antibody, has shown promise as an effective medication for IgG4-related disease,8 but relapses are also common following rituximab discontinuation.9 Therefore, it is necessary to elucidate the etiology of IgG4-related disease to define more effective, long-term treatment strategies.
Plasma cell granuloma of the conjunctiva in a young female
Published in Orbit, 2021
Md. Shahid Alam, Vathsalya Vijay, Atanu Barh, Krishnakumar Subramanian
Plasma cell granuloma is a rare non-neoplastic inflammatory condition of unknown etiology. Histologically it is characterised by proliferation of polyclonal plasma cells, lymphocytes, neutrophils, eosinophils, and histiocytes in a fibrotic background.1 The exact etiology and natural history of these lesions remain unclear. Some authors include it within the spectrum of IgG4-related disease.2 Though any part of the body can be involved, the most frequently reported are lungs and gastrointestinal tract3 There is no consensus on the exact management of this otherwise enigmatic disorder; surgical excision, radiotherapy, steroids, and immunosuppressants have been used with varying results.4 Conjunctival disease per se is very rare and usually associated with involvement of other organs.5 The current case report describes a bilateral conjunctival plasma cell granuloma in a young patient without any systemic involvement. The present article adheres to the tenets of the declaration of Helsinki. The patient’s parents gave consent for publication of photograph and other details for research purpose.
Is Kimura’s disease associated with juvenile temporal arteritis? A case report and literature review of all juvenile temporal arteritis cases
Published in Modern Rheumatology Case Reports, 2021
Takafumi Tomizuka, Hirotoshi Kikuchi, Kurumi Asako, Daisuke Tsukui, Yoshitaka Kimura, Yoshinao Kikuchi, Yuko Sasajima, Hajime Kono
A biopsy of the left temporal artery revealed eosinophil infiltration in all layers of the arterial wall, accompanied by stenosis of the lumen due to intimal thickening, thrombus formation, and fibrinoid degeneration. Capillary hyperplasia and fibrosis were seen around the artery, and eosinophil infiltration was observed. No morphological difference in eosinophils was observed between the Kimura’s disease lesion and the JTA lesion by optical microscopy. No granulomas were noted, but a few multinucleated giant cells were focally observed (Figure 4). IgG4-related disease was excluded by IgG4 staining. These findings are consistent with the diagnosis of JTA, although some multinucleated giant cells were observed. The patient was not diagnosed with giant cell arteritis because of the absence of systemic symptoms and increase in erythrocyte sedimentation rate; moreover, our histological findings did not match with those reported by Wakugawa et al. [5]. The patient was treated with 30 mg/day prednisolone followed by gradual tapering, which led to a decrease in size of the masses of the posterior auricle. No recurrence of JTA has been detected for 2 years to date.