A Clinicopathologic Case Study of Two Patients with Pediatric Orbital IgG4-related Disease

Griepentrog, Gregory J.; Vickers, Rob W.; Karesh, James W.; Azari, Amir A.; Albert, Daniel M.; Bukat, Cat N.

doi:10.3109/01676830.2013.822899

Cited by 24 publications

(16 citation statements)

References 11 publications

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“…IgG4-RD typically affects middle-aged to elderly men, with sporadic reports of paediatric cases [9]. The clinical presentation is usually indolent, with signs and symptoms becoming evident over months or even years.…”

Section: Overview Of Clinical Manifestationsmentioning

confidence: 99%

Immunology of IgG4-related disease

Della‐Torre

Lanzillotta

Doglioni

2015

Clinical and Experimental Immunology

183

215

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SummaryImmunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that derives its name from the characteristic finding of abundant IgG4 1 plasma cells in affected tissues, as well as the presence of elevated serum IgG4 concentrations in many patients. In contrast to fibrotic disorders, such as systemic sclerosis or idiopathic pulmonary fibrosis in which the tissues fibrosis has remained largely intractable to treatment, many IgG4-RD patients appear to have a condition in which the collagen deposition is reversible. The mechanisms underlying this peculiar feature remain unknown, but the remarkable efficacy of B cell depletion in these patients supports an important pathogenic role of B cell/T cell collaboration. In particular, aberrant T helper type 2 (Th2)/ regulatory T cells sustained by putative autoreactive B cells have been proposed to drive collagen deposition through the production of profibrotic cytokines, but definitive demonstrations of this hypothesis are lacking. Indeed, a number of unsolved questions need to be addressed in order to fully understand the pathogenesis of IgG4-RD. These include the identification of an antigenic trigger(s), the implications (if any) of IgG4 antibodies for pathophysiology and the precise immunological mechanisms leading to fibrosis. Recent investigations have also raised the possibility that innate immunity might precede adaptive immunity, thus further complicating the pathological scenario. Here, we aim to review the most recent insights on the immunology of IgG4-RD, focusing on the relative contribution of innate and adaptive immune responses to the full pathological phenotype of this fibrotic condition. Clinical, histological and therapeutic features are also addressed.

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Section: Overview Of Clinical Manifestationsmentioning

confidence: 99%

Immunology of IgG4-related disease

Della‐Torre

Lanzillotta

Doglioni

2015

Clinical and Experimental Immunology

183

215

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“…Although there are myriad excellent studies published by the Asian medical community, it is unclear whether this publishing trend represents an increased prevalence of IgG4-RD in Asia, or simply an increased interest in publishing the data given an early recognition of the disease [1]. IgG4-RD appears to have a predilection for the middle-aged or older [4], though pediatric systemic and orbital IgG4-RD have been reported [5][6][7][8]. Sex may influence IgG4-RD organ involvement.…”

Section: Introductionmentioning

confidence: 80%

Management of orbital IgG4-related disease

Glass

Freitag

2015

Current Opinion in Ophthalmology

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“…Until September 2017, to the best of our knowledge, 13 cases -10 females and 3 males-of children with ophthalmic manifestations have been described [13][14][15][16][17][18][19][20][21][22]. The most common symptomatology was unilateral protrusion/swelling (11/13, 84.6%), while eyelid involvement was also mentioned in 6/13 (46.1%) cases (Table-1 Serum IgG4 levels were found to be increased in 5/13 (38.5%) of the patients, inflammation markers in 3/6 (50%), while autoimmune profile was negative in all the patients for whom data were available (Table-1) Clinical findings and imaging studies, in these patients, suggested "orbital mass" as the most common finding.…”

Section: Paediatric Populationmentioning

confidence: 99%

Ocular manifestations of IgG4-related disease in children. More common than anticipated? Review of the literature and case report

2017

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IgG4-related disease (IgG4-RD) is an entity with various clinical manifestations. Histopathologically, it is characterized by lymphoplasmacytic infiltrates enriched in IgG4 (+) plasmacytes and usually fibrosis of the affected tissue. Most of the patients have also increased IgG4 serum levels and they respond to glucocorticosteroids. In children, due to its rare occurrence, IgG4-RD is ill defined. From the published studies, so far, it appears that ocular manifestations are very common in the paediatric population with IgG4-RD. Herein, we describe a new case of a child with IgG4-RD with ocular involvement manifested with orbit and eyelid swelling, successfully treated with steroids. In addition, we review the clinical, laboratory, histopathologic and radiologic characteristics of the published paediatric cases with IgG4-RD and ocular involvement, critically comparing them with the characteristics of the adult population. It seems that ocular manifestations are more frequently observed in children than in adults. Also, the pattern of involvement is different, with extraocular muscles and soft tissues being more commonly affected than the lacrimal glands.

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A Clinicopathologic Case Study of Two Patients with Pediatric Orbital IgG4-related Disease

Abstract: The purpose of this report is to describe the clinical, radiographica, and histopathologic findings in two pediatric patients with orbital IgG4-related disease.

Cited by 24 publications

References 11 publications

Immunology of IgG4-related disease

Immunology of IgG4-related disease

Management of orbital IgG4-related disease

Ocular manifestations of IgG4-related disease in children. More common than anticipated? Review of the literature and case report

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