IgG4 Orbital Inflammation in a 5-Year-Old Child Presenting as an Orbital Mass

Kalapesi, Freny B; Garrott, Helen; Moldovan, Corina; Williams, Mark; Ramanan, Athimalaipet V; Herbert, H.

doi:10.3109/01676830.2013.764449

Cited by 31 publications

(12 citation statements)

References 4 publications

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“… Griepentrog 2013 [ 7 ] 14y F IgG4-ROD H+ N (L U) Pred, dosage unknown, and MMF MMF because of relapse after tapering pred, successful. Kalapesi 2013 [ 10 ] 5y F IgG4-ROD H+ El (1.52 g/l) Pred 1 mg/kg and MMF (600 mg/m2) Weaned off pred and maintained on MMF successfully. Naghibi 2013 [ 15 ] 16y F IgG4-related colitis, in the past AIP-1 H+ El (210 mg/dl) Adalimumab Refractory disease to pred 0.5 mg/kg, Aza and infliximab.…”

Section: Resultsmentioning

confidence: 99%

“…Furthermore, despite the presence of IgG4 positive plasma cells in the tissue, two case reports concerning Rosai-Dorfman disease and ALK-1 positive inflammatory myofibroblastic tumor [ 22 , 23 ] were excluded, because according to Boston consensus these diseases should not be considered as IgG4-RD. Serum IgG4 was measured in 23 of the 25 cases, and was found to be elevated in 16 cases [ 5 , 6 , 8 , 10 , 12 , 14 – 19 , 24 – 27 ] (70 %).…”

Section: Resultsmentioning

confidence: 99%

“…DMARDs were attempted as steroid-sparing agents in 11 cases. Mycophenolate mofetil was successful as a steroid-sparing agent in 3 of the 5 cases in which it was used [ 7 , 9 , 10 , 14 , 16 ]. Azathioprine was a successful as a steroid sparing agent in 2 of 4 cases in which it was used [ 5 , 15 , 17 , 25 ], while methotrexate was successful in 1 of 2 cases [ 9 ].…”

Section: Resultsmentioning

confidence: 99%

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IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

et al. 2016

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BackgroundImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of the body. If untreated, the disease can lead to fibrosis and irreversible organ damage. IgG4-RD mostly has been described in adults, hence it is generally unknown among pediatricians. This systematic search of the literature provides an overview of all reports published on IgG4-RD in children in order to create awareness of IgG4-RD in pediatrics and to emphasize the broad clinical presentation of this disease.MethodsA systematic literature search of Embase, Medline, Web-of-Science, PubMed publisher, Cochrane and Google Scholar was performed for case reports on IgG4-RD in children.ResultsOf total 740 articles identified by the search, 22 case reports including 25 cases of IgG4-RD in children were found. The median age of the children was 13 years, of which 64 % were girls. IgG4-related orbital disease (44 %) and autoimmune pancreatitis type 1/IgG4-related pancreatitis (12 %) predominantly occurred. Less frequently, other manifestations as pulmonary manifestation, cholangitis and lymphadenopathy were also found. Almost all cases were histologically proven. Prednisone was the first choice of treatment leading to favorable clinical response in 83 % of the cases. Maintenance therapy with steroid sparing agents was required in 43 % of the cases needing therapy. Rituximab was successful in all 4 cases, whereas, the disease modifying rheumatic drugs (DMARDs) mycophenolate mofetil, azathioprine and methotrexate were effective in almost 50 % of the cases.ConclusionIgG4-RD in children is a generally unknown disease among pediatricians, but several pediatric cases have been described. Prednisone is the first choice of treatment leading to disease remission in the majority of the cases. DMARDs and rituximab are alternative effective steroid sparing agents with more positive evidence for the latter.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

et al. 2016

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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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“…20 The mean age of onset for IgG4-ROD is 55.5 years (SD 12.9), slightly younger than for AIP, at somewhere between 58 and 69 years. [22][23][24][25] In 2 Japanese studies, patients with AIP had lacrimal and salivary gland lesions in 39% and 47.5%, 26,27 but a third Japanese study looking at 56 AIP patients found extrapancreatic lesions synchronously in 18, and metachronously in 18 (the AIP followed the other lesions), but only 2 of these were lacrimal gland lesions, and 8 were salivary gland lesions. [22][23][24][25] In 2 Japanese studies, patients with AIP had lacrimal and salivary gland lesions in 39% and 47.5%, 26,27 but a third Japanese study looking at 56 AIP patients found extrapancreatic lesions synchronously in 18, and metachronously in 18 (the AIP followed the other lesions), but only 2 of these were lacrimal gland lesions, and 8 were salivary gland lesions.…”

Section: Epidemiologymentioning

confidence: 99%