Immunoglobulin G4–related sclerosing disease with orbital inflammation in a 12-year-old girl

Sane, Mona; Chelnis, James; Kozielski, Rafał; Fasiuddin, Airaj

doi:10.1016/j.jaapos.2013.05.017

Cited by 21 publications

(24 citation statements)

References 10 publications

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“… Pifferi 2013 [ 26 ] 15y M IgG4-R pulmonary disease H+ El (1090 mg/dl) Pred 0.6 mg/kg/d Treatment for 4 weeks. Sane 2013 [ 11 ] 12y F IgG4-ROD and nephrotic syndrome H+ N (L U) Methylpred and rituximab The nephrotic syndrome also resolved. Initial good response to pred 40 mg, but relapse occured.…”

Section: Resultsmentioning

confidence: 99%

“…Other manifestations were IgG4-related pancreatitis/autoimmune pancreatitis type 1 (AIP 1) (12 %), IgG4-related cholangitis (8 %), IgG4-related pulmonary disease (8 %), and the remaining cases (28 %) were single cases of Riedel’s thyroiditis/IgG4-related thyroid disease, IgG4-related sialadenitis, IgG4-related mesenteritis, IgG4-related lymphadenopathy, IgG4-related dacryoadenitis, IgG4-related sinonasal disease and IgG4-related hepatic mass. Kidney involvement was seen in three cases in combination with other organ manifestations [ 11 , 13 , 14 ]. Systemic IgG4-RD (two or more organ manifestations) occurred in 40 % of the cases [ 8 , 11 – 19 ].…”

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%

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%

IgG4-Related Ophthalmic Disease. Part I

McNab

McKelvie

2015

Ophthalmic Plastic &Amp; Reconstructive Surgery

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Immunoglobulin G4–related sclerosing disease with orbital inflammation in a 12-year-old girl

Cited by 21 publications

References 10 publications

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

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

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

IgG4-Related Ophthalmic Disease. Part I

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