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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.

CMR identifies heart involvement in a significant percentage of SLE children with cardiac symptoms and normal routine noninvasive evaluation.The incidence of heart disease is lower in SLE children compared with SLE adults.Predominance of myocarditis and myocardial infarction is observed in SLE children and SLE adults, respectively.

Cardiac involvement in pediatric systemic autoimmune diseases has a wide spectrum of presentation ranging from asymptomatic to severe clinically overt involvement. Coronary artery disease, pericardial, myocardial, valvular and rythm disturbances are the most common causes of heart lesion in pediatric systemic autoimmune diseases and cannot be explained only by the traditional cardiovascular risk factors. Therefore, chronic inflammation has been considered as an additive causative factor of cardiac disease in these patients. Rheumatic fever, juvenile idiopathic arthritis, systemic lupus erythematosus, ankylosing spondylitis/spondyloarthritides, juvenile scleroderma, juvenile dermatomyositis/polymyositis, Kawasaki disease and other autoimmune vasculitides are the commonest pediatric systemic autoimmune diseases with heart involvement. Noninvasive cardiovascular imaging is an absolutely necessary adjunct to the clinical evaluation of these patients. Echocardiography is the cornerstone of this assessment, due to excellent acoustic window in children, lack of radiation, low cost and high availability. However, it can not detect disease acuity and pathophysiologic background of cardiac lesions. Recently, the development of cardiovascular magnetic resonance imaging holds the promise for early detection of subclinical heart disease and detailed serial evaluation of myocardium (function, inflammation, stress perfusion-fibrosis) and coronary arteries (assessment of ectasia and aneurysms).

BackgroundCommon practice is to withhold vaccination with live-attenuated vaccines in patients with rheumatic diseases on high-dose DMARDs, glucocorticosteroids or biological agents, due to limited safety data, and the (theoretical) risk of introducing an infectious disease to the patient. Evidence for this approach is low. We collected data from pediatric rheumatologists who vaccinate these patients, to obtain additional safety data, which might update and revise this approach.ObjectivesTo collect retrospective data in patients with JIA and other diseases who received live booster MMR or MMRV while on DMARDs, glucocorticosteroids or biological agents.MethodsData from 13 pediatric rheumatology centers in 10 countries were collected.Results234 patients were reported; mean age 5± 2.7, 70% girls. 206 had JIA;. 46% oligoarticular, 36% polyarticular, 8% systemic, 5% SPA types, 5% JIA and uveitis. 48% of JIA patients were in remission on medication. Disease activity was low in 38%, high in 2%, moderate in 7%; 11 patients had juvenile dermatomyositis, 3 systemic and 2 localized scleroderma, 4 isolated idiopathic uveitis, 1 CINCA syndrome, 1 MKD, and 1 FMF.110 patients had MMR/V booster while on MTX; 3 reported mild side-effects of local skin reaction and pain, none had disease flare. 76 had booster while on MTX+ anti-TNF; 7 reported mild and transient adverse events of local skin reaction, fever and URTI. 39 had booster while on anti-TNF alone; 1 reported fever. 3 had booster while on tocilizumab, 7 on anakinra, and 5 on canakinumab. There was no relation between disease activity, type or duration, sex, age and outcome of vaccinations. No vaccine infection related to measles, rubella, mumps and varicella were reported.ConclusionThis large, retrospective data collection demonstrates that live-attenuated booster vaccine is probably safe in children with rheumatic diseases, on immunosuppressive therapies. This strengthens the new PRES recommendation: “Vaccination of live-attenuated vaccines in patients on high-dose DMARD, high-dose glucocorticosteroids or biological agents can be considered on a case-by-case basis, weighing the risk of infections against the hypothetical risk of inducing infection through vaccination.” These data provide the basis for a large, prospective data collection study that is planned by the PReS vaccination study group. It will increase the current level of evidence for the safety of vaccinations in our pediatric rheumatology population.Disclosure of InterestsYosef Uziel: None declared, Veronica Moshe Bergonzo: None declared, Beata Onozo: None declared, Andrea Kulcsar: None declared, Diana Tróbert-Sipos : None declared, Jonathan Akikusa: None declared, Gecilmara Salviato Pileggi : None declared, Despoina Maritsi: None declared, Ozgur KASAPCOPUR: None declared, Roubini Smerla: None declared, Donato Rigante: None declared, Erato Atasali: None declared, Mariana Rodrigues: None declared, Balahan Makay Speakers bureau: Enzyvant, Novartis, Roche, Abbvie, Nico Wulffraat: None declared, Natasa Toplak: None...