Ab1007 prevalence and Characteristics of Temporo-Mandibular Joint (Tmj) Involvement in a Cohort of Young Adult Patients With Diagnosis of Juvenile Idiopathic Arthritis (Jia) and Non-Jia Chronic Inflammatory Arthropaties

Lercara, Adriano; Crepaldi, G.; Licciardi, Francesco; Davico, Marco; Cirillo, Stefano; Marouen, Sarah; Vandelli, E.; Pers, Yves‐Marie; Lomater, Claudia

doi:10.1136/annrheumdis-2019-eular.3050

Cited by 2 publications

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“…Clinical examination has been shown to have high specificity but low sensitivity in revealing TMJ inflammation 3 . To date MRI is the gold standard to assess TMJ involvement 4 Objectives: To investigate the prevalence of TMJ involvement in young adults with JIA and young adults with non-JIA inflammatory rheumatisms. Methods: Patients were recruited prospectively in 2 clinical centers.…”

Section: Discussionmentioning

confidence: 99%

Ab1005 the Unusual Adverse Events Including Multiple Sclerosis Developed Under Abatacept Therapy in a Patient With Jia

Latypova

Shapovalenko

Nikishina

2019

Abstracts Accepted for Publication

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BackgroundBiological therapy in patients with pediatric rheumatology disease may be associated with adverse events up to demyelinative lesion of CNS including multiple sclerosis (MS). It is a rare condition in the pediatric practice.Objectivesto describe the clinical case of newly diagnosed psoriasis and subsequently developing of multiple sclerosis in pediatric patient revealed in JIA who has been followed-up in Pediatric department of V.A. Nasonova Research Institute of Rheumatology for 8 years.MethodsDescription of clinical case of 13 y.o. male Caucasian pts presented with polyarthritis variant of juvenile idiopathic arthritis (JIA) since 2006.ResultsThe first symptoms (at the age of 1,5) were polyarthritis with fever, morning stiffness and inflammatory activity in laboratory tests in chronological link of DTP – re-vaccination. The patient admitted to the regional hospital where JIA was diagnosed and he received traditional therapy including NSAIDS, methotrexate, numerous of intraarticular injections, methylprednisolone pulse therapy with initial response. By the 2012 the child deteriorated with progressive polyarthritis, deformation of wrist joints, bilateral camptodactylia with progressive destruction on X-ray. Under the administration of abatacept therapy in our clinic in 2012 the significant improvement (70-90% ACR response) was achieved. 2 years later the plaque psoriasis developed. The cutaneous changes were impaired by the regular local application of GC so infusions of abatacept were continued. In 4th years of regular therapy (in the age of 13) sudden neurological symptoms such as headache, loss of sensitivity, ataxia, visual field defect appeared. He was admitted to neurological clinic. Multiple sclerosis was diagnosed based on the presenting signs and symptoms supporting cranial MRI – multiple focal areas of demyelination.ConclusionWe presented a patient with JIA who developed two different immunological related adverse events under long-term abatacept using: psoriasis “de novo” appeared as a native origin of juvenile arthritis and CNS – multiple sclerosis. This is the first case of developing MS under the abatacept therapy among the 1118 pts (2002-2018) who receive biological therapy in our clinic.Disclosure of InterestsNone declared

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Section: Discussionmentioning

confidence: 99%

Ab1005 the Unusual Adverse Events Including Multiple Sclerosis Developed Under Abatacept Therapy in a Patient With Jia

Latypova

Shapovalenko

Nikishina

2019

Abstracts Accepted for Publication

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mentioning

confidence: 99%

Ab1006 the Clinical Spectrum of Two Heterozygous Mutations in the MVK Gene Confirming Hyperimmunoglobulin D Syndrome

Lazarević

Vojinović

2019

Abstracts Accepted for Publication

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BackgroundAutoinflammatory syndromes represent the wide spectrum of rare diseases (associated with genetic disturbances) characterized by the presence of chronic or recurrent systemic inflammation with diverse clinical presentation.ObjectivesWe report a case report of child with confirmed two heterozygous mutations in the MVK gene with clinical features associated hyperimmunoglobulin D syndrome.ResultsWe present girl with clinical symptoms starting just after birth when in the first days of life she was examined at gastroenterology department in Belgrade due to elevated parameters of inflammation, anemia, direct hyperbilirubinaemia, abdominal bloating and hepatosplenomegalia. Breastfeeding was discontinued due to galactosuria and lactose free diet was advised. Due to the maintenance of hepatomegaly, a detailed hematological, virusological and gastroenterological diagnostic testing was performed. Since liver biopsy has showed portal and lobular hepatitis with cholestasis without fibrosis she was on ursofalk treatment with partial response. At five months of age she started to have recurrent episodes of fever every month for few days with no associated infection, but always followed with digestive symptomatology (abdominal pain and abdominal flatulence) and elevation of inflammatory parameters. Despite antibiotics, episodes of fever continued to repeat twice monthly with accompanying occurrence of hypersalivation, small ulcers in the mouth, skin rash, cervical lymphadenopathy, hepatosplenomegaly, abdominal pain and abdominal bloating with elevated inflammatory markers. One year later she was admitted for the first time at our department and detailed differential diagnostic testing was performed. Clinical spectrum of presenting symptoms with specific phenotypic aspect (hypertelorismus and frontal bossing) were enough to suspect on autoinflammatory disease background (hyper IgD syndrome), but without IgG increase. Genetic testing have revealed presence of two heterozygous mutations ( heterozygous variant c.790del p.(Leu264Serfs*2) and heterozygous variant c.1129G>A p.(Val377Ile) in the mevalonate kinase deficiency gene (MVK gene) confirming hyperimmunoglobulin D syndrome. Introduction of nonsteroidal antiinflammatory drug and corticosteroids have led to complete or partial remission during a fever episodes. As fever episodes continued on every two weeks we are about to start with donated fully-humanized protein (canakinumab), highly efficacious in fever episodes resolution by blocking interleukin-1 beta cytokine.ConclusionDiverse clinical manifestations of some patients with autoinflammatory diseases can provoke differential diagnostic and treatment dilemmas. Genetic testing is of great importance for establishing the final diagnosis and starting accurate treatment in order to prevent potential serious complications seen in these patients. Disclosure of InterestsNone declared

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Ab1007 prevalence and Characteristics of Temporo-Mandibular Joint (Tmj) Involvement in a Cohort of Young Adult Patients With Diagnosis of Juvenile Idiopathic Arthritis (Jia) and Non-Jia Chronic Inflammatory Arthropaties

Cited by 2 publications

References 1 publication

Ab1005 the Unusual Adverse Events Including Multiple Sclerosis Developed Under Abatacept Therapy in a Patient With Jia

Ab1005 the Unusual Adverse Events Including Multiple Sclerosis Developed Under Abatacept Therapy in a Patient With Jia

Ab1006 the Clinical Spectrum of Two Heterozygous Mutations in the MVK Gene Confirming Hyperimmunoglobulin D Syndrome

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