61 research outputs found
A meta-analysis to estimate the “real” placebo effect in juvenile idiopathic arthritis (JIA) trials
A new approach to defining inactive disease, minimal disease activity and parent- and child-acceptable symptom state in juvenile idiopathic arthritis
Thrombotic thrombocytopenic purpura and defective apoptosis due to CASP8/10 mutations: The role of mycophenolate mofetil
Impact of involvement of individual joint groups on subdimensions of functional ability scales in juvenile idiopathic arthritis
Therapeutic approaches for the treatment of new onset and flared juvenile systemic lupus erythematosus with active renal disease: an international multicenter PRINTO study
The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders
OBJECTIVES: To test the usefulness of an extended panel of lymphocyte subsets in combination with Oliveira's diagnostic criteria for the identification of autoimmune lymphoproliferative syndrome (ALPS) in children referred to a paediatric rheumatology centre. METHODS: Patients referred from 2015 to 2018 to our rheumatology unit for an autoimmune or autoinflammatory condition were retrospectively analysed. Oliveira's required criteria [chronic lymphoproliferation and elevated double-negative T (DNT)] were applied as first screening. Flow cytometry study included double-negative CD4-CD8-TCR\u3b1\u3b2+ T lymphocytes (DNT), CD25+CD3+, HLA-DR+CD3+ T cells, B220+ T cells and CD27+ B cells. Data were analysed with a univariate logistic regression analysis, followed by a multivariate analysis. Sensitivity and specificity of the Oliveira's required criteria were calculated. RESULTS: A total of 264 patients were included in the study and classified as: (i) autoimmune diseases (n\u2009=\u200926); (ii) juvenile idiopathic arthritis (JIA) (35); (iii) monogenic systemic autoinflammatory disease (27); (iv) periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (100); (v) systemic undefined recurrent fever (45); (vi) undetermined-systemic autoinflammatory disease (14); or (vii) ALPS (17). Oliveira's required criteria displayed a sensitivity of 100% and specificity of 79%. When compared with other diseases the TCR\u3b1\u3b2+B220+ lymphocytes were significantly increased in ALPS patients. The multivariate analysis revealed five clinical/laboratory parameters positively associated to ALPS: splenomegaly, female gender, arthralgia, elevated DNT and TCR\u3b1\u3b2+B220+ lymphocytes. CONCLUSIONS: Oliveira's required criteria are useful for the early suspicion of ALPS. TCR\u3b1\u3b2+B220+ lymphocytes should be added in the diagnostic work-up of patients referred to the paediatric rheumatology unit for a suspected autoimmune or autoinflammatory condition, providing a relevant support in the early diagnosis of ALPS
Biomechanical investigation of a minimally invasive posterior spine stabilization system in comparison to the Universal Spinal System (USS)
Fatigue in patients with Juvenile Idiopathic Arthritis: relationship to perceived health, physical health, self-efficacy, and participation
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