Abstract

Objective To investigate the clinical features, auxiliary examination, treatment and efficacy of human leucocyte antigen(HLA)-B27-positive enthesitis related arthritis (ERA). Methods The clinical manifestations, auxiliary examination, treatment and follow-up of HLA-B27-positive ERA diagnosed in the Children′s Hospital of Chongqing Medical University from January 2007 to August 2017 were analyzed retrospectively.SPSS 19.0 software was used for data processing. Results A total of 70 children were enrolled, including 67 males and 3 females, and the average age of onset was(10.08±2.67) years old.Clinical features: all of the 70 children had peripheral arthritis with or without enthesitis, and axial arthritis symptoms appeared in 24.3% (17/70 cases) children, and 35.7% (25/70 cases) children showed enthesitis.The onset of peripheral arthritis accounted for 87.1% (61/70 cases), and all cases showed peripheral arthritis during the course of the disease, mostly the knees.Two cases were complicated with uveitis.Forty-seven cases (67.1%) were positive in Patrick test, and 9 cases(12.9%)had sacroiliac joint tenderness.The average Juvenile Arthritis Disease Activity Score with 27 joints (JADAS27) score was 20.12±8.61.Twenty-seven cases(38.6%) had positive family history.Auxiliary examination: Erythrocyte sedimentation rate, C-reactive protein(CRP)and platelets increased in most children, and a few with leukocytosis increased slightly, nearly half of children with anemia mildly or moderately, and serum tumor necrosis factor-α(TNF-α), interleukin (IL)-6, IL-1β, IL-10 levels increased.Eighty-eight point six percent (31/35 cases) children had decreased bone mass; the positive rate of X-ray examination was 81.1%(43/53 cases), including 5 cases of sacroiliac joint disease; the positive rate of ultrasonography was 81.5%(44/54 cases); the positive rate of nuclear magnetic resonance(MRI)was 95.1%(58/61 cases), including 30 cases (42.9%) of sacroiliitis.Treatment and outcome: Nonsteroidal antiinflammatory drugs (NSAIDs), disease modifying antirheumatic drugs (DMARDs), glucocorticoids, calcium and alfacalcidol were added, and 50.0% (35/70 cases) patients had added TNF-α antagonist.The follow-up of all of the patients showed improvement in terms of clinical symptoms, laboratory tests and JADAS27 score after standard treatment. Conclusions It is difficult to diagnose but easy to misdiagnose because of the lack of specificity of ERA clinical symptoms. The improvement of imaging techniques, especially MRI, is helpful for the early diagnosis of ERA.To strengthen the understanding and management of ERA, and to grasp its clinical features, immunology and imaging features, help to make timely diagnosis and reasonable treatment, and to improve physical function and quality of life of patients, and to avoid or delay disability. Key words: Enthesitis-related arthritis; Juvenile idiopathic arthritis; Human leucocyte antigen-B27; Clinical feature; Prognosis

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