27 research outputs found

    Severe laryngeal sarcoidosis in a child managed by intralesional steroid, debulking, and methotrexate

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    AbstractLaryngeal sarcoidosis is a rare condition, particularly in the pediatric population. Acute airway intervention may be required in severe cases, but guidelines to support the therapeutic strategy are absent. Moreover, evidence regarding systemic therapy is scarce. This encouraged us to report the result of airway management and systemic methotrexate at a one-year follow-up in a child with severe laryngeal sarcoidosis. A 14-year-old Caucasian female presented with dyspnea, dysphagia, and hoarseness. Fiberoptic laryngoscopy revealed profound swelling of the epiglottis and the arytenoid regions. Laryngeal sarcoidosis was suspected, and direct laryngoscopy, including biopsies, cold steel debulking, and intralesional corticosteroid injections, was performed to secure the airway. Histology was compatible with sarcoidosis, and long-term therapy with methotrexate was prescribed. At one-year follow-up, the patient was asymptomatic, and fiberoptic laryngoscopy demonstrated a near complete remission

    Long‐term outcomes in juvenile idiopathic arthritis : Eighteen Years of Follow-Up in the Population-Based Nordic Juvenile Idiopathic Arthritis Cohort

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    Objective The present study was undertaken to assess the long-term course, remission rate, and disease burden in juvenile idiopathic arthritis (JIA) 18 years after disease onset in a population-based setting from the early biologic era. Methods A total of 510 consecutive cases of JIA with disease onset between 1997 and 2000 from defined geographic regions in Denmark, Norway, Sweden, and Finland were prospectively included in this 18-year cohort study. At the follow-up visit, patient-reported demographic and clinical data were collected. Results The study included 434 (85%) of the 510 eligible JIA participants. The mean +/- SD age was 24.0 +/- 4.4 years. The median juvenile arthritis disease activity score in 71 joints (JADAS-71) was 1.5 (interquartile range [IQR] 0-5), with the enthesitis-related arthritis (ERA) category of JIA having the highest median score (4.5 [IQR 1.5-8.5], P = 0.003). In this cohort, 46% of patients still had active disease, and 66 (15%) were treated with synthetic disease-modifying antirheumatic drugs and 84 (19%) with biologics. Inactive disease indicated by a JADAS-71 score of Conclusion A substantial proportion of the JIA cohort did not achieve CR despite new treatment options during the study period. The ERA category showed the worst outcomes, and in general there is still a high burden of disease in adulthood for JIA.Peer reviewe
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