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

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

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

Long-term outcomes in juvenile idiopathic arthritis: 18 years of follow-up in the population-based Nordic Juvenile Idiopathic Arthritis (JIA) cohort.

Objectives This study assessed 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 age ± SD was 24.0 ± 4.4 years. The median juvenile arthritis disease activity (JADAS71) score was 1.5 (IQR 0‐5), with the ERA category of JIA having the highest median score, 4.5 (IQR 1.5–8.5) (P=0.003). In this cohort, 46% still had active disease, and 66 (15%) were treated with synthetic disease‐modifying anti‐rheumatic drugs and 84 (19%) with biologics. Inactive disease indicated by JADAS71 <1 was seen in 48% of participants. Clinical remission off medication (CR) was documented in 33% of the participants with high variability among the JIA categories. CR was most often seen in persistent oligoarticular and systemic arthritis and least often in ERA (P<0.001). Conclusions A high prevalence 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

Long-term outcomes in juvenile idiopathic arthritis: 18 years of follow-up in the population-based Nordic Juvenile Idiopathic Arthritis (JIA) cohort.

Objectives - This study assessed 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 age ± SD was 24.0 ± 4.4 years. The median juvenile arthritis disease activity (JADAS71) score was 1.5 (IQR 0‐5), with the ERA category of JIA having the highest median score, 4.5 (IQR 1.5–8.5) (P=0.003). In this cohort, 46% still had active disease, and 66 (15%) were treated with synthetic disease‐modifying anti‐rheumatic drugs and 84 (19%) with biologics. Inactive disease indicated by JADAS71 Conclusions - A high prevalence 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

Longterm Outcomes of Temporomandibular Joints in Juvenile Idiopathic Arthritis: 17 Years of Followup of a Nordic Juvenile Idiopathic Arthritis Cohort.

Objective. To determine the prevalence of orofacial symptoms, dysfunctions, and deformities of the temporomandibular joint (TMJ) in juvenile idiopathic arthritis (JIA) 17 years after disease onset. Methods. Drawn from a prospective, population-based Nordic JIA cohort with disease onset from 1997 to 2000, 420 consecutive cases were eligible for orofacial evaluation of TMJ involvement. The followup visit included demographic data, a standardized clinical orofacial examination, and full-face cone-beam computed tomography (CBCT). For comparison, 200 age-matched healthy controls were used. Results. Of 420 eligible participants with JIA, 265 (63%) were included (mean age 23.5 ± 4.2 yrs) and completed a standardized clinical orofacial examination. Of these, 245 had a full-face CBCT performed. At least 1 orofacial symptom was reported by 33%. Compared to controls, the JIA group significantly more often reported TMJ pain, TMJ morning stiffness, and limitation on chewing. Further, among participants reporting complaints, the number of symptoms was also higher in JIA. The mean maximal incisal opening was lower in the JIA group (p < 0.001), and TMJ pain on palpation was more frequent. Condylar deformities and/or erosions were observed in 61% as assessed by CBCT, showing bilateral changes in about 70%. Risk factors of condylar deformities were orofacial dysfunction or biologic treatment; enthesitis-related arthritis was protective. Conclusion. This study of the longterm consequences of TMJ involvement in a population-based JIA cohort reports persistence of comprehensive symptoms, dysfunctions, and damage of the TMJ into adulthood. We suggest interdisciplinary followup of JIA patients also in adulthood

Longterm Outcomes of Temporomandibular Joints in Juvenile Idiopathic Arthritis:17 Years of Followup of a Nordic Juvenile Idiopathic Arthritis Cohort

Objective - To determine the prevalence of orofacial symptoms, dysfunctions, and deformities of the temporomandibular joint (TMJ) in juvenile idiopathic arthritis (JIA) 17 years after disease onset. Methods - Drawn from a prospective, population-based Nordic JIA cohort with disease onset from 1997 to 2000, 420 consecutive cases were eligible for orofacial evaluation of TMJ involvement. The followup visit included demographic data, a standardized clinical orofacial examination, and full-face cone-beam computed tomography (CBCT). For comparison, 200 age-matched healthy controls were used. Results - Of 420 eligible participants with JIA, 265 (63%) were included (mean age 23.5 ± 4.2 yrs) and completed a standardized clinical orofacial examination. Of these, 245 had a full-face CBCT performed. At least 1 orofacial symptom was reported by 33%. Compared to controls, the JIA group significantly more often reported TMJ pain, TMJ morning stiffness, and limitation on chewing. Further, among participants reporting complaints, the number of symptoms was also higher in JIA. The mean maximal incisal opening was lower in the JIA group (p Conclusion - This study of the longterm consequences of TMJ involvement in a population-based JIA cohort reports persistence of comprehensive symptoms, dysfunctions, and damage of the TMJ into adulthood. We suggest interdisciplinary followup of JIA patients also in adulthood