RESUMO
OBJECTIVE: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody-associated vasculitis. The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR)-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in pediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. METHODS: Retrospective data of pediatric patients with GPA in 20 centers from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. RESULTS: The study included 77 patients with GPA and 108 controls (immunoglobulin A vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1), and Cogan's syndrome (n = 1)) with a median age of 17.8 and 15.2 years, respectively. Of patients with GPA, constitutional symptoms (85.7%) and ear-nose-throat involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (p= 0.229 and p= 0.733, respectively). CONCLUSION: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly.
RESUMO
OBJECTIVES: Assessment of active synovitis is crucial for the management of juvenile idiopathic arthritis (JIA). We aimed to investigate the correlation of musculoskeletal ultrasound (MSUS) and clinical examination results and relate them to arthritis relapse rate. METHODS: JIA patients with questionable presence of active arthritis (Q-joints) and controls (JIA and healthy children) were recruited. MSUS of Q-joints, active joints and their inactive counterparts was performed at study entry. Standard disease activity parameters were prospectively recorded. RESULTS: Of 481 joints of 138 JIA patients, 99 joints (20.6%) of 58 patients had one or more Q-joints with 54/99 (54.5%) having MSUS features of active disease. Clinically inactive joints had lower proportion of MSUS synovitis (78/253, 30.8%) while MSUS activity was present in 114/129 (88.4%) of clinically active joints and in 2/105 (1.9%) joints of 36 healthy controls. Within the 15-month follow-up 23/99 (22%) Q-joints and 31/253 (12%) clinically inactive joints relapsed. Joints with subclinical synovitis relapsed more frequently than MSUS inactive ones (p<0.001). The relapse rate was higher in MSUS-active Q-joints (19/23, 82%) than in clinically inactive ones (16/31, 52%) with MSUS synovial hypertrophy as the main relapse predictor in multivariate analysis. Ankle and knee joints relapsed most frequently. CONCLUSIONS: Acknowledgement of joints with questionable synovitis may contribute to the assessment of disease activity in JIA. Presence of MSUS synovitis carries a clinically meaningful risk of disease recurrence in these joints. In clinical practice, our findings encourage timely MSUS assessment of the joints in question, especially in patients without any other features of active disease.
