Comparison of EULAR/PRINTO/PReS Ankara 2008 and 2022 ACR/EULAR classification criteria for granulomatosis with polyangiitis in children.

OBJECTIVE: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 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 paediatric 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 paediatric patients with GPA in 20 centres 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 [IgA 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. Among patients with GPA, constitutional symptoms (85.7%) and ENT 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.

Utility of pan-immune-inflammation value as a predictor of the prognosis of childhood lupus.

BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic inflammatory multisystemic disease. Monitoring disease activity thoughtout the disease course is important for effective management and assessment of disease outcome. OBJECTIVE: To assess whether the pan-immune inflammation value (PIIV) at diagnosis could predict organ involvement and disease activity in childhood SLE (cSLE) patients after 12 months of disease onst. METHODS: This is an observational retrospective multicenter study that comprised cSLE patients seen and followed at the participating centers between January 2010 and December 2022. All patients met the EULAR/ACR-19 criteria, were immunosuppressive drug-naïve at the time of SLE diagnosis and had a minimal follow-up period of 12 months. The data included clinical and laboratory findings and disease activity using the SLEDAI-2K. Receiver operating characteristic (ROC) curves were employed to determine the optimal cut-off value of PIIV and assess its predictive potential for disease activity, and organ involvement. RESULTS: A total of 125 patients (104 female) with a median age of 16.0 (IQR 5.6) years, a median age at disease onset of 10.9 (IQR 3.0) years, and a median disease duration of 4.8 (IQR 5.3) years were included. The most frequent involved organs at diagnosis were hematological (89.6%), musculoskeletal (68.8%), mucocutaneous (63.2%), and renal (58.4%). However, at a 12-month follow-up visit, the most frequent involved organs were renal (40.0%), hematological (39.2%), musculoskeletal (15.2%), and mucocutaneous (10.4%). The median PIIV at diagnosis was 139 (IQR 229.6), while the median SLEDAI was 12 (IQR 6.5) and 3.5 (IQR 7.0) at diagnosis and 12 months, respectively. An optimal PIIV cut-off of 250 was found to be a predicative for disease activity, with a sensitivity of 45% and a specificity of 86%. The study revealed that the PIIV successfully predicted four systems in our cohort of patients. CONCLUSION: Our work suggests the PIIV might be a reasonable predictor for organ involvement and disease activity in newly diagnosed cSLE, though further research, particularly larger studies, is required to validate these findings, especially regarding organ involvement.

The use of a tumor necrosis factor inhibitor-adalimumab-in the management of arthritis in an adolescent with HIV: A case report.

Human immunodeficiency virus (HIV) infection is associated with a myriad of musculoskeletal manifestations. Inflammatory arthritis has been described in association with HIV in both adults and children. Biologic disease-modifying anti-rheumatic drugs, particularly tumor necrosis factor inhibitors (TNFi), have been reported to manage inflammatory arthritis in adults with HIV when conventional therapy fails to control arthritis. In this report, we describe the management of arthritis and enthesitis in a 12-year-old adolescent male with HIV using the TNFi adalimumab. At the time of presentation, the patient was on highly active antiretroviral therapy for 1 year. His viral load was <40 copies/mL, and the CD4+ T-cell count was 1280 cells/mm3 . He had a positive antinuclear antibody and HLA-B27. Rheumatoid factor was negative. After screening for hepatitis B and C and latent tuberculosis, the patient was started on adalimumab. This report describes the successful control of recalcitrant arthritis and enthesitis in a pediatric patient with HIV infection using adalimumab.

Oral or Parenteral Methotrexate for the Treatment of Polyarticular Juvenile Idiopathic Arthritis.

OBJECTIVE: Subcutaneous methotrexate injections are considered to be more effective or work faster than oral methotrexate. Therefore, the extent and the kinetics of response were analyzed in juvenile idiopathic arthritis patients treated with oral versus subcutaneous methotrexate. METHODS: The BIKER databank was searched for biologics-naive juvenile idiopathic arthritis patients treated with methotrexate as initial treatment. The Juvenile Arthritis Disease Activity Score-10 defini- tion of remission and the pediatric American College of Rheumatology's response parameters were utilized as outcome criteria. RESULT: A total of 410 polyarticular juvenile idiopathic arthritis patients receiving oral methotrexate were compared to 384 patients receiving subcutaneous methotrexate. Rheumatoid factor-negative polyarthritis was the most common juvenile idiopathic arthritis category (50%/51%) in this cohort followed by extended oligoarthritis (27%/26%), polyarticular psoriatic arthritis (18%/16%), and few had rheumatoid factor-positive polyarthritis (5%/8%). The oral cohort's disease duration (2.3 ± 3.0 vs. 1.9 ± 2.7) was significantly longer (P=.04), although their age at onset and baseline were similar. Furthermore, at baseline, disease activity (Juvenile Arthritis Disease Activity Score-10 16.5 ± 7.2 vs. 14.7 ± 8.2; P = .001 due to a higher active joint count 9.0 ± 10.1 vs. 7.4 ± 7.7; P = .011) was higher in the subcutaneous cohort. The weekly methotrexate doses were comparable with 13.6 ± 5.4 mg/m2 and 13.3 ± 4.5 mg/m2, respectively. With oral/subcutaneous methotrexate, a pediatric American College of Rheumatology's 90 was achieved in 98(38.3%)/128(40.4%), while 96(38.1 %)/75(40.1%) attained Juvenile Arthritis Disease Activity Score remission after 12 months of therapy. There was no difference in the early kinetics of response according to Kaplan-Meyer analysis. Adverse events including nausea, vomiting, and increased transaminases were considerably more common after methotrexate subcutaneous administration than after oral treatment. CONCLUSION: In terms of effectiveness, but not safety, our retrospective analysis found some advan- tages of subcutaneous methotrexate. Adverse effects limit treatment continuance and thus must be considered a disadvantage. Furthermore, oral methotrexate eliminates the need for injections, which is especially essential for younger children. Controlled, randomized prospective trials in children and juvenile patients are necessary for definitive recommendations for the subcutaneous route of admin- istration of methotrexate therapy.

Performance of the EULAR/ACR 2019 classification criteria for systemic lupus erythematous in monogenic lupus.

OBJECTIVE: To evaluate the application of the EULAR/ACR-2019 criteria to monogenic lupus patients and compare its performance against the SLICC-2012 criteria. METHODS: In a multicenter retrospective cohort study, consecutive patients with monogenic lupus from three tertiary lupus clinics were enrolled. The diagnosis of monogenic lupus was based on the expert physician's opinion or fulfilling the SLICC-2012 criteria. All enrolled patients had genetic variants. A control group of sporadic childhood SLE (cSLE) and non-SLE patients, were included. A descriptive data analysis was conducted, and the EULAR/ACR-2019 and SLICC-2012 criteria were applied to both groups. RESULTS: Forty-nine patients with monogenic lupus with a median age at diagnosis of 6.0 (IQR 3.0-10.8) years and 104 controls (55 patients with cSLE and 49 non-lupus patients with a median age at diagnosis of 10.0 and 5.0 respectively) were included. Forty-four (89.8%) patients with monogenic lupus fulfilled the EULAR/ACR-2019 with a mean score of 22.3±8.9. The most frequent domains were immunologic (93.9%), musculoskeletal and renal (each 57.1%), and mucocutaneous (55.1%). Fifty-four (98.2%) cSLE patients and six (12.2%) non-lupus patients met the EULAR/ACR-2019 criteria with a mean score of 22.5±9.2 and 8.5±5.2, respectively. The sensitivity of the EULAR/ACR-2019 criteria in monogenic lupus was 89.9% (95% CI: 78.3-90.2), while the specificity was 87.6% (95% CI: 75.2-88.7). CONCLUSION: This is the first and largest cohort of monogenic lupus patients testing the performance of the 2019-EULAR/ACR criteria. It efficiently classifies monogenic lupus patients, irrespective of the underlying genetic variants. Further studies are needed before these criteria are adopted worldwide. Key Points • Typically, patients with monogenic lupus have early onset severe disease, especially with mucocutaneous manifestations and a strong family history of SLE. • Monogenic lupus is a distinctive entity and might differ from the sporadic childhood SLE. • Our study includes a large multinational cohort of monogenic lupus with heterogeneous phenotypic features and underlying genetic variants. • Our study demonstrates that the EULAR/ACR-2019 criteria efficiently classified monogenic lupus patients, irrespective of the diversity of the underlying genetic variants.

Consensus clinical approach for a newly diagnosed systemic juvenile idiopathic arthritis among members of the pediatric rheumatology Arab group.

BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is a diagnosis of exclusion. The complex nature and clinical variety of the disease, as well as the vast clinical variation of disease presentation, may lead to difficulties in disease detection and subsequent delays in treatment. AIM: To provide a consensus guidance on the management of newly diagnosed sJIA patients among pediatric rheumatologists in Arab countries. METHODS: This work was conducted in two phases. The first phase utilized an electronic survey sent through an email invitation to all pediatric rheumatologists in Arab countries. In the second phase, a Task Force of ten expert pediatric rheumatologists from Arab countries met through a series of virtual meetings. Results obtained in phase one were prioritized using a nominal group and Delphi-like techniques in phase two. RESULTS: Seven overarching principles and a set of recommendations were approved by the Task Force to form the final consensus. CONCLUSION: This is the first consensus on a clinical approach for pediatric rheumatic diseases among Arab pediatric rheumatologists. It is presented as a guidance on the clinical approach to sJIA that requires further evidence, and future updates are anticipated.