Axial involvement in enthesitis-related arthritis: results from a single-center cohort.

BACKGROUND: Axial involvement in children with enthesitis-related arthritis (ERA) has characteristics that differ from those of peripheral involvement. This study characterized their clinical characteristics and treatment. METHODS: Patients with ERA at the Children's Hospital of Nanjing Medical University between January 2018 and December 2020 were included. The ERA cohort was divided into two based on the presence or absence of axial joint involvement. Demographic characteristics, clinical features, and treatments were described and compared. RESULTS: In total, 105 children with ERA were enrolled (axial ERA, n = 57; peripheral ERA, n = 48). The age at disease onset of the axial group tended to be higher (11.93 ± 1.72 vs. 11.09 ± 1.91 years) and the diagnosis delay was bigger in patients with axial ERA (10.26 ± 11.66 months vs. 5.13 ± 7.92 months). The inflammatory marker levels were significantly higher in patients with axial. There were no differences in HLA-B27 positivity between the groups (34 [59.65%] vs. 28 [58.33%], P > 0.05). Hip involvement was more frequent in the axial group (52.63% vs 27.08%; X2 = 7.033). A total of 38 (66.67%) and 10 (20.83%) patients with axial and peripheral ERA, respectively, were treated with biological disease-modifying anti-rheumatic drugs (DMARDs) at diagnosis. The administration of biologics increased gradually in the axial ERA group, peaking at 18 months and decreasing thereafter, whereas that in the peripheral ERA group peaked at 6 months and began to decline thereafter. CONCLUSIONS: Axial ERA is a persistent active disease and requires a more aggressive treatment. Classification and early recognition of axial involvement may help with timely diagnosis and appropriate management.

Performance of recent PRINTO criteria versus current ILAR criteria for systemic juvenile idiopathic arthritis: A single-centre experience.

OBJECTIVES: The purpose of this study is to evaluate the performances of recently proposed Pediatric Rheumatology International Trials Organization criteria versus current International League of Associations for Rheumatology criteria for systemic juvenile idiopathic arthritis (sJIA). METHODS: The study was performed at the Department of Pediatric Rheumatology in Istanbul Faculty of Medicine with a retrospective design, covering the date range 2010-2021. Patients diagnosed with sJIA, Kawasaki disease and common autoinflammatory diseases were included. Both the International League of Associations for Rheumatology and Pediatric Rheumatology International Trials Organization classification criteria were applied to each patient and cross-checked with expert rheumatologist diagnosis. RESULTS: Eighty-two patients with sJIA were compared against 189 (74 Kawasaki disease, 83 familial Mediterranean fever, 16 mevalonate kinase deficiency, 10 cryopyrin-associated periodic syndromes, and 6 tumour necrosis factor receptor-associated periodic syndrome) patients. The Pediatric Rheumatology International Trials Organization criteria demonstrated higher sensitivity (62.2% vs 80.5%, P =.003) but comparable specificity (90.5% vs 91%) as regards the International League of Associations for Rheumatology criteria. CONCLUSIONS: The revised criteria appear to enhance the ability to provide early recognition and pertinent classification of sJIA. No superiority was observed in segregating sJIA from common autoinflammatory diseases and Kawasaki disease, namely in terms of specificity.

Navigating the initial diagnosis and management of adult IgA vasculitis: A review.

Background: IgA vasculitis in adults has not been thoroughly studied. This has left a practice gap related to the management and follow-up of a population that is at an increased risk of comorbidities and potentially poor outcomes. For this reason, it is important to synthesize evidence from the current literature because this can help direct the movement for more robust studies to clarify best practice recommendations. Objective: We sought to create a narrative review for the practicing dermatologist when diagnosing and leading the care of IgA vasculitis in adult patients. Methods: A broad literature search was performed with a focus on articles that were published after the introduction of the most updated European Alliance of Associations for Rheumatology/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society criteria. Results: The characteristics and management guidelines for IgA vasculitis in adults have been refined, although more rigorous studies are needed to develop best practice recommendations. Limitations: Because of the lack of sufficient randomized controlled trials on IgA vasculitis in adults, this narrative review is composed of mostly observational, descriptive studies. Conclusion: Adults with IgA vasculitis are at an increased risk of complicated disease course, necessitating formal diagnostic assessment and clear-cut follow-up recommendations to manage and prevent poor health outcomes related to various comorbidities.

Performance of the new Eurofever/PRINTO classification criteria in Familial Mediterranean fever patients with a single exon 10 mutation in childhood.

The diagnosis of Familial Mediterranean fever (FMF) based on clinical findings supported by genetic mutation. Recently, the new Eurofever/PRINTO classification criteria including genetic analysis were established. The aim of this study is to evaluate the performance of the new criteria in FMF patients with a single exon 10 mutation in childhood. The study group consisted of FMF patients who had a single exon 10 mutation in a referral center in Turkey. Patients with periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome were included as a control group. The medical charts of all patients were reviewed retrospectively. A total of 106 FMF patients (59 boys) were enrolled in the study group. The median age at first symptom was 5; the median age at diagnosis was 7 years. The mean follow-up was 33 ± 35.4 months. Majority of the patients (n = 58, 54.7%) had heterozygous M694V, 16 (15%) patients had M694V/E148Q and 13 (13.8%) patients had heterozygous M680I mutation. The sensitivity of the Yalcinkaya-Ozen criteria was 98.1% and it was 97.1% for the Eurofever/PRINTO classification criteria. The specificity of the new Eurofever/PRINTO classification criteria was 96.7% and it was 74.1% for the Yalcinkaya-Ozen criteria. The new Eurofever/PRINTO classification criteria have a good sensitivity as the Yalcinkaya-Ozen criteria in patients with a single exon 10 mutation. Additionaly, the new criteria have better specificity. It should be useful to apply the clinical only criteria where the carrier rate is high.

The Challenges and Opportunities of Classifying Childhood Arthritis.

PURPOSE OF REVIEW: Childhood arthritis is in need of a new system of classification, owing to deficiencies in the International League of Associations for Rheumatology (ILAR) criteria. We briefly review the history of classification of childhood arthritis, discuss the major criticisms of the current system, and highlight current initiatives to address those concerns. RECENT FINDINGS: Recent studies in both pediatric and adult rheumatology into the biologic basis of disease as well as the clinical patterns of presentation have informed the efforts toward developing a new classification system. Several efforts are currently underway to improve the classification of childhood arthritis, most notably the project of the Pediatric Rheumatology International Trials Organization (PRINTO). This international alliance of pediatric rheumatologists has begun a 4-step process to create new classification criteria for childhood arthritis. They are currently on step 3 of the process.

Performance of the new 'Eurofever/PRINTO classification criteria' in FMF patients.

OBJECTIVE: Recently a new set of criteria proposed for the classification of auto inflammatory recurrent fevers including familial Mediterranean Fever (FMF). We aimed to compare the sensitivity and specificity of the new Eurofever/PRINTO classification criteria with those of the Tel Hashomer and Yalcinkaya-Ozen criteria. METHODS: 151 consecutive FMF patients between February and May 2019 who were followed at Hacettepe University Department of Pediatric Rheumatology were included in this study. A group of 82 patients with periodic fever 66 periodic fever, aphthosis, pharyngitis and adenitis syndrome (PFAPA), nine cryopyrin-associated periodic syndrome (CAPS) and seven mevalonate kinase deficiency/hyperimmunoglobulin D syndrome (MKD/HIDS) patients) served as controls. GraphPad 6.0 was used for statistical analysis. RESULTS: Three different classification criteria were analyzed in 151 FMF patients with a median age at diagnosis of 5 years and in 82 controls with a median age at diagnosis of 3 years. The sensitivity of the new Eurofever/PRINTO criteria (96%) was highest (Tel Hashomer criteria-88.4% and Yalcinkaya-Ozen criteria-93.4%). However, the specificity of these criteria (73.1%) was lowest (Tel Hashomer criteria-92.6% and Yalcinkaya-Ozen criteria-84.1%). The new Eurofever/PRINTO criteria achieved the highest sensitivity (100%) in biallelic exon 10 mutation patients (Tel Hashomer criteria-87.4% and Yalcinkaya-Ozen criteria-94.2%). However, the new set had the lowest sensitivity (88.2%) in heterozygote exon 10 mutation patients (Tel Hashomer criteria 94.1% and Yalcinkaya-Ozen criteria 94.1%). CONCLUSION: In this Turkish cohort, the new Eurofever/PRINTO criteria have a better sensitivity but lower specificity with higher misclassifications than other two well-known criteria. The combination of clinical manifestations with genotype increased the sensitivity. The lower specificity may be due to the high carrier rate in our population. Although the ethnicity information lowers the specificity, 'clinical-only' criteria set may still guide the clinician to perform appropriate genetic testing in patients with recurrent fever.

Cardiorespiratory fitness in long-term juvenile dermatomyositis: a controlled, cross-sectional study of active/inactive disease.

OBJECTIVES: To compare cardiorespiratory fitness (CRF) expressed as maximal oxygen uptake (VO2max) between patients with long-term JDM and controls and between patients with active and inactive disease, as well as to explore exercise limiting factors and associations between CRF and disease variables. METHODS: JDM patients (n = 45) and age- and gender-matched controls (n = 45) performed a cardiopulmonary exercise test (CPET) on a treadmill until exhaustion. Physical activity was measured by accelerometers. Disease activity, damage and muscle strength/function were assessed by validated tools. Clinically inactive disease was defined according to PRINTO criteria. RESULTS: The mean disease duration was 20.8 (s.d. 11.9) years and 29/45 (64%) patients had inactive disease. A low VO2max was found in 27% of patients vs 4% of controls (P = 0.006). The mean VO2max and maximal ventilation (VEmax) were lower in patients with active and inactive disease compared with controls. Patients with active disease also had lower maximal voluntary ventilation (MVV) compared with controls and lower VEmax and MVV compared with those with inactive disease. Patients with inactive disease had lower physical activity levels compared with controls. VO2max correlated negatively with disease damage in patients with inactive disease and positively with muscle strength/function in patients with active disease. CONCLUSION: CRF was lower in JDM patients, both with active and inactive disease, compared with controls after a mean 20 years disease duration. Cardiopulmonary exercise test results suggested different limiting factors contributing to the reduced CRF according to disease activity, including deconditioning in inactive disease and reduced ventilatory capacity in active disease. Further research is needed to verify this.

Spectrum of renal disease in HIV-infected children: report of five cases.

There is a paucity of literature on renal diseases associated with HIV infection in Asian countries. Renal disease in HIV-infected children can involve the glomerulus, interstitium, tubules or blood vessels of the kidney. In this case series, five HIV-infected children with various forms of renal disease are reported. The renal pathology included HIV-associated nephropathy, collapsing focal segmental glomerulosclerosis without tubular changes, tubule-interstitial nephritis and minimal change disease (MCD). Case five fulfilled the classification criteria for childhood polyarteritis nodosa (PAN). It is important to screen all HIV-infected children for renal disease to enable detection at an early stage.

Prevalencia y manifestaciones clínicas más frecuentes de la Púrpura de Henoch Schõnlein en la población pediátrica de la Clínica Universitaria Colombia y Clínica Reina Sofía entre los años 2009-2015 / Prevalence and most frequent clinical manifestations of the Henoch-Schõnlein Purpura in the pediatric population of the Clínica Universitaria Colombia and Clinica Reina Sofia between the years 2009-2015

Objetivo: estimar la prevalencia y determinar las manifestaciones clínicas más frecuentes de la Púrpura de Henoch-Schõnlein teniendo en cuenta los criterios EULAR-PRINTO-PReS en la población pediátrica que ingresó a la Clínica Universitaria Colombia y Clínica Reina Sofía a los servicios de urgencias y hospitalización entre los años 2009 al 2015. Método: estudio descriptivo, cohorte histórica, que se realizó en la ciudad de Bogotá, se incluyeron 109 pacientes menores de 18 años con diagnóstico de Púrpura de Henoch-Schõnlein. Resultados: Se encontró una prevalencia en el ámbito hospitalario de 2,07 casos por cada 1.000 hospitalizaciones y en el escenario de urgencias 0,2 por cada 1.000 consultas, respecto a las manifestaciones clínicas se comportó así: Purpura palpable (100 por ciento), artritis/artralgia (80 por ciento), Dolor abdominal (33 por ciento), nefropatía (6 por ciento), Depósitos IgA (0 por ciento). Conclusiones: En nuestro estudio, esta enfermedad fue mucho más prevalente en niñas, respecto a las manifestaciones clínicas de acuerdo a los criterios diagnósticos EULAR/PRINTO/Pres se comportó de manera similar a otros estudios.

Objective: estimate the prevalence and determine the most frequent clinical manifestations of Henoch-Schõnlein Purpura, taking into account the EULAR-PRINTO-PReS criteria in the pediatric population that entered the emergency and Hospitalization services in the Clinica Universitaria Colombia and Clínica Reina Sofía between the years 2009 and 2015. Method: descriptive study, historical cohort, carried out in the city of Bogotá, included 109 patients under the age of 18 years with diagnosis of Henoch-Schõnlein Purpura. Results: A prevalence of 2.07 cases per 1000 hospitalizations was found in the hospital setting and in the emergency setting 0.2 per 1000 visits, in relation to the clinical manifestations, it was: Palpable purpura (100percent), arthritis / arthralgia 80 percent, Abdominal pain (33 percent), nephropathy (6 percent), IgA deposits (0 percent). Conclusions: In our study, this disease was much more prevalent in girls, on the other hand in regard to the clinical manifestations according to diagnostic criteria of EULAR / PRINTO / Pres, our population behaved in a similar way to other studies.