Incidence of juvenile idiopathic arthritis in Finland, 2000-2020.

OBJECTIVE: Previous epidemiological data of JIA in Finland are from the turn of the millennium. We aimed to determine the recent annual incidence of JIA in several consecutive years in Finland and to explore the differences in incidence between sexes, age groups, and regions. METHODS: We analyzed all children <16 years of age who met the ILAR classification criteria for JIA. Cases from 2000-2020 were identified from two national registers: the Care Register for Health Care of the Finnish Institute for Health and Welfare and the Reimbursement Register containing medication data from the Social Insurance Institution of Finland; cases from 2016-2020 were identified from the Finnish Rheumatology Quality Register. RESULTS: The incidence of JIA was 31.7 per 100 000 (95% CI 30.2, 33.1), according to the Care Register in 2000-2020 and peaked in 2010-2014. No considerable differences in incidence rates were observed among registers. In all age groups, incidence in girls was predominant compared with boys. The incidence in girls peaked at the ages of 2 years and 14-15 years. Decreasing incidence was observed among boys 0-3 years old during the entire study period, whereas increasing incidence was observed among teenage girls and boys 4-7 years old in 2000-2013. CONCLUSION: The incidence of JIA is not only very high with respect to that in other parts of the world but also higher than previously reported in Finland. The incidence varied by region and year but was not higher at the end than the beginning of the study period.

Microbial orchestra in juvenile idiopathic arthritis: Sounds of disarray?

The role of the microbiota in multiple autoimmune diseases, including juvenile idiopathic arthritis (JIA) has earned substantial attention in the last 10 years. Increasing evidence suggests that the microbiota's link to JIA begins in early childhood, as early life events that influence the nature of the microbiota also appear to influence disease risk. In this review, we discuss these early life events including mode of delivery, infant feeding practice, antibiotics exposure, and other events and their impacts on the microbiota and on disease risk; reported abnormalities of the microbiota in children with JIA; mechanisms by which an altered microbiota at birth and later on in childhood may influence disease risk; and the prospects for therapeutic alteration of the microbiota in children with JIA.

Identification of monogenic variants in more than ten per cent of children without type 1 diabetes-related autoantibodies at diagnosis in the Finnish Pediatric Diabetes Register.

AIMS/HYPOTHESIS: Monogenic forms of diabetes (MODY, neonatal diabetes mellitus and syndromic forms) are rare, and affected individuals may be misclassified and treated suboptimally. The prevalence of type 1 diabetes is high in Finnish children but systematic screening for monogenic diabetes has not been conducted. We assessed the prevalence and clinical manifestations of monogenic diabetes in children initially registered with type 1 diabetes in the Finnish Pediatric Diabetes Register (FPDR) but who had no type 1 diabetes-related autoantibodies (AABs) or had only low-titre islet cell autoantibodies (ICAs) at diagnosis. METHODS: The FPDR, covering approximately 90% of newly diagnosed diabetic individuals aged ≤15 years in Finland starting from 2002, includes data on diabetes-associated HLA genotypes and AAB data (ICA, and autoantibodies against insulin, GAD, islet antigen 2 and zinc transporter 8) at diagnosis. A next generation sequencing gene panel including 42 genes was used to identify monogenic diabetes. We interpreted the variants in HNF1A by using the gene-specific standardised criteria and reported pathogenic and likely pathogenic findings only. For other genes, we also reported variants of unknown significance if an individual's phenotype suggested monogenic diabetes. RESULTS: Out of 6482 participants, we sequenced DNA for 152 (2.3%) testing negative for all AABs and 49 (0.8%) positive only for low-titre ICAs (ICAlow). A monogenic form of diabetes was revealed in 19 (12.5%) of the AAB-negative patients (14 [9.2%] had pathogenic or likely pathogenic variants) and two (4.1%) of the ICAlow group. None had ketoacidosis at diagnosis or carried HLA genotypes conferring high risk for type 1 diabetes. The affected genes were GCK, HNF1A, HNF4A, HNF1B, INS, KCNJ11, RFX6, LMNA and WFS1. A switch from insulin to oral medication was successful in four of five patients with variants in HNF1A, HNF4A or KCNJ11. CONCLUSIONS/INTERPRETATION: More than 10% of AAB-negative children with newly diagnosed diabetes had a genetic finding associated with monogenic diabetes. Because the genetic diagnosis can lead to major changes in treatment, we recommend referring all AAB-negative paediatric patients with diabetes for genetic testing. Low-titre ICAs in the absence of other AABs does not always indicate a diagnosis of type 1 diabetes.

Paediatric rheumatologists do not score the physician's global assessment of juvenile idiopathic arthritis disease activity in the same way.

OBJECTIVES: To assess the heterogeneity in factors affecting physician's global assessment of disease activity (PhGA) and in PhGA scoring of multiple JIA patient's case scenarios. METHODS: An electronic web-based questionnaire of factors potentially considered in PhGA was sent worldwide to members of PRINTO and the Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN). The respondents were asked to rate from 0 to 100 the relevance of 17 factors possibly affecting PhGA scoring and to derive a PhGA score of 17 detailed JIA patient cases. The median and interquartile range was used to measure the heterogeneity in the scoring. To demonstrate the consistency among the PhGA scores of the patient cases provided by multiple physicians, we assessed the inter-rater reliability using intra-class correlation. RESULTS: The questionnaire was completed by 491 respondents. A large individual variation was observed in the impact of different factors on PhGA when assessing JIA. For non-systemic JIA the presence of fever had the largest variation and swollen joint count had the smallest. For sJIA, the largest variation was seen in the presence of erosions and the smallest in the presence of fever. The intra-class correlation of the group for PhGA scoring of patient cases was 0.53 (95% CI 0.38, 0.72). CONCLUSIONS: In a sample of worldwide respondents, the scoring of the PhGA is divergent. Consensus on PhGA scoring guidelines is required to obtain a consistent assessment of patients.

Use of psychotropic medication in incident juvenile idiopathic arthritis patients from 2000 to 2014: a case-control cohort study.

OBJECTIVES: To explore the use of psychotropic medications in patients with juvenile idiopathic arthritis (JIA) compared to population controls. METHODS: Using register data from the Social Insurance Institution of Finland and the National Population Registry, we collected all incident JIA patients with index dates from 2000 to 2014 (n=4,180) and three population comparators for each case (n=12,512). For these individuals, we obtained information on their psychotropic medication from the registry on prescriptions, which includes all purchases of prescription medicines in pharmacies. The study populationwas followed from their index dates until 31 December 2015. The data were analysed using generalised linear models. RESULTS: The mean age (SD) of the JIA patients at disease onset was 8.3 (4.8) years, and 14.8 (6.4) years at the end of the follow-up period. During the follow-up years, 566 (13%) of the JIA patients had purchased some psychotropic drug from a pharmacy, whereas the number in the control group was 1,294 (10%; p<0.001). Antidepressants were the most purchased drugs in both groups. Further analysis by gender showed that females with JIA used antidepressants more often than males with JIA. CONCLUSIONS: The use of psychotropic medication, particularly antidepressants, was more common in patients with JIA compared to comparators in the general population. This reflects the presence of clinically important mental health problems in JIA patients and the need for multiprofessional collaboration in patient care.

Disease burden measured by patient-reported outcomes: does psoriatic arthritis feel worse than rheumatoid arthritis? A cross-sectional nationwide study.

OBJECTIVES: To study the subjective disease burden of patients with psoriatic arthritis (PsA) and rheumatoid arthritis (RA), using patient-reported outcomes (PROs) cross-sectionally. METHODS: Data of 3598 patients with PsA and 13913 with RA were extracted from the database. Measures included the VAS-values of pain, fatigue and patient global assessment (PGA), HAQ, and disease activity at the most recent visit/remote contact in the period 1.2020 to 9.2021. Values were compared between patients with PsA and RA overall, and by sex and age (<50, 50-59, 60-69 and ≥70 years). Regression analyses were applied. RESULTS: The overall median (IQR)-values for pain were 29 (10, 56) for PsA and 26 (10, 51) for RA, 29 (9, 60) and 28 (8, 54) for fatigue, 28 (10, 52) and 29 (11, 51) for PGA, 0.4 (0, 0.9) and 0.5 (0, 1.0) for HAQ (p<0.001 for all comparisons; adjusted for sex and age). The median (IQR)-values for pain, fatigue, PGA and HAQ were higher for PsA vs. RA in most age groups for males and females. All PROs were higher in older patients with both diagnoses. The median values for DAS28, doctor global assessment, ESR and CRP were 1.9 vs. 2.0, 8 vs. 8, 7 vs. 8 and 2 vs. 3 in PsA and RA, respectively. CONCLUSIONS: Overall, both PsA and RA groups showed moderate disease control by patients' perspective, but the burden of disease was higher especially in women with PsA compared to RA. Disease activity was similar and low in both diseases.

Heterogeneity in the presentation of clinical type 1 diabetes defined by the level of risk conferred by human leukocyte antigen class II genotypes.

OBJECTIVES: The association between human leukocyte antigen (HLA) class II genotypes and susceptibility to type 1 diabetes (T1D) is well established. This study aimed at examining whether there are differences in the presentation of T1D depending on the HLA genotype. RESEARCH DESIGN AND METHODS: We divided the study participants (N = 5798) in the Finnish Pediatric Diabetes Register into two groups based on the T1D risk conferred by their HLA genotype (high and moderate-risk genotypes, Group 1 vs. other genotypes, Group 2). We then examined differences in clinical, metabolic, and immunological characteristics. Children included in the study were 0-14-year-old and diagnosed between January 2003 and December 2019. RESULTS: Participants in Group 1 were younger at the time of diagnosis (P < 0.001) and had more frequently family members affected by T1D (P < 0.001). Diabetic ketoacidosis (DKA) was more frequent among participants in Group 2 (P = 0.014) who also had a longer duration of symptoms before diagnosis (P < 0.001) and higher hemoglobin A1c (P = 0.001) at diagnosis. The HLA genotype was not, however, directly related to the DKA frequency. The frequency of islet cell antibodies (P < 0.003), insulin autoantibodies (P < 0.001), and islet antigen 2 autoantibodies (P < 0.001) was higher in Group 1 whereas glutamic acid decarboxylase autoantibodies were more frequent (P < 0.001) in Group 2. Group 1 had more participants with multiple autoantibodies (P = 0.027) whereas antibody negativity was more frequent in Group 2 (P = 0.003). CONCLUSIONS: These findings indicate disease heterogeneity in relation to both clinical disease presentation and humoral autoimmunity, in particular. This heterogeneity is, at least partly, defined by HLA Class II genotypes.

A Family With A20 Haploinsufficiency Presenting With Novel Clinical Manifestations and Challenges for Treatment.

BACKGROUND: Tumor necrosis factor α-induced protein 3 gene (TNFAIP3, also called A20) haploinsufficiency (HA20) leads to autoinflammation and autoimmunity. We have recently shown that a p.(Lys91*) mutation in A20 disrupts nuclear factor κB signaling, impairs protein-protein interactions of A20, and leads to inflammasome activation. METHODS: We now describe the clinical presentations and drug responses in a family with HA20 p.(Lys91*) mutation, consistent with our previously reported diverse immunological and functional findings. RESULTS: We report for the first time that inflammasome-mediated autoinflammatory lung reaction caused by HA20 can be treated with interleukin 1 antagonist anakinra. We also describe severe anemia related to HA20 successfully treated with mycophenolate. In addition, HA20 p.(Lys91*) was found to associate with autoimmune thyroid disease, juvenile idiopathic arthritis, psoriasis, liver disease, and immunodeficiency presenting with specific antibody deficiency and genital papillomatosis. CONCLUSIONS: We conclude that HA20 may lead to combination of inflammation, immunodeficiency, and autoimmunity. The condition may present with variable and unpredictable symptoms with atypical treatment responses.

Efficacy and safety of tocilizumab in a real-life observational cohort of patients with polyarticular juvenile idiopathic arthritis.

OBJECTIVES: To evaluate the patterns of usage, efficacy and safety of tocilizumab in polyarticular JIA. METHODS: An observational study of 56 consecutive polyarticular JIA patients was conducted using patient charts and electronic JIA databases. Efficacy was assessed by tocilizumab survival, rates of low disease activity (LDA) and of inactive disease by 10-joint Juvenile Arthritis Disease Activity Score (JADAS-10), and of clinically inactive disease according to Wallace's preliminary criteria. Efficacy and rate of adverse events (AEs) were evaluated during a 24-month period after tocilizumab commencement. RESULTS: Tocilizumab was started on average as third-line biological agent (median, range first- to fourth-line) at a median disease duration of 5.2 years (interquartile range 3.0-7.7). Survival rates were 82% at 12 months and 64% at 24 months. The reasons for discontinuation were inadequate treatment effect in 50%, AE plus inadequate treatment effect in 37.5% and AE alone in 12.5%. LDA (JADAS-10 ⩽3.9) was reached in 58% at 12 months and in 84% at 24 months, inactive disease (JADAS-10 ⩽0.7) in 19% and 44%, and clinically inactive disease in 28% and 46%, respectively. The rate of AEs was 200.9/100 patient years and of serious AEs 12.9/100 patient years. CONCLUSION: Survival of tocilizumab was high and a large proportion of the treatment-resistant patients reached LDA at 12 months of treatment. The LDA rate continued to increase throughout 24 months. The rates of AEs and serious AEs were higher than in register studies but lower than in the originator study of tocilizumab.

Extended family history of type 1 diabetes in HLA-predisposed children with and without islet autoantibodies.

OBJECTIVE: The aim of this study was to explore the extended family history of type 1 diabetes in children at genetic risk and define the impact of a positive family history on the development of islet autoimmunity and type 1 diabetes. METHODS: The subjects were participants in The Finnish Type 1 Diabetes Prediction and Prevention (DIPP) study and carried increased HLA-conferred risk for type 1 diabetes. The case children (N = 343) were positive for at least one islet autoantibody, and the control children (N = 343) matched by age, gender and class II HLA genotype were negative for islet autoantibodies at the time of data collection. Extended family history of type 1 diabetes was obtained by using a structured questionnaire. RESULTS: Among children who were autoantibody positive and progressed to type 1 diabetes 62.2% (28/45) had at least one relative with type 1 diabetes. Interestingly, 57.8% of these children (26/45) had such a relative outside the nuclear family compared to 30.7% of children with no autoantibodies (P = .001), 35.2% of those with only classical islet cell antibodies (P = .006), and 35.2% of non-progressors with biochemical autoantibodies (P = 0.011). A positive history of type 1 diabetes in the paternal extended family was more common in children with multiple biochemical autoantibodies compared to those with only one biochemical autoantibody (P = .010). No association between the specificity of the first appearing autoantibody and family history of the disease was found. CONCLUSIONS: Type 1 diabetes in relatives outside the nuclear family is a significant risk factor for islet autoimmunity and progression to clinical disease in HLA susceptible children.

The mortality rate and causes of death among juvenile idiopathic arthritis patients in Finland.

OBJECTIVES: To explore mortality rates and causes of death in juvenile idiopathic arthritis (JIA) patients in Finland compared with the general population. METHODS: All incident patients with JIA (age <16 years at the index day) during 2000-2014 were collected from the nationwide register maintained by the Social Insurance Institution of Finland and The National Population Registry identified three age-, sex- and residence-matched controls for each case. They were followed up together until 31st Dec 2015. RESULTS: Altogether 4,180 JIA patients (62% females) were identified. Mean age at diagnosis was 8.3 years. The average follow-up time was 6.6 years (IQR 3.1-10.5). The patients were compared with 12,511 controls. During 28,941 follow-up years, 11 JIA patients (6 females, 5 males) and 23 controls (12 females, 11 males) died. The mean age at death was 20.3 (range: 11-30) in JIA patients and 23.1 (range: 9-29) years in the control group, (p=0.17). Cumulative mortality in JIA was 0.6% (95% Cl 0.3-1.2) compared to 0.6% (95% Cl 0.4-1.0) in the controls; (hazard ratio 1.44, 95% Cl 0.70-2.95). Accidents were the most common (54%) cause of death in JIA, whereas suicide (39%) in the controls. Substance abuse and depression contributed more to deaths in the controls (39%) than in the JIA patients (10%), (p=0.053). CONCLUSIONS: The mortality rate was not elevated in patients with juvenile idiopathic arthritis.

The Finnish version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR).

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Finnish language. The reading comprehension of the questionnaire was tested in ten JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity). A total of 173 JIA patients (1.2% systemic, 46.2% oligoarticular, 39.9% RF-negative polyarthritis, 12.7% other categories) and 100 healthy children, were enrolled in five paediatric rheumatology centres. The JAMAR components discriminated well healthy subjects from JIA patients. All JAMAR components revealed good psychometric performances. In conclusion, the Finnish version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Characterisation of rapid progressors to type 1 diabetes among children with HLA-conferred disease susceptibility.

AIMS/HYPOTHESIS: In this study, we aimed to characterise rapid progressors to type 1 diabetes among children recruited from the general population, on the basis of HLA-conferred disease susceptibility. METHODS: We monitored 7410 HLA-predisposed children participating in the Finnish Type 1 Diabetes Prediction and Prevention (DIPP) study for the development of beta cell autoimmunity and type 1 diabetes from birth over a median follow-up time of 16.2 years (range 0.9-21.1 years). Islet cell antibodies (ICA) and autoantibodies to insulin (IAA), GAD (GADA) and islet antigen 2 (IA-2A) were assessed as markers of beta cell autoimmunity. Rapid progression was defined as progression to clinical type 1 diabetes within 1.5 years of autoantibody seroconversion. We analysed the association between rapid progression and demographic and autoantibody characteristics as well as genetic markers, including 25 non-HLA SNPs predisposing to type 1 diabetes. RESULTS: Altogether, 1550 children (21%) tested positive for at least one diabetes-associated autoantibody in at least two samples, and 248 (16%) of seroconverters progressed to type 1 diabetes by the end of 2015. The median time from seroconversion to diagnosis was 0.51 years in rapid progressors (n = 42, 17%) and 5.4 years in slower progressors. Rapid progression was observed both among young (<5 years) and early pubertal children (>7 years), resulting in a double-peak distribution of seroconversion age. Compared with slower progressors, rapid progressors had a higher frequency of positivity for multiple (≥2) autoantibodies and had higher titres of ICA, IAA and IA-2A at seroconversion, and there was a higher prevalence of the secretor genotype in the FUT2 gene among those carrying the high-risk HLA genotype. Compared with autoantibody-positive non-progressors, rapid progressors were younger, were more likely to carry the high-risk HLA genotype and a predisposing SNP in the PTPN22 gene, had higher frequency of ICA, IAA, GADA and IA-2A positivity and multipositivity, and had higher titres of all four autoantibodies at seroconversion. CONCLUSIONS/INTERPRETATION: At seroconversion, individuals with rapid progression to type 1 diabetes were characterised by a younger age, higher autoantibody titres, positivity for multiple autoantibodies and higher prevalence of a FUT2 SNP. The double-peak profile for seroconversion age among the rapid progressors demonstrates for the first time that rapid progression may take place not only in young children but also in children in early puberty. Rapid progressors might benefit from careful clinical follow-up and early preventive measures.

Cow's Milk Allergy in Infancy and Later Development of Juvenile Idiopathic Arthritis: A Register-Based Case-Control Study.

We examined the association between cow's milk allergy (CMA) and juvenile idiopathic arthritis (JIA). The material for this case-control study was collected from national registers of all children born in Finland between 2000 and 2010 and diagnosed with JIA (n = 1,298) and age-, sex-, and place-matched controls (n = 5,179). We identified 235 children with CMA; 66 of these children also had JIA. A conditional logistic regression analysis was performed to evaluate the association between CMA and JIA and to test whether exposure to antibiotics would be a covariate for this association. In boys (but not in girls), a diagnosis of CMA and the use of hypoallergenic formula in infancy were associated with the later development of JIA (odds ratio = 2.4, 95% confidence interval: 1.6, 3.6). The association was most evident in boys who were diagnosed with JIA before age 3 years or diagnosed with CMA with predominantly gastrointestinal symptoms. There was no statistically significant additive interaction between CMA and antibiotic exposure in the later development of JIA. These associations may reflect impaired maturation of intestinal immunity and integrity in boys with a risk of JIA. Predisposing factors related to JIA pathogenesis seem to display a sex-linked disparity.

Finding specific 10-joint Juvenile Arthritis Disease Activity Score (JADAS10) and clinical JADAS10 cut-off values for disease activity levels in non-systemic juvenile idiopathic arthritis: a Finnish multicentre study.

OBJECTIVES: To establish the cut-off values for inactive disease, as well as low disease activity (LDA), moderate disease activity (MDA) and high disease activity (HDA) in non-systemic JIA based on the Juvenile Arthritis Disease Activity Score (JADAS) and assessed with the 10-joint JADAS (JADAS10) and clinical JADAS10 (cJADAS10). METHODS: In a multicentre cross-sectional study consisting of ∼20% of all patients with JIA in Finland (n = 509), we obtained data on their most recent registered visits between January 2013 and January 2014. We calculated the JADAS10 and cJADAS10 and established the cut-off values of both of these scores using two different receiver operating characteristics-based statistical methods. RESULTS: Of the 509 patients studied, 65.8% were females and 53.8% had polyarticular disease. The most suitable method for determining cut-off values was the Youden index. In oligoarticular patients, a JADAS10 score of 0-0.5 represented inactive disease, 0.6-2.7 LDA and ≥2.8 MDA. In polyarticular disease, a JADAS10 score of 0-0.7 represented inactive disease, 0.8-3.9 LDA and ≥4.0 MDA. The cut-off values for HDA were not possible to establish because only two visits fulfilled HDA criteria. CONCLUSION: We established cut-off values for LDA and MDA. A reliable definition for HDA will require more patients. In the clinical setting, both the cJADAS10 and JADAS10 serve equally well both for research and quality control purposes. In the future, uniform clinical disease activity levels should be established. We also suggest revising and validating the criteria for HDA. Valid and robust cut-off values for disease activity levels can guide both clinicians and researchers and equip them for quality control.

[Chronic nonbacterial osteomyelitis]. / Krooninen-ei-bakteeriperäinen osteomyeliitti.

Chronic nonbacterial osteomyelitis is an autoinflammatory disease occurring mainly in children and adolescents, typically involving recurrent or persistent osteitic foci. The symptom is bone pain, possibly accompanied by soft tissue tenderness. Some patients exhibit symptoms of systemic inflammation. The. precise etiology of the disease is not known, but an imbalance of inflammatory and anti-inflammatory cytokines is presumed to play a role in the development of the disease. While an anti-inflammatory analgesic is in most cases sufficient to calm down the osteitis, the use of corticosteroids, anti- TNF-a inhibitors or bisphosphonates is required in some cases.

Occurrence of adverse events in patients with JIA receiving biologic agents: long-term follow-up in a real-life setting.

OBJECTIVE: The aim of this study was to carry out a safety evaluation of biologic agents in patients with JIA and associated uveitis. METHODS: In three tertiary centres in Finland, all adverse events (AEs) in 348 consecutive patients were collected. AEs were classified according to the Common Terminology Criteria for AEs. RESULTS: A total of 1516 patient-years (py) were included: 710 on etanercept, 591 on infliximab, 188 on adalimumab, 8 on rituximab, 5 on anakinra, 6 on tocilizumab, 6 on abatacept and 1 on golimumab. The median follow-up of an individual patient was 51 months (range 1-155). The most common of the 2902 AEs (191/100 py) observed were mild infections, infusion or injection site reactions and alanine aminotransferase elevations. At least one AE occurred in 319 (92%) patients and 121 (35%) had at least one serious AE (SAE). The rate of SAEs was 11.4/100 py on etanercept, 11.8 on infliximab, 10.1 on adalimumab, 15.7 on abatacept, 31.2 on tocilizumab and 87.5 on rituximab, higher than with most anti-TNF agents (P = 0.005). No cases of malignant neoplasms or tuberculosis were detected. New-onset uveitis occurred in 9 patients, psoriasis or psoriasiform lesions in 13 and IBD in 6. CONCLUSION: Mild and moderate AEs in patients with JIA treated with biologics were more frequent than previously reported. SAEs were observed in one-third of the patients, but SAEs seldom led to drug discontinuation.

[Still's disease--juvenile arthritis with systemic onset]. / Stillin tauti--yleisoireinen lastenreuma.

Systemic onset juvenile idiopathic arthritis is a rare form of juvenile arthritis in which, contrary to autoimmune diseases in general, no association with a certain tissue type has been detected. Together with this fact, the lack of autoantibodies and the general symptoms belonging to the diagnostic criteria of the illness such as high fever, rather speak for its classification into autoinflammatory diseases. Treatment is usually started with anti-inflammatory drugs, often requiring combination with a systemic glucocorticoid. Recognition of interleukins 1 and 6 as central mediators in the pathogenesis of the disease has brought new possibilities for its treatment.

Similar levels of disease activity and remission rates in patients with psoriatic arthritis and rheumatoid arthritis-results from the Finnish quality register.

OBJECTIVES: To compare the current disease activity and remission rates, and their regional variation in patients with psoriatic arthritis (PsA) and rheumatoid arthritis (RA) in Finland. METHODS: Data of patients' most recent visit in 1/2020-9/2021 were extracted from the Finnish Rheumatology Quality Register. Measures for disease activity and remission included joint counts, DAS28, cDAPSA, CDAI, the Boolean definition, and physician assessment. Regression analyses were applied, adjusted for age and sex. RESULTS: Data of 3598 patients with PsA (51% female, mean age 54 years) and 13,913 patients with RA (72% female, 74% ACPA-positive, mean age 62 years) were included. The median (IQR) DAS28 was 1.9 (1.4, 2.6) in PsA and 2.0 (1.6, 2.7) in RA (p = 0.94); for cDAPSA, the median (IQR) values were 7.7 (3.1, 14) in PsA and 7.7 (3.3, 14) in RA (p < 0.001). In all regions in both diseases, the median DAS28 was ≤ 2.6 and the median cDAPSA < 13. Remission rates included DAS28 < 2.6 in 73% in PsA and 69% in RA (p = 0.17) and Boolean remission in 17% in PsA and 15% in RA (p < 0.001). By other definitions of remission, the rates ranged between 30% and 46%. Methotrexate was currently used by 49% in PsA and 57% in RA (p < 0.001). Self-administered bDMARDs were currently used by 37% in PsA and 21% in RA (p < 0.001). CONCLUSION: The overall disease activity was low and similar in patients with PsA and RA across the country. Remission rates varied between 15 and 73%, depending on the definition but were similar in PsA and RA. Key Points • The disease activity and clinical picture was similar between patients with PsA and RA, in a cross-sectional setting in 1.2020-9.2021. • A significant majority of patients with PsA had low disease activity or were in remission according to cDAPSA. Majority of patients with RA were in remission according to DAS28. • Patients with PsA and RA used methotrexate similarly. The utilization of bDMARDs was more prevalent in patients with PsA.

Intense symptoms of pain are associated with poor sleep, fibromyalgia, depression and sleep apnea in patients with rheumatoid arthritis and psoriatic arthritis. A register-based study.

OBJECTIVES: To study whether poor sleep and comorbidities are associated with high symptom levels of patient-reported outcomes (PROs) pain, patient global assessment and fatigue in patients with rheumatoid arthritis (RA) and psoriatic arthritis (PsA), in a nation-wide cross-sectional setting. METHODS: Clinical data were extracted from The Finnish Rheumatology Quality Register between 1.2021 and 9.2022. Self-reported sleep was categorized as "good" (little/no difficulties) or "poor" (great difficulties/can't) sleep. Data concerning comorbidities were collected from national registers. Descriptive statistics were used. Regression analyses were applied to analyze independent associations of sleep status, comorbidities and disease activity with pain in RA and PsA, adjusting for age and sex. RESULTS: Among 13,512 patients with RA, 6052 [mean (SD) age 62 (13), 71% female] had sleep status reported; in PsA 1861/3636 [age 55 (13), 48% female]. In RA, 5072 (84%) reported good and 980 (16%) poor sleep; the corresponding numbers in PsA were 1460 (78%) and 401 (22%). Median values for objective disease activity were low and similar in patients with poor sleep and good sleep in both diseases. Among patients with no swollen joints, the median values for PROs were approximately three times higher for patients with poor sleep vs. good sleep in both diagnoses (P<0.001). In regression analyses, "poor" sleep was independently associated with higher symptoms in pain [B (95%CI) 20 (18,22) in RA and 23 (19, 26) in PsA], followed by comorbid fibromyalgia, as well as depression in RA and sleep apnea in PsA. CONCLUSION: "Poor" sleep quality and comorbidities are independently associated with pain. Patient's sleep status is important to know especially in patients with severe symptoms without objective disease activity.