Assessment of quality of life and physical activity in patients with oligoarticular juvenile idiopathic arthritis in remission.

The purpose of this study was to evaluate physical activity (PA) and health-related quality of life (HRQOL) in children with oligoarticular juvenile idiopathic arthritis (JIA) in remission in comparison with healthy peers and to determine the disease-related factors affecting PA levels. This study was conducted with 50 oligoarticular JIA patients in remission and 50 healthy peers between 9 and 14 years. Demographic and clinical characteristics, laboratory parameters, and treatments were noted from electronic medical records. HRQOL was assessed with the Pediatric Quality of Life Inventory (PedsQL). PA was evaluated with the Physical Activity Questionnaire for Children (PAQ-C). Oligoarticular JIA patients had significantly lower self-reported median PedsQL scores in the domains of school functioning and social functioning compared to the control group (67.5 (10) vs. 75 (25), p = 0.001 and 70 (15) vs. 85 (26.3), p < 0.001, respectively). The median PAQ-C score was 2.6 (1.1) in patients with JIA and 3 (0.9) in their healthy peers (p = 0.02). The PAQ-C score was 2.8 (1.2) in patients < 8 years at the disease onset and 2.3 (1) in those aged ≥ 8 years (p = 0.022). There was no significant difference in the number of affected joints, type of affected joint, MTX and biologic agent treatment, and remission with or without drugs with the total score of the PedsQL and PAQ-C. All PedsQL domains were positively correlated with the PAQ-C.  Conclusion: Oligoarticular JIA patients demonstrated lower PA and HRQOL scores compared to healthy controls despite favorable disease control. What is Known: • Oligoarticular JIA has fewer functional limitations and disabilities compared to other JIA subtypes. • As JIA can affect all aspects of a child's life, there is a need to improve the quality of life related to the disease. What is New: • It should be considered that patients with oligoarticular JIA may show lower PA and HRQOL scores compared to healthy controls despite favorable disease control. • Since there may be a relationship between PA and HRQOL, factors that may affect PA should be investigated to provide a holistic approach to JIA treatment.

When should the use of biological agents be considered in persistent oligoarticular juvenile idiopathic arthritis patients?

The purpose of this study was to compare the demographic and clinical characteristics of the groups with and without bDMARDs added to the treatment of persistent oligoarticular juvenile idiopathic arthritis (JIA) patients on methotrexate (MTX) and also to determine the predictors of adding bDMARDs to treatment. This study included 86 oligoarticular JIA patients on MTX. Patients were divided into two groups receiving MTX (n = 69) and MTX plus bDMARD (n = 17). Predictors of adding bDMARDs were investigated by comparing demographic, clinical features and laboratory findings. Gender, age at diagnosis, time elapsed from the onset of symptoms to diagnosis, and disease duration, the number and distribution of affected joint at the time of diagnosis were similar in both groups. The mean JADAS10 at the time of diagnosis were 18.8 ± 4.2 and 19.5 ± 6.4 in the MTX and MTX plus bDMARDs groups, respectively (p = 0.68). JADAS10 at 3rd and 6th month were significantly higher in patients on MTX plus bDMARDs (p = 0.001, p = 0.004, respectively). In multivariate analysis, the risk of adding bDMARD was shown to increase 1.24-fold (p = 0.004, 95% CI: 1.07-1.43) for each point increase on the JADAS 10 at 3rd months. The number (p = 0.64) or type (p = 0.18) of joint involvement at disease onset were not predictors of adding a bDMARD. CONCLUSION: JADAS10 indicating ongoing severe disease activity at 3rd and 6th months rather than baseline JADAS10 is associated with the addition of bDMARDs. WHAT IS KNOWN: • Oligoarticular JIA patients have the best outcomes among JIA categories and respond favorably to first-line therapies such as non-steroidal anti-inflammatory drugs and intraarticular corticosteroid injections. • Clinically inactive disease rates have increased with the widespread use of biological agents in oligoarticular JIA patients who have not responded to initial therapies. WHAT IS NEW: • Approximately one-fifth of patients with persistent oligoarticular JIA on methotrexate may require the addition of a biological disease modifying anti-rheumatic drug during follow-up. • The JADAS10 calculated at 3 and 6 months is a valuable tool to identify patients who should be added biological disease modifying anti-rheumatic drugs in persistent oligoarticular JIA.

Sacroiliitis in familial Mediterranean fever: A rare joint involvement of the disease.

AIM: Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterised by recurrent episodes of fever and polyserositis. Sacroiliac joint involvement is rare in FMF patients. The purpose of this study was to evaluate the demographic, clinical, laboratory and imaging findings of patients with FMF who developed sacroiliitis. METHODS: The files of paediatric patients aged 0-18 years who were followed up with a diagnosis of FMF were retrospectively reviewed. FMF patients with evidence of sacroiliitis on magnetic resonance imaging (MRI) were included in the study. RESULTS: Among 1062 FMF patients, 22 (12 males; median age 8.5) (2.1%) of them were found to have sacroiliitis. FMF was diagnosed before sacroiliitis in nine (40.9%) patients and after in 13 (59.1%) patients. The most common symptom in patients with sacroiliitis was low back pain (n = 21, 95.5%). In MEFV gene analysis, M694V was found in 16 (72.7%) patients and was the most common mutation. MRI showed evidence of sacroiliitis in all patients. All patients were using colchicine. Patients with FMF-associated sacroiliitis, remission was achieved with non-steroidal anti-inflammatory drugs in 12 (54.5%), conventional disease-modifying antirheumatic drugs in six (27.3%) and tumour necrosis factor inhibitor treatment in four (31.8%). Four (31.8%) patients experienced sacroiliitis when colchicine incompatible and four (31.8%) patients experienced sacroiliitis while using biologic agents for colchicine-resistant FMF. CONCLUSIONS: FMF-associated sacroiliitis should be considered especially in patients with M694V mutation if they have symptoms such as low back pain. Colchicine-resistant FMF patients should be evaluated for sacroiliitis symptoms at each visit.

Chronic Nonbacterial Osteomyelitis Associated With Familial Mediterranean Fever in Children.

OBJECTIVE: Chronic nonbacterial osteomyelitis (CNO) is a noninfectious autoinflammatory bone condition that frequently occurs alongside other inflammatory diseases, such as familial Mediterranean fever (FMF). We aimed to determine the demographic, clinical, laboratory, and radiological characteristics of patients diagnosed with both FMF and CNO. METHODS: We reviewed the medical records of pediatric patients with both CNO and FMF at 3 pediatric rheumatology centers in Turkey from December 2008 to 2022. Patients' demographics, laboratory features, imaging findings, and treatment were recorded. RESULTS: Twelve patients with FMF and CNO were included in the study. Half of them were female. The mean ages at onset for FMF and CNO symptoms were 80 and 116 months, whereas the ages at diagnosis for FMF and CNO were 100 and 125 months, respectively. Ten patients (83.3%) had M694V mutation on at least 1 allele of the Mediterranean fever (MEFV) gene. The most common sites of osteitis were the long bones (58.3%), pelvis (50%), and clavicles (25%). Ten patients (83%) received nonsteroidal anti-inflammatory drugs; 8 (66%) received disease-modifying antirheumatic drugs; biological therapy was administered to 5 patients (41%), who did not respond to these treatments; and all patients received colchicine. CONCLUSION: The increased frequency of FMF in patients with CNO is of interest. Because most patients with CNO and FMF carried a homozygous or combined heterozygous M694V mutation, we speculated that the M694V mutation may play a role in the development of osteitis. Further studies are needed to elucidate the link between FMF and CNO.

Assessment of quality of life, school performance, and physical activity in adolescents with Familial Mediterranean fever.

OBJECTIVES: The aim of this study is to evaluate differences in school performance, school attendance, quality of life, and physical activity in adolescents with Familial Mediterranean fever (FMF) compared to healthy controls. METHODS: One hundred and twenty-nine patients with FMF and 154 healthy controls between 13 and 18 years were included in the study. Demographic, school performance (according to grade point average), school absenteeism, and type and frequency of exercise were recorded. Quality of life was evaluated with the Pediatric Quality of Life Inventory (PedsQL) 4.0. RESULTS: The mean age of FMF patients was 15.1 ± 2.7 years, and 69 patients (53.5%) were female. School performance was significantly higher in the control group compared to FMF patients (P < 0.001). In the control group, there were significantly higher participants who engaged in professional sports (P < 0.001). Patients with FMF had significantly lower self-reported PedsQL scores in school functioning, physical, and psychosocial health domains compared to those in the control group (P = 0.001, P < 0.001, and P = 0.028, respectively). CONCLUSIONS: FMF patients demonstrated lower school performance and quality-of-life scores compared to healthy controls. In addition to improving symptoms in chronic diseases, it is important to evaluate and improve the quality of life of patients in routine practice and to ensure psychosocial well-being.

Predictors of disease course in systemic juvenile idiopathic arthritis.

OBJECTIVES: The aim of this study was to identify potential predictors of the disease course of systemic juvenile idiopathic arthritis (sJIA) at the time of diagnosis. METHODS: This retrospective observational study was conducted in patients diagnosed with sJIA in our hospital between April 2009 and October 2023. The relationship between the disease course of sJIA patients and demographic, clinical, laboratory findings and complications was analysed. RESULTS: Of the 51 patients diagnosed with sJIA, 26 (51%) patients had monocyclic, 7 (13.7%) polycyclic, and 18 (35.2%) persistent disease course. The presence of arthritis, polyarticular involvement, and hip involvement at the time of diagnosis were associated with persistent disease course (P = .009, P = .003, P = .003). Serositis and higher white blood cell and neutrophil counts at the time of diagnosis were associated with a monocyclic disease course (P = .034, .002, .008). However, no significant correlation was found between macrophage activation syndrome and disease course (P = 1). CONCLUSIONS: sJIA patients with polyarthritis and hip involvement at disease onset may develop a persistent course. Although macrophage activation syndrome is an important complication of sJIA, its effect on the course of the disease was not found in this study.

Predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis in remission off medication.

The aim of this study was to evaluate the predictors of relapse in patients with oligoarticular juvenile idiopathic arthritis (oJIA) who achieved clinical remission off medication. This retrospective observational study was conducted between June 2009 and July 2022 in 126 patients with oJIA who achieved remission off medication. The relationships between relapse status and demographic, clinical and laboratory findings, and treatment details were evaluated using electronic medical records. Of the 126 oJIA patients who achieved remission off medication, 85 (67.5%) were female. Relapse occurred in 31 patients (24.6%) with remission off medication after a median of 18 months (IQR 7-26). No statistically significant relationship was found between gender, age at diagnosis, oJIA subtype, number of joints, ANA, ESR, CRP level, initial Juvenile Arthritis Disease Activity Score and relapse in oJIA patients who achieved remission off medication (p = 0.66, p = 0.25, p = 1, p = 0.54, p = 0.29, p = 0.59, p = 0.95 and p = 0.52, respectively). There was a statistically significant relationship between the number of intraarticular corticosteroid injections (IACIs) and relapse (p = 0.01). Patients who underwent IACI 2-3 times had more relapses than those who never underwent IACI and those who underwent IACI only once (p = 0.01, p = 0.02, respectively). A relationship was found between the length of follow-up and relapse in patients with oJIA who achieved remission off medication (p = 0.035).    Conclusion: In oJIA patients who achieve remission off medication, the probability of relapse increases in patients who need ≥ 2 IACI during the period until remission. The length of follow-up period is associated with the probability of relapse. What is Known: • Approximately one-fourth of oJIA patients who are in remission off medication have relapse. • There is a need for markers that can predict the risk of relapse in oJIA patients who achieve remission on or off medication. What is New: • The possibility of relapse should be considered in patients with oJIA who need ≥ 2 IACIs until achieving remission off medication. • The relapse rate may increase as the follow-up period prolongs in patients who achieve remission off medication.

A case series of intracardiac thrombi and vascular involvement in pediatric Behçet's disease.

To evaluate the general characteristics of pediatric Behçet's disease (BD) patients with thrombus and to present the clinical features, treatment responses and prognosis of patients with intracardiac thrombus. The clinical characteristics and outcomes of 15 patients with thrombus among 85 pediatric BD patients followed in the Department of Pediatric Rheumatology were evaluated retrospectively. Of the 15 BD patients with thrombus, 12 (80%) were male, 3 (20%) were female. The mean age at diagnosis was 12.9 ± 1.1 years. Thrombus was present at the time of diagnosis in 12 patients (80%), while thrombus developed in three patients within the first three months after diagnosis. The most common site of thrombus was the central nervous system (n = 9, 60%), followed by deep vein thrombus (n = 6, 40%) and pulmonary artery thrombus (n = 4, 26.6%). Three male patients (20%) developed intracardiac thrombus. The overall intracardiac thrombus rate in the 85 patients was 3.5%. Two of the three patients had thrombus in the right, and one had thrombus in the left heart cavity. In addition to steroids, 2 of the 3 patients received cyclophosphamide, while the patient with thrombus localized in the left heart cavity was given infliximab. In the follow-up, the two patients with thrombus in the right heart cavity were switched to infliximab because of resistance to cyclophosphamide. Complete resolution was observed in 2 of the 3 patients on infliximab; a significant reduction in the thrombus of the other patient was achieved. Intracardiac thrombus is a rare presentation of cardiac involvement in BD. It is usually observed in males and in the right heart. Although steroids and immunosuppressive agents such as cyclophosphamide are recommended as first-line treatment, favorable outcomes can be achieved with anti-TNFs in resistant cases.

Intussusception as a rare manifestation of immunoglobulin A vasculitis: Risk factors and treatment.

AIM: To identify the risk factors associated with intussusception in children with immunoglobulin A vasculitis (IgAV)-gastrointestinal (GI) tract involvement and to evaluate the outcomes of medical treatment and surgical intervention and the course of patients with intussusception. METHODS: This retrospective study was conducted in 157 patients under 18 years of age who were followed up with the diagnosis of IgAV-GI tract involvement between January 2015 and September 2022. The characteristics of the patients who developed intussusception were evaluated in detail. RESULTS: One hundred and fifty-seven patients with GI tract involvement were included in the study. The mean age of patients with IgAV-GI tract involvement was 8.7 ± 3.7 years. The female-to-male ratio was 1:1.5. Intussusception was detected in 14 patients (8.9%). Two patients (14.3%) underwent surgery, and the remaining 12 patients (85.7%) had their medical therapy intensified. Patients with GI tract involvement were divided into two groups as with (n = 14) and without (n = 143) intussusception. There was a statistically significant difference between the groups in the time from the onset of the first symptom of IgAV to the onset of steroids (P = 0.001). There were no statistically significant differences between the groups in age at onset of IgAV, gender distribution, erythrocyte sedimentation rate and C-reactive protein levels. CONCLUSIONS: The time from the onset of the first symptom of IgAV to the start of steroids is a risk factor for the development of intussusception in patients with IgAV-GI tract involvement. In these patients, medical treatment usually reduces intussusception without the need for surgical intervention.

The role of indices in predicting disease severity and outcomes of multisystem inflammatory syndrome in children.

BACKGROUND: The aim of this study was to evaluate the role of the systemic immune inflammation index (SII), C-reactive protein/albumin ratio (CAR), the monocyte/lymphocyte ratio (MLR), and the neutrophil/lymphocyte ratio (NLR) in predicting disease severity, treatment, and prognosis in multisystem inflammatory syndrome in children (MIS-C). METHODS: This medical record review retrospectively evaluated the clinical and laboratory findings of 191 MIS-C patients followed in the Department of Pediatric Rheumatology at Ankara City Hospital, Turkey. The patients were grouped by disease severity: mild, moderate, and severe. SII, CAR, MLR, and NLR were calculated for each group. RESULTS: All patients had fever at the time of admission; 153 (80.1%) had gastrointestinal tract involvement, 74 (38.7%) had rash, 63 (33%) had conjunctivitis, 107 (56%) had cardiac involvement, 32 (15.6%) had renal involvement, and 143 (74.9%) had hematological involvement. According to logistic regression analysis, SII, NLR, MLR, and CAR were found to be predictive indexes for disease severity, need for intensive care, need for inotropes, and anakinra treatment in MIS-C. The cut-off values of ≥1605.3 for SII, ≥9.1 for NLR, and ≥3.9 for CAR increased the risk of severe disease by 3.4, 7.1, and 5.7 times, respectively. CONCLUSION: NLR, SII, MLR, and CAR are effective and useful for predicting the severity of MIS-C, the need for intensive care, and the need for anakinra treatment.

Preadolescent-versus adolescent-onset immunoglobulin A vasculitis: The impact of age on prognosis.

BACKGROUND: This study aimed to investigate the characteristics of patients with preadolescent- and adolescent-onset immunoglobulin A vasculitis (IgAV) and to determine whether age affects IgAV outcomes in adolescents. METHODS: Demographic, clinical, and laboratory data of 333 patients diagnosed with IgAV at the Department of Pediatric Rheumatology, University of Health Sciences, Ankara City Hospital, were evaluated retrospectively. The patients were classified into two groups: preadolescents (<10 years) and adolescents (10-19 years). Subgroup analyses were also performed by grouping the adolescent patients into early, middle, and late adolescent groups. RESULTS: Of the 333 patients, 219 (65.8%) and 114 (34.2%) were preadolescents and adolescents. Palpable purpura, renal, joint, and gastrointestinal (GI) tract involvement were detected in 333 (100%), 78 (23.4%), 79 (23.7%), and 124 (37.2%) patients, respectively; testicular involvement was observed in 25 (13.3%) of 187 male patients. The frequency of renal involvement was significantly higher in the adolescent group than in the preadolescent group at the time of diagnosis (p = 0.030). Notably, joint involvement was significantly higher in the adolescent group (p = 0.001). The need for aggressive therapy was significantly higher in the adolescent group than in the preadolescent group (p = 0.003). There was no significant difference in clinical data, demographic characteristics, and laboratory findings between the adolescent subgroups (p > 0.05). CONCLUSIONS: Immunoglobulin A vasculitis can occur at any age but the disease prognosis appears to worsen with age. The present study reported that joint involvement, kidney involvement, and the need for more aggressive treatment were higher in the adolescent group than in the preadolescent group.

Clues for inflammatory diseases in the differential diagnosis of a child with sacroiliitis.

BACKGROUND: The purpose of this study was to compare the demographic, clinical and laboratory characteristics of patients with enthesitis-related arthritis (ERA), familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD), which are inflammatory diseases that may develop sacroiliitis. Thus, it was aimed to reveal various findings that may indicate primary disease in patients with sacroiliitis. METHODS: Pediatric patients aged 6-18 years, who were being followed with a diagnosis of ERA (n = 62), FMF (n = 590), and IBD (n = 56) over the period 2013-2021 were included in the study. Sacroiliitis (n = 55) was diagnosed by magnetic resonance imaging of the sacroiliac joint, obtained from clinically suspected patients. RESULTS: Sacroiliitis was detected in 54.8% of ERA patients, 2.3% of FMF patients, and 12.5% of IBD patients. The mean follow-up period was 4.1 ± 2.8 years (10 months-8 years) for the entire study group. The most common MRI finding for sacroiliitis was bone marrow edema. Peripheral joint involvement (73.5%) and HLA B27 positivity (64.7%) was significantly higher in ERA patients, and ERA was diagnosed more frequently in patients presenting with sacroiliitis. Non-steroidal anti-inflammatory drugs (NSAIDs) were the first choice of treatment agent when sacroiliitis developed in all three patient groups. CONCLUSIONS: The clinical and laboratory findings of ERA, FMF and IBD can sometimes be intertwined or can even coexist. Treatment may differ depending on the disease associated with sacroiliitis, although NSAIDs may be used in the first-line treatment of all three diseases. Sacroiliitis patients with HLA B27 positivity and peripheral arthritis may need to be addressed as ERA.

Does switching from coated colchicine to compressed colchicine improves treatment response in patients with familial Mediterranean fever?

AIM: To evaluate the treatment response to compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine. The secondary aim was to determine the demographic and clinical characteristics of responders to compressed colchicine. METHODS: We retrospectively reviewed the medical records of 1574 pediatric patients with FMF treated at Ankara Bilkent City Hospital. Sixty-one patients did not respond to coated colchicine and were switched to compressed colchicine. In these patients, the number of attacks and the International Severity Score for FMF (ISSF) during the 6 months before and 3, 6, 9, 12, and 24 months after switching from coated colchicine to compressed colchicine were recorded. RESULTS: Twelve of 61 patients (19.7%) who were switched to compressed colchicine due to intolerance responded to treatment. Of the 49/61 patients (80.3%) who were switched due to uncontrolled attacks and persistent subclinical inflammation, 25 responded to treatment. The frequency of attacks and ISSF decreased after switching. At the end of the two-year follow-up, 42 patients responded to compressed colchicine, and 19 patients received compressed colchicine plus interleukin-1-targeting drugs. CONCLUSIONS: Compressed colchicine was shown to be a useful treatment option before initiating biological agents in non-responders to coated colchicine, especially those with side effects.

Factors Associated with the Development of Thrombosis in Pediatric Behçet Disease.

OBJECTIVE: The present study aimed to compare the demographic, clinical, and laboratory characteristics of patients with pediatric Behçet disease (BD) with and without thrombosis to elucidate the factors that may contribute to the development of thrombosis. METHODS: This observational, descriptive, medical records review study included patients with BD (n = 85) who were diagnosed at age younger than 16 years at our clinic between 2010 and 2022. The demographic, clinical, and available laboratory data of patients with and without thrombosis were compared. The potential risk factors for the development of thrombosis were evaluated with multivariable logistic regression analysis. RESULTS: Central venous sinus thrombosis was the most common type of thrombosis. Thrombosis was significantly more common in male patients ( p = 0.002), and regression analysis revealed that being male was a risk factor for developing thrombosis. Genital ulcers were less common in patients with thromboses. Patients with thrombosis had higher erythrocyte sedimentation rates, C-reactive protein, leukocyte, and neutrophil counts, as well as antinuclear antibody positivity. In contrast, mean platelet volume and lymphocyte counts were significantly lower in patients with thrombosis. According to the logistic regression analysis, erythrocyte sedimentation rate value >17 mm/h was a risk factor for developing thrombosis (odds ratio, 1; confidence interval, 1.1-1.8; p = 0.012). CONCLUSIONS: Male sex has been associated with an increased risk of thrombosis in children with BD. Inflammatory parameters may serve as predictive factors for thrombosis in pediatric BD.

Biological Agent Switching in Patients With Juvenile Idiopathic Arthritis: A Tertiary Center Experience.

OBJECTIVE: The purpose of this study is to investigate the causes and outcomes of switching biological agents in juvenile idiopathic arthritis (JIA) patients using biological agents and compare the characteristics of patients whose biological agents are switched and those whose are not. METHODS: This medical records review study was conducted with 128 patients who were diagnosed with JIA at our clinic between January 2009 and January 2022 and were receiving biologic agents. Factors affecting the biologic agent switching were investigated. RESULTS: The JIA subtype with the most frequent switching in biological agents was systemic JIA (n = 13, 40.6%). Systemic JIA was followed by rheumatoid factor-negative polyarticular JIA and persistent oligoarticular JIA with 5 patients (15.6%), extended oligoarticular JIA and enthesitis-related JIA with 3 patients (9.3%), rheumatoid factor-positive polyarticular JIA with 2 patients (6.2%), and undifferentiated JIA with 1 patient (3.1%). Among the patients, 32 (25%) patients had their biological agent switched once, and 5 (3.9%) had theirs switched twice. The most frequently used biological agent was etanercept (n = 76, 59.3%), whereas the most frequently observed cases of biological agent switching were from an anti-TNF agent to another anti-TNF agent (40.6%). The reason for switching was unresponsiveness to the agent in 22 patients (68.8%), adverse effects in 6 patients (18.7%), drug intolerance in 1 patient (3.1%), and other reasons in 3 patients (9.3%). CONCLUSIONS: The most frequently used biological agent was etanercept; the most frequent cases of biological agents switching were from an anti-TNF agent to another anti-TNF agent.

The Effect of Clinical and Genetic Variables of Familial Mediterranean Fever Patients: Real Life Data.

BACKGROUND: The Eurofever/the Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for familial Mediterranean fever (FMF) include a combination of clinical symptoms and genotype. The pathogenicity of gene variants associated with FMF is categorized by the International Study Group for Systemic Autoinflammatory Diseases (INSAID) classification criteria. OBJECTIVE: The aim of this study was to evaluate the real-life impact and usefulness of the Eurofever/PRINTO classification criteria and the INSAID classification criteria in patients with FMF and their impact on treatment management. METHODS: In this medical records review study, the files of FMF patients who met the Eurofever/PRINTO classification criteria were reviewed. The MEFV (MEditerranean FeVer) variants were grouped according to the INSAID classification criteria. RESULTS: Of the 1062 patients, the female-to-male ratio was 1:1.01. In group 1, there were 150 patients (14.1%) who met the clinical criteria. Group 2 consisted of 912 patients (85.9%) who met the criteria according to genetic variants. The mean ages at symptom onset in groups 1 and 2 were 5.6 ± 3.8 and 1.5 ± 1.2 years, respectively ( p = 0.024). Whereas the mean annual attack frequency was 2.7 ± 3.1/year in group 1, it was 4.1 ± 2.3/year in group 2 ( p = 0.04). The pathogenic variant was higher in the colchicine-resistant group compared with the responders ( p = 0.12). CONCLUSIONS: The Eurofever/PRINTO classification criteria may provide a new perspective on the diagnosis and clinical follow-up of FMF patients. Patients with a pathogenic variant who meet the Eurofever/PRINTO classification criteria including genetic variables have earlier onset of disease and more frequent attacks than those who meet the criteria including clinical variables. These patients need regular and closer follow-ups in terms of attack frequency, colchicine dose adjustment, and colchicine resistance.

The Juvenile Spondyloarthritis Disease Activity Index Is a Useful Tool in Enthesitis-Related Arthritis: Real-Life Data.

OBJECTIVE: To compare enthesitis-related arthritis (ERA) patients with active and inactive disease at 6 months and define baseline predictors for disease inactivity. In addition, to evaluate the demographic, clinical, and laboratory characteristics of ERA patients and to identify the real-life impact of the Juvenile Spondyloarthritis Disease Activity Index (JSpADA) in predicting active disease in ERA. METHODS: This medical record review study was conducted with 56 patients who were diagnosed with ERA at our clinic between June 2009 and June 2022. Demographic and clinical characteristics, laboratory parameters, treatment, and JSpADA were recorded. RESULTS: The patients were divided into 2 groups as active (n = 34) and inactive (n = 22) according to their disease activity at month six. Sex, age at diagnosis, number and type of affected joints, and presence of sacroiliitis were similar in both groups. There was no difference in baseline erythrocyte sedimentation rate, but there was a significant difference in erythrocyte sedimentation rate at the third month ( p = 0.52 and p = 0.018, respectively). The median JSpADA values at disease onset were 3.5 (interquartile range [IQR], 3.0-4.5) and 3.3 (IQR, 2.5-4.0) in the active and inactive groups, respectively ( p = 0.27). At the third month, the median JSpADA values were 1.5 (IQR, 0.5-2.1) in the active group and 0.5 (IQR, 0.5-1.5) in the inactive group ( p = 0.037). The cutoff value for JSpADA at the third month for active disease persisting at the month six was determined as 1 point (area under the curve, 0.662 ± 0.06; p = 0.042; 95% confidence interval, 0.51-0.80) by receiver operating characteristic curve analysis. CONCLUSION: In ERA patients, a persistently high JSpADA value at follow-up is a predictive factor for active disease at the sixth month.

The Importance of Small Joint Involvement in Oligoarticular Juvenile Idiopathic Arthritis.

OBJECTIVES: Our study aimed to evaluate the relationship of small joint involvement with demographic, clinical, and laboratory findings and to determine its possible effects on prognosis. METHODS: This retrospective observational study was conducted in patients diagnosed with oJIA in the pediatric rheumatology department of our hospital between April 2009-September 2022. The relationship between small joint involvement and demographic, clinical, laboratory findings and prognosis were investigated by statistical methods with the data recorded from the medical records of oJIA patients. RESULTS: Of the 198 patients diagnosed with oJIA, small joint involvement was observed in a total of 20 (10%) patients, 11 (5.5%) at the time of diagnosis, and 9 (4.5%) during the follow-up period. The frequency of small joint involvement in extended oJIA was significantly higher than in persistent oJIA (p=0.001). Patients with small joint involvement had significantly higher ESR and CRP values at admission (p=0.047, p=0.038) and the JADAS at 3, 6, and 12 months (p=0.001, p=0.001, p=0.018). The need for cDMARDs and bDMARDs was significantly higher in patients with small joint involvement (p=0.001, p=0.001). CONCLUSIONS: oJIA patients with small joint involvement may have higher acute phase reactants at diagnosis, a more extended course and active disease in follow-up, and the need for treatment escalation.

A nationwide retrospective study in Turkish children with nephrocalcinosis.

BACKGROUND: Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey. METHODS: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010-2019) were included in the study. The medical records from the centers were reviewed and demographic data, admission complaints, medical history, systemic and genetic disorders, risk factors for NC, treatment details, and presence of NC after one-year follow-up, were recorded retrospectively. RESULTS: The study sample included 195 patients (88 females, 107 males). The mean age at diagnosis was 39.44 ± 47.25 (0.5-208) months; 82/190 patients (43.2%) were diagnosed incidentally; 46/195 patients (23.6%) had an underlying disease; idiopathic hypercalciuria was detected in 75/195 (38.4%) patients. The most common systemic diseases were distal renal tubular acidosis in 11/46 patients (23.9%), primary hyperoxaluria in 9/46 patients (19.6%) and Bartter syndrome in 7/46 patients (15.3%). After one year of follow-up, NC resolved in 56/159 patients (35.2%) and they all did not have an underlying systemic disease. DISCUSSION: The most common presentation of NC was incidental. Distal renal tubular acidosis and primary hyperoxaluria were the main systemic diseases leading to NC, while hypercalciuria was the most common metabolic risk factor. Nephrocalcinosis was found to remain in most of the patients at a one-year follow-up. It may resolve particularly in patients with no underlying systemic disease.

Urolithiasis in children; The importance of stone localization in treatment and follow-up.

To evaluate the demographic, etiologic, treatment, and follow-up differences in stones according to their location within the kidney. This retrospective study comprised 337 patients with urolithiasis between 2015 and 2019. Patients were classified into 2 groups according to stone location as lower pole stones (LPS) and upper-middle pole stones (UMPS). The patient's data were recorded at 3-month intervals for one year. One hundred and eighty-three (54.3%) female and 154 (45.7%) men were included in the study. One hundred and twenty-nine (38.3%) of the stones were in the LPS and 208 (61.7%) in the UMPS. UMPS was more common in patients aged > 12 months (p < 0.01). At least one metabolic risk factor was present in 93 (72.1%) patients with LPS and 164 (78.4%) with UMPS. The most common urinary metabolic risk factors were hyperoxaluria (31.8%) in patients with LPS and hypocitraturia (34.1%) in patients with UMPS. ROC analysis results showed that cut-off values of 5.5 mm for LPS and 6.1 mm for UMPS did not provide improve with medical treatment. At the 6- and 12-month follow-ups, the improvement rates were higher in the UMPS group than in the LPS group (p < 0.05). During the follow-up, recurrence was detected in 43 patients: 29% of patients with LPS and 5.8% of patients with UMPS (p < 0.01). Patients with small stones can be followed up. Surgical treatment may be considered for small stones in the LPS. In addition, the risk of recurrence is higher in patients with LPS, and close follow-up is required.