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Amaç: Bu çalışmada immünglobulin A vasküliti İmmünoglobulin A vasküliti (IgAV; eski adıyla Henoch Schonlein Purpurası) olan çocuklarda böbrek tutulumunun zamanını etkileyen faktörlerin ortaya konulması amaçlanmıştır. Yöntemler: Ocak 2015- Haziran 2022 arasında IgAV tanısı ile en az 1 yıl takip edilen hastaların verileri geriye dönük olarak incelendi. Hastaların demografik, klinik, laboratuar verileri ve uygulanan tedaviler kaydedildi. Bulgular: Çalışmaya 372 IgAV hastası dahil edildi, bu hastaların 81’inde IgAV böbrek tutulumu vardı. Hastaların 46’sı (%56,8) erkek, 35’i (%43,2) kızdı. Hastaların tamamında cilt tutulumu, 32’sinde (%39,5) eklem, 44’ünde (%54,3) gastrointestinal sistem (GİS), 2’sinde (%2,5) nörolojik tutulum vardı. 3 hastada tanı anında hipertansiyon vardı. Böbrek tutulumu, 45 hastada (%55,5) tanı anında, 10 hastada (%12,4) ilk 2 hafta içinde, 12 hastada (%14,8) 2-4. haftalar arasında, 14 hastada (%17,3) 1 ay-3 ay arasında saptandı. 3 aydan sonra böbrek tutulumu olmadı. Tanı anında böbrek tutulumu ile tanı yaşının büyük ve albümin düzeyinin düşük olması; 1-3 ayda böbrek tutulumu gelişmesi ile eklem ve GİS tutulumu arasında ilişki bulundu. Böbrek tutulumu olan hastalar içinde iyileşme süresi en uzun olanlar, tanı anında böbrek tutulumu saptananlar ve nefrotik düzeyde proteinüri olan hastalardı. İyileşme süresi en kısa olanlar ise, 1-3 ay arası böbrek tutulumu olan ve hematüri saptananlardı. Sonuç: Büyük yaştaki çocuklarda ve düşük albümin düzeyi olanlarda IgAV-böbrek tutulumunun gelişimi dikkatle takip edilmelidir. Özellikle ilk 3 ay böbrek tutulumunun saptanması için riskli dönemdir.

Amaç: Çalışmamızda pediatrik sistemik lupus eritematozus (SLE) hastalarında, hastalık prezentasyon bulgularının, hastalığın 2. yılındaki düşük hastalık aktivitesi ve remisyona ulaşmadaki etkilerinin incelenmesi amaçlanmıştır. Gereç ve Yöntemler: Merkezimizde takipli olan pediatrik SLE hastalarının elektronik tıbbi kayıtları ve hasta dosyalarından demografik, klinik ve laboratuvar verileri kaydedildi. Hastalığın 2. yılındaki aktivite ölçümü SLE Hastalığı Aktivite İndeksi-2000 (SLEDAI-2K) skoru baz alınarak hesaplandı. Prezentasyon bulguları ile düşük hastalık aktivitesi ve remisyon arasında ilişki olup olmadığı istatiksel yöntemlerle incelendi.Sonuçlar: Bu çalışmaya merkezimizde pediatrik SLE tanısı almış ve en az 2 yıl düzenli takibe gelmiş 29 hastayı dahil ettik. Tanıdan sonraki 2. yılda hastalarda SLE aktivite ölçümüne göre 14 hastada (%48.2) HDAS, 15 hastada (%51,7) LDAS-remisyon elde edilmişti. İki grup arasında başlangıçtaki prezentasyon bulguları açısından anlamlı istatiksel farklılık saptanmadı. 5. yılda 15 hastadan 6’sında (%40) LDAS-remisyon, 9’unda (%60) HDAS mevcuttu. 2. yılda HDAS’a sahip hastaların 5. yıl SLEDAI-2K skorları, 2. yılda LDAS-remisyona sahip hastaların 5. yıl SLEDAI-2K skorlarına göre anlamlı olarak yüksekti (p= 0.028). Ayrıca 2. yılda aktif olan 8 hastanın (%80) 5. yılda HDAS’ın devam ettiği gözlendi.Tartışma: Çalışmamızda pediatrik SLE prezentasyon bulgularının hastalığın 2. yılındaki düşük hastalık aktivitesi ve remisyon üzerine belirleyici etkilerinin olmadığını gösterildi. Ayrıca 2. yılda elde edilen düşük hastalık aktivitesi ve remisyon 5. yıldaki düşük hastalık aktivitesi ve remisyonun belirleyicisi olabilir.

In this study, it was aimed to evaluate the demographic, clinical and laboratory characteristics of MIS-C patients in our hospital, to share our treatment approach, and to assess the outcomes of short- and long-term follow-up.MIS-C patients who were admitted and treated in our hospital between July 2020 and July 2021 were evaluated. Demographic, clinical, laboratory, and follow-up data were collected from patient records retrospectively.A total of 123 patients with MIS-C (median age, 9.6 years) were included the study. Nineteen (15.4%) were mild, 56 (45.6%) were moderate, and 48 (39%) were severe MIS-C. High CRP, ferritin, pro-BNP, troponin, IL-6, and D-dimer values were found in proportion to the severity of the disease (p 0.001, p 0.001, p 0.001, p 0.001, p = 0.005, p 0.001), respectively. Two (1.6%) patients died. The mean follow-up period was 7.8 months. Valve failure, left ventricular dysfunction/hypertrophy, coronary involvement, and pericardial effusion were the most common cardiac pathologies in the short- and long-term follow-up of the patients. In the long-term follow-up, the most common reasons for admission to the hospital were recurrent abdominal pain (14.2%), cardiac findings (14.2%), pulmonary symptoms (8%), fever (7.1%), neuropsychiatric findings (6.2%) and hypertension (3.5%). Neuropsychiatric abnormalities were observed significantly more common in severe MIS-C patients at follow-up (p = 0.016). In the follow-up, 6.2% of the patients required recurrent hospitalization.MIS-C is a serious and life-threatening disease, according to short-term outcomes. In addition to the cardiac findings of patients with MIS-C, long-term outcomes such as neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms should be monitored. Key Points • In MIS-C patients, attention should be paid not only to cardiac findings, but also to symptoms related to other systems. • Patients should be followed up in terms of neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms that may occur during follow-up.

ObjectivesThe 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.MethodsOne 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.ResultsThe 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 ConclusionsFMF 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.

To determine the interval between disease onset and admission to pediatric rheumatology clinic of patients with juvenile idiopathic arthritis (JIA) and, to identify the factors that affect the admission time (AT) to rheumatology center.We designed a retrospective observational study in children with JIA. The study variables were age, gender, JIA subtype, acute phase reactants (APR), disease activity scales, presence of a pediatric rheumatologist, and distance to a pediatric rheumatology center. Outcome parameter was the duration between onset of symptoms and first visit of rheumatologist. The parameters were evaluated with variance analysis and regression models.198 patients (female:120 (60.6%)) were included. There were 112 (56.5%) patients in oligo-articular JIA, 27 (13.6%) in rheumatoid factor negative poly-articular JIA, 22 (11.1%) in enthesitis related arthritis (ERA), 29 (14.6%) in systemic-JIA, 4 (2%) in rheumatoid factor positive poly-JIA, two patients each in undifferentiated and psoriatic arthritis. The median AT in the systemic-JIA and other groups was 16 (IQR 10.5-27.5) and 71 (IQR 33.5-211) days, respectively. There was a significantly longer AT in the ERA group than others (p=0.005). We found a correlation between longer AT and older age, low back pain, enthesitis, and low erythrocyte sedimentation rate (ESR). In the multivariate analysis, only low ESR and enthesitis contributed an increase in AT [OR 2.05 (1.07-3.93), 6.22 (1.29-29.99)].The older age, low back pain, enthesitis and low ESR contribute to the late AT. JIA requires high suspicion in children with poorly defined findings and low APR.

Purpose: Mutations in the Mediterranean FeVer (MEFV) gene, which causes familial Mediterranean fever(FMF), may also cause the emergence of other specific rheumatic diseases. This study aims to determine thefrequency of other rheumatologic diseases in paediatric FMF patients, evaluate whether there are clinical andgenetic differences between those with and without concomitant rheumatologic diseases, and compare the datawith previous studies.Materials and methods: The files of FMF patients who were followed up at the paediatric rheumatologydepartment were reviewed retrospectively. Demographic data, MEFV mutations, treatment, disease severityscores, and concomitant rheumatic diseases were recorded from the files.Results: There were 303 FMF patients (154 female/149 male). The mean age at diagnosis was 7.04±3.9 years.The mean disease duration was 5.33±3.13 years. In the cohort, 41 FMF patients (13.5%) were diagnosed withanother rheumatic disease. There were 22 cases of juvenile idiopathic arthritis (53.6%), seven cases of vasculitis(17%), six cases of periodic fever aphthous stomatitis and adenitis syndrome (14.6%), three cases of Behçet’sdisease (7.3%), two cases of acute rheumatic fever (4.8%), and one case of systemic lupus erythematosus(2.4%). Thirty-two of these of these 41 FMF patients (78%) had the M694V mutation (homozygous in 11,heterozygous in 21). Disease activity scores Pras and ISSF scores were higher in FMF patients with rheumaticdiseases (p=0.002 and p

Aim Joint hypermobility is a term used to describe an excessive range of joint motion. Joint hypermobility can be symptomatic or not. The present study aimed primarily to define the prevalence of joint hypermobility in healthy schoolchildren, and secondly, to determine the relationship between hypermobility and pain, physical activity, joint injury, quality of life, and balance. Materials and Methods In this cross-sectional study, the joints of 737 schoolchildren, aged 8 to 15 years, were examined according to the Beighton score (BS). Generalized joint hypermobility was defined by using a cut-off point of ≥6 joints. The participants with a BS between 1 and 5 were accepted as localized hypermobile. If the Beighton score was 0, the participants were accepted as non-hypermobile. Participants were evaluated using questionnaires or tests for pain, balance, physical activity, and quality of life. Results The 350 (47.5%) males and 387 (52.5%) females had a mean age of 11.47 ± 1.3 (8-15) years. The prevalence of generalized hypermobility was 13.4%, and we observed localized hypermobility in 65.9% of children and non-hypermobility in 20.6% of children. The most common pain localizations in children were neck (15.9%), lower back (13.7%), upper back (10.6%), shoulders (10.2%), and knees (7.9%). There was no association between pain and hypermobility in children aged 8 to 15 years. Conclusion The generalized joint hypermobility group was younger, shorter, and thinner than other groups. Additionally, we observed that hypermobility did not make a difference in terms of pain, quality of life, physical capacity, and balance in school-age Turkish children.

Objectives The aims of this study were to describe the clinical features, comorbidities and outcome of systemic childhood polyarteritis nodosa (PAN) and to evaluate PAN-like diseases in differential diagnosis. Methods The study group consisted of patients who were diagnosed as PAN in a referral center in Turkey. The files of all patients were reviewed retrospectively. Disease activity was evaluated with pediatric vasculitis activity score (PVAS). Results A total of 19 (13 boys/six girls) patients were enrolled in the study. The mean age of patients was 10.37 ± 3.6 years. The mean duration of follow-up was 5.73 ± 3.74 years. Eight patients (42.1%) were also diagnosed with familial Mediterranean fever (FMF). The cutaneous involvement was higher in patients with PAN than those with FMF-associated PAN (p = .03). The median (min–max) PVAS at diagnosis was 5 (3–7). There was no correlation between PVAS scores at the time of diagnosis and age, clinical findings and relapse. CECR1 mutation was detected in one patient leading to deficiency of adenosine deaminase 2. Conclusion The clinical presentation is variable in children with PAN. PAN-like diseases characterized by necrotizing vasculitis should be considered. The possibility of FMF should be kept in mind if inflammation cannot be controlled.

Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening hyperinflammation syndrome emerging after COVID-19. The serum delta neutrophil index (DNI) reflects the fraction of circulating immature granulocytes and is evaluated in infection and inflammation. The aim of this study is to evaluate the usefulness of DNI as a diagnostic marker in patients with MIS-C and to assess its role in determining the severity of MIS-C. This retrospective, observational study included 83 patients with MIS-C and 113 patients with COVID-19, and 102 healthy controls. C-reactive protein (CRP), the absolute neutrophil count (ANC), absolute lymphocyte count (ALC), DNI, and the platelet count were recorded. The DNI levels were 4.60 ± 5.70% in the MIS C group, 0.30 ± 0.99% in the COVID group, and 0.20 ± 0.56% in the control group (p < 0.001). According to the severity of MIS-C, the DNI level was found to be 1.22% in mild MIS-C, 4.3% in moderate MIS-C, and 5.7% in severe MIS-C. There was a statistically significant correlation between DNI levels and the severity of MIS-C. The cutoff value of DNI for predicting MIS-C was 0.45%. In the analysis of the diagnostic performance of DNI compared with CRP, ANC, ALC and platelet counts, sensitivity, specificity, positive predictive value, and negative predictive value were found to be 79.5%, 97.1%, 95.7%, and 85.3%, respectively.Conclusions: The delta neutrophil index was identified as a diagnostic marker for MIS-C such as ANC, ALC, platelet count, and CRP. DNI levels in hemogram analysis may guide clinicians in determining the diagnosis and severity of MIS-C. What is Known: • Although CRP, sedimentation, ALC, ANC, platelet count, sodium, and albumin are used as first step tests, there is no specific laboratory marker used in the diagnosis of MIS C. • The serum delta neutrophil index (DNI) reflects the fraction of circulating immature granulocytes and is elevated in infection and inflammation. What is New: • DNI is a promising and easily accessible marker that can be used with other markers in the diagnosis and determines the severity of MIS C. • DNI is an easily accessible, inexpensive, and dynamic marker and its levels in simple hemogram analysis will guide pediatricians in determining the diagnosis and severity in MIS C.

Objective: The aim of this study was to evaluate the treatment response of compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine. Another objective was to determine the demographic and clinical characteristics of the group that responded to compressed colchicine preparations. Methods: In this retrospective study, the files of 1574 patients with FMF in our center were reviewed. Sixty-one patients who did not respond to coated colchicine and were switched to compressed colchicine treatment were evaluated. 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: Of the 61 patients, 36 (59%) were boys, and 25 (41%) were girls. The mean age of symptom onset was 34±22 months. The mean age at diagnosis was 8.4±5.1 years. The mean age at the time of switching from coated colchicine to compressed colchicine was 11.8±3.5 years. Among 61 patients whose treatments were switched to compressed colchicine, 12 had intolerance, while 49 had uncontrolled attacks and persistent subclinical inflammation. The problems of 12 patients who were switched to compressed colchicine due to intolerance were resolved. Response to compressed colchicine was observed in 25/49 patients with uncontrolled attacks and persistent subclinical inflammation. The frequency of attacks decreased significantly at the 3rd and 6th months after switching from coated colchicine to compressed colchicine (prd and 6th months after switching to compressed colchicine were 3.2±2.1 and 2.6±1.7, respectively. Twenty-four of the 49 patients did not respond to compressed colchicine. Anakinra was added to the treatments of 14 of the 24 patients, and canakinumab was added to the treatments of 10. The frequency of attacks and ISSF scores of the patients who did not respond to compressed colchicine significantly decreased after the addition of IL-1-targeting drugs to their treatments. At the end of the two-year follow-up, 42 patients were tolerant to compressed colchicine, and 19 patients (4 anakinra, 15 canakinumab) were on compressed colchicine plus IL-1-targeting drugs. Conclusions: Compressed colchicine was shown to be a useful treatment option before initiating biological agents in patients who were unresponsive to coated colchicine, especially those with side effects.

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.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.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 value17 mm/h was a risk factor for developing thrombosis (odds ratio, 1; confidence interval, 1.1-1.8; p = 0.012).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.

The aim of this study is to evaluate the outcomes of patients who received intravenous immunoglobulin (IVIG) for immunoglobulin A vasculitis (IgAV) with gastrointestinal (GI) tract involvement, and to determine the differences between the groups that responded to IVIG and those that did not.This retrospective study comprised 152 patients with IgAV between 2018 and 2022. Sixty-five patients (43%) had GI tract involvement. Patients with IgAV-GI involvement who had been treated with IVIG were evaluated. Patients were classified with IgAV according to the 2008 Ankara-EULAR/PRINTO/PRES. Their demographics, presentation, and management are reported.Twelve (7 boys/5 girls) of these patients were treated with IVIG. The median age was 90.1 (31-177) months. The mean follow-up period was 30.6 ± 9.9 months. All patients had skin involvement, joint involvement (arthralgia or arthritis), and abdominal pain. All 12 patients were given steroids (30 mg/kg/day pulse methylprednisolone for 3-7 days, followed by 2 mg/kg/day steroids) before IVIG. Nine patients received cyclophosphamide treatment (four before IVIG and five after IVIG). Complete remission was achieved in 5 of the patients with IVIG. Four patients were diagnosed with IgAV concomitant familial Mediterranean fever, and colchicine treatment was initiated.IVIG may be used in steroids and/or immunosuppressive drug resistant IgAV. It can be considered as a treatment option, especially in patients with multi-organ/system involvement, comorbid inflammatory diseases such as familial Mediterranean fever, and in patients with IgAV-GI tract involvement resistant to standard treatment in the advanced pediatric age group.

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.Demographic, clinical, and laboratory data of 333 patients diagnosed with IgAV at the Department of Pediatric Rheumatology were evaluated retrospectively. The patients were classified into two groups: preadolescents (10 years) and adolescents (10-19 years). In addition, subgroup analyses were performed by grouping the adolescent patients into early, middle, and late adolescent groups.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; moreover, 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 (p0.05).IgAV 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.

Juvenile Behcet’s disease is a rare and severe disease of childhood characterized by a chronic inflammatory vasculitis. The aim of the present study is reporting demographic, clinical and therapeutic outcomes of juvenile Behcet’s disease in a tertiary center. The retrospective study included patients who were diagnosed Behcet’s disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded. Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone. In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death.

Objective: The aim of this study was to assess the factors that cause delay in diagnosis of familial Mediterranean fever (FMF), which is a common disease in our country.Material and Methods: The data of 101 patients diagnosed with FMF in our pediatric rheumatology center between 2010 and 2021 were retrospectively analyzed. The time elapsed from the onset of the patients' symptoms to the time of diagnosis was defined as the delay in diagnosis. Pras disease severity score was used to determine disease severity.Results: Fifty-nine of 101 patients were girls (58.4%). The median age at onset of symptoms was 5 (min-max: 2-8) years. The median age at diagnosis of the patients was 6 (min-max: 4-9) years. Delay in diagnosis was 3 years in 20 patients. There were 39 (38.6%) M694V homozygous, 34 (33.7%) M694V heterozygous, 13 (12.9%) non-M694V mutations, 2 (2%) patients with a single mutation except M694V, and 13 (%13) patients with no mutation. According to the Pras disease severity score, disease severity was classified as mild in 22.8%, moderate in 43.6%, and severe in 33.7% of the patients. There was no relationship between family history of FMF, consanguineous marriage, clinical findings, mutations, and delay in diagnosis. A statistically significant relationship was found between the delay in diagnosis of more than 3 years and the age of onset of symptoms of the patients (p, Amaç: Bu çalışmada ülkemizde sık görülen ailevi Akdeniz ateşinin tanısının konulmasında gecikmeye neden olan faktörlerin ortaya konulması amaçlanmıştır.Gereç ve Yöntemler: Çocuk Romatoloji Polikliniğinde 2010-2021 yılları arasında ailevi Akdeniz ateşi tanısı alan 101 hastanın dosya kayıtları retrospektif olarak incelendi. Hastaların şikâyetlerinin başlama zamanından tanı aldıkları zamana kadar geçen süre tanı gecikmesi olarak tanımlandı. Hastalık ağırlığını belirlemek için Pras hastalık ağırlık skoru kullanıldı.Bulgular: Yüz bir hastanın 59’u kızdı (%58.4). Şikâyetlerin ortanca başlama yaşı 5 yıl (min-maks: 2-8) olarak saptandı. Hastaların ortanca tanı alma yaşı 6 yıl (min-maks:4-9) idi. Tanı gecikmesi 41 hastada 3 yıldı. M694V homozigot olan 39 (%38.6), M694V heterozigot 34 (%33.7), M694V dışı iki mutasyon taşıyan 13 (%12.9), M694V dışında tek mutasyon olan 2 (%2), mutasyon saptanmayan 13 (%13) hasta olduğu görüldü. Pras hastalık ağırlık skoruna göre, hastalık ağırlığı hastaların %22.8’sinde hafif, %43.6’sında orta, %33.7’sinde ağır şiddetli olarak sınıflandırıldı. Tanı gecikmesi ile ailede ailevi Akdeniz ateşi öyküsü, akraba evliliği olması, klinik bulgular ve mutasyonlarla ilişki gösterilemedi. Tanı gecikmesinin >3 yıl olması ile hastaların semptomlarının başlama yaşı arasında istatistiksel olarak anlamlı ilişki saptandı (p3 yıl olması arasında istatistiksel anlamlı ilişki olduğu görüldü (p=0.026).Sonuç: Ailevi Akdeniz ateşi’nin tanısının konulması, şikâyetler süt çocukluğu döneminde başlarsa ve hafif hastalıkla uyumluysa gecikebilir. Erken çocukluk döneminde tekrarlayan ateş ve karın ağrısı gibi bulguları olan çocuklarda ailevi Akdeniz ateşi akılda tutulması gereklidir.

Background: Systemic lupus erythematosus (SLE) is a common multisystemic autoimmune disease characterized by the presence of autoantibodies and multiorgan system involvement. The aim of this study was to describe the presenting clinical manifestations, laboratuary findings, clinical course and prognosis of SLE in children. Methods: We performed a retrospective study patients with SLE, diagnosed before the age of 18 years. Clinical and laboratory data were collected from initial admission to study initiation. Results: Thirty-five children and adolescents SLE (cSLE) were recorded, 85.7% of female. The median age at disease onset was 12(range 4-17) years, and median follow up duration was 5 (1-14) years. The most common clinical feature was arthritis (65.1%), followed by constitutional symptoms (48.6%), malar rash (31.4%), photosensitivity (5.7%), alopecia (5.7%) and oral ulcers (5.7%). Renal involvement accounted 4/5 of the patients (80%). Hematuria and proteinuria were the most frequent presenting findings (48.5% and 45.7% respectively). Renal biopsy was performed in 27 patients. According to WHO classification: 1 patient had classV nephritis, 10 had class IV, 4 had class III and 12 had class II nephritis. 20% of children developed neurologic symptoms. One patient died during the follow-up period. At the last follow up none of the patients had renal failure but, proteinuria persisted in 4 of them (11.4%). All neurological findings of patients are in remission apart from sequelae. Conclusion: Clinical outcome was favorable in our patients. Renal involvement is common but progression to end stage kidney disease, at least in the short term, is rare.

Aim: The aim of this study is to evaluate the demographic and clinic findings in immunoglobulin A-associated vasculitis (IgAV) patients with scrotal involvement and also to determine predictive factors for assessing the development of scrotal involvement. Materiel and Methods: The medical records of 181 boys who were diagnosed with IgAV in the Pediatric Rheumatology Clinic of our center between September 2015-January 2021 were evaluated retrospectively. Results: A total number of 181 boys with IgAV included in the study. Twenty-seven (14.9%) of the 181 boys with IgAV had scrotal involvement. Among the scrotal-involved patients, 160 boys (88.4%) had scrotal swelling, 120 (66.3%) had erythema and 145 (80.1%) had scrotal pain or tenderness. Scrotal involvement was the first symptom of IgAV in one patient. CRP and WBC were significantly higher in the scrotal-involved group compared to the non-involved group (p=0.018, p=0.04, respectively). There were no significant differences in clinical findings and organ involvements between two groups. On ultrasonography, bilateral scrotal soft tissue thickening was observed in all patients. In 130 of the patients (71.8%), the epididymis was swollen with increased vascularity. The size, echogenicity and vascularity of the testicles were within normal limits. Eighty-seven of the patients (48%) were given steroids, 22 of them (11.7%) nonsteroidal anti-inflammatory drug. Conclusion: Scrotal involvement in boys with IgAV is not rare, it should be considered in the differential diagnosis in patients with scrotal pain, swelling and erythema. In addition, inflammatory markers may be higher in patients with scrotal involvement.

Chronic non-bacterial osteomyelitis is a chronic sterile inflammatory bone condition. We aimed to describe patients' clinical and radiographic findings and to evaluate their response to therapy and their quality of life.This cross-sectional study included 18 patients from a single center in Turkey whose clinical, radiological features, and outcomes were reviewed retrospectively. The quality of the patients' lives after treatment was compared with healthy controls using the Pediatric Quality of Life Inventory 4.0.The median age of disease onset was 12 years (IQR 10-14 years) and 11 (61.1%) patients were male. The median follow-up duration was 15 months (IQR 12-22 months). The persistent form of chronic non-bacterial osteomyelitis was the most common pattern in 15 (83.3%) patients and a recurrent pattern was defined in three (16.7%) patients. The lesions were multifocal in all patients and 15 (83.3%) patients had symmetric distribution in whole-body magnetic resonance imaging. The most common sites of arthritis were the knee and sacroiliac joints. Methotrexate was used in 16 (88.9%) patients as first-line therapy. However, some patients were unresponsive to the first-line therapy and needed tumor necrosis factor-α inhibitors (55.6%) and bisphosphonates (16.7%). We observed remission in only four (22.2%) patients, and three (16.7%) patients were unresponsive. The patients had a significantly poorer quality of life than controls (P = 0.005).Chronic non-bacterial osteomyelitis is an insidious disease that requires detailed analysis for diagnosis and whole-body magnetic resonance imaging is an effective tool for its diagnosis. Despite the advanced treatment, patients with chronic non-bacterial osteomyelitis have a poor quality of life.

PurposeChronic non-bacterial osteomyelitis is a chronic sterile inflammatory condition of the bone. We aimed to describe the clinical and radiographic findings of patients and to evaluate their response to therapy and quality of life. MethodsThis cross-sectional study included 18 patients whose clinical, radiological features and outcomes were reviewed retrospectively. The quality of patients’ life after treatment was compared to healthy controls by using the Pediatric Quality of Life Inventory 4.0. ResultsThe median age of disease onset was 12 (IQR 10-14) years and 11 (61.1%) patients were male. The median follow-up duration was 15 (IQR 12-22) months. The persistent form of chronic non-bacterial osteomyelitis was the most common pattern in 15 (83.3%) patients and a recurrent pattern was defined in 3 (16.7%) patients. The lesions were multifocal in all patients and 15 (83.3%) patients had symmetric distribution in whole-body magnetic resonance imaging. The most common sites of arthritis were the knee and sacroiliac joints. Methotrexate was used in 16 (88.9%) patients as first-line therapy. However, some patients were unresponsive to first-line therapy and needed tumor necrosis factor-α inhibitors (55.6%) and bisphosphonates (16.7%). We observed remission in only 4 (22.2%) patients and 3 (16.7%) patients were unresponsive. The patients had a significantly poorer quality of life than controls (p=0.005).ConclusionsChronic non-bacterial osteomyelitis is an insidious disease that requires detailed analysis for diagnosis and whole-body magnetic resonance imaging is an effective tool for diagnosis. Despite the advanced treatment, patients with chronic non-bacterial osteomyelitis have a poor quality of life.

Amaç: Çocuklarda SARS-Cov-2 enfeksiyonu sonrasında ateş, kardiyojenik şok ve hiperinflamasyon bulguları ile gelişen multisistem inflamatuar sendrom tablosu yaşamı tehdit edici olabilmektedir. Bu çalışma ile multisistem inflamatuar sendrom tanısı alan hastaların başvuru şikayet ve sürenin hastalık şiddetine etkisini araştırmak amaçlandı.Gereç ve yöntem: Eylül 2020-Ağustos 2021 tarihleri arasında merkezimizde Hastalık Kontrol ve Önleme Merkezleri (CDC) vaka tanımı ile multisistem inflamatuar sendrom tanısı alan 99 çocuk hasta tıbbi kayıtları geriye dönük olarak değerlendirildi. Demografik özellikleri, başlangıç klinik bulguları, başvuru süreleri not edildi. Hastalar yoğun bakım ihtiyacına göre sınıflandırıldı.Bulgular: Hastaların medyan yaşı 10 (6-14) ve %62,6’sı erkekti. Tanıdan önceki medyan semptom süresi 4 (3-5) gündü. Başvuru sırasında hastaların tümünde ateş, %81,8’inde gastrointestinal ve %75,8’inde kardiyovasküler tutulum mevcuttu. Ağır ve orta şiddette MİS-C olarak değerlendirilen %56,6 hastanın yoğun bakım gereksinimi oldu. Ateş süresinin uzun olması, sağlık kuruluşuna gecikmiş başvuru, hastanın kardiyovasküler etkileniminin ön planda olması, yüksek inflamatuvar belirteçler, lenfopeni ve trombositopeni varlığı yoğun bakım gereksinimini etkileyen önemli parametreler olarak bulundu.Sonuç: Çocuklarda multisistem inflamatuar sendrom, SARS-CoV-2 enfeksiyonu ile ilişkili ateş, inflamasyon belirtileri ve organ disfonksiyonu ile karakterize yeni bir hastalıktır. Gecikmiş başvuru ile uzamış ateş, ilk başvuru anında kardiyak ve inflamatuar belirteçlerin yüksek olması yoğun bakım gereksinimini artırmaktadır., Purpose: Multisystem inflammatory syndrome that occurs after SARS-Cov-2 infection with fever, cardiogenic shock and hyperinflammation in children, can be life threatening. In this study, it was aimed to investigate the effects of the complaint and duration at diagnosis on the severity of multisystem inflammatory syndrome in children.Materials and methods: The medical records of 99 pediatric patients, who were diagnosed multisystem inflammatory syndrome between September 2020 and August 2021 according to Centers for Disease Control and Prevention, were evaluated retrospectively. Demographic features, initial findings, and admission time of patients were noted. Patients were categorized according to intensive care necessity.Results: The median age of the patients was 10 (2-18) and 62 (62.6%) of patients were male. The median duration before admission was 4 (1-10) days. All patients has fever, 81.8% had gastrointestinal and 75.8% had cardiovascular involvement at admission. The patients (56.6%) who were accepted as severe and moderate MIS-C required intensive care. Prolonged fever, delayed admission, cardiovascular involvement, high inflammatory markers, lymphopenia and thrombocytopenia were found to key parameters determining the need for intensive care.Conclusion: Multisystem inflammatory syndrome in children is a new disease characterized by fever, signs of inflammation and organ dysfunction associated with SARS-CoV-2 infection. Delayed admission, high cardiac and inflammatory markers at diagnosis increase the need for intensive care.

Behcet's disease is an inflammatory vasculitic disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular findings, and multiple organ involvement. Mucocutaneous findings are the most common symptoms. The most used diagnostic criteria are International Criteria for BD (ICBD), International Study Group (ISG) criteria and pediatric Behcet’s disease criteria (PEDBD). Although diagnostic criteria have been defined, the diagnosis is still difficult due to clinical findings developed in pediatric patients. The aim of this study was to evaluate the clinical findings, phenotype characteristics, sensitivity and specificity of diagnostic criteria, and the course of pediatric Behcet's disease (BD). We evaluated retrospectively the files of 67 (29 M/38 F) patients diagnosed with BD according to expert opinion. The patients were reclassified according to ISG, ICBD, and PEDBD criteria. The control group consisted of a total of 69 patients with BD-mimicking diseases or presenting at least one major BD sign followed at the same center. Sensitivity and specificity were evaluated for the criteria. The mean age of diagnosis was 13.38 ± 3.2 years. There were oral aphthous ulcers in 98.5%, genital ulcers in 68.7%, skin lesions in 31.3%, vascular involvement in 17.9%, neurological involvement in 11.9%, positive pathergy test in 33.8%, and positive HLA-B51 in 57.1% of patients. The sensitivity of ICBD, ISG and PEDBD criteria was 88.1, 43.3, and 37.3%, respectively. The specificity of ICBD, ISG, and PEDBD criteria was 100%. Diagnosis of BD in childhood is still difficult. ICBD criteria have the highest sensitivity among the diagnostic criteria. These criteria can also be used in childhood.

Background: Corneal and lenticular optical properties are not well-documented in pediatric patients with inflammatory diseases. Here we aimed to evaluate corneal and lenticular optical density as well as corneal morphology in children with ocular and/or systemic inflammation by Scheimpflug imaging. Methods: Fifty-five eyes of 29 children with non-infectious uveitis, 56 eyes of 28 children with systemic inflammation without uveitis and 60 eyes of 31 healthy children were included. Corneal/lenticular optical density, corneal volume, central corneal thickness, keratometry were analyzed by Pentacam HR. Corneal endothelial cell density, hexagonal cell ratio and coefficient of variation were measured by specular microscope. Results: The mean age was 12.0 ± 3.1 years, 11.9 ± 4.0 years and 11.3 ± 3.4 years whereas the female/ratio was 15/14, 15/13 and 14/17 in uveitis, systemic inflammation and control groups respectively. Uveitis type was anterior uveitis in 16 (29.1%) eyes, intermediate uveitis in 32 (58.2%) eyes and panuveitis in 7 (12.7%) eyes. Twenty-two (40%) eyes had active uveitis whereas 33 (60%) eyes had inactive uveitis. Corneal optical density was greater in uveitis group compared with other groups (p = 0.001, Kruskal-Wallis test). Lenticular density and corneal parameters other than optical density were not different between the groups (p > 0.05). Corneal optical density was higher in active uveitis than inactive uveitis (22/33 eyes, p = 0.017). Conclusion: Children with uveitis had decreased corneal clarity compared with systemic inflammation group and healthy controls, while lenticular clarity was similar between the groups. Corneal endothelial parameters did not change significantly based on ocular/systemic inflammation. Scheimpflug imaging provides objective corneal and lenticular optical density measurements. © 2022

İmmunglobulin A vasküliti küçük damarlarda immun kompleks birikimi ile oluşan ve genellikle kendini sınırlayarak iyi seyir gösteren çocukluk çağının en sık vaskülittir. Bu çalışma ile kliniğimizde takip edilen immunglobulin A vasküliti tanılı hastaların klinik özelliklerini, seyirlerini, tedavilerini tanımlamak ve MEFV tetkik edilen hastaları detaylandırarak hastalık seyrine katkısı olup olmadığını ortaya koymaktır.Gereç ve Yöntemler Retrospektif olarak düzenlenen bu çalışma Ocak 2012 ile Haziran 2021 tarihleri arasında immunglobulin A vasküliti tanısı almış olguları içermektedir. Hastaların elektronik dosyalarından demografik, klinik, laboratuvar özellikleri, görüntüleme sonuçları ve tedavileri kaydedildi. Bulgularİmmunglobulin A vasküliti tanısı ile çalışmaya dahil olan 345 hastanın %42,30’ü kızdı. Hastaların medyan tanı yaşı 6,5 yıl (1-17,50). Şikayetlerin başladığı dönem mevsimlere göre %31,30 sonbahar, %28,70 ilkbahar, %27,50 kış ve %12,50 yaz oranlarında dağılmaktaydı. Tüm hastaların döküntüsü mevcuttu. Hastalık sürecinde %38,80 hastada kas iskelet, %35,70 hastada gastrointestinal, %7 hastada böbrek ve %8,10 hastada epididimal tutulum gelişti. MEFV taraması yapılan 54 hastanın 27 (%7,80)’sinde mutasyon pozitifti ve gruplar arasında cinsiyet, hastalık başlama yaşı, klinik tutulum ve tedavi parametreleri açısından anlamlı fark yoktu.Sonuçİmmunlobulin A vasküliti çocukluk çağının çoğunlukla iyi prognoza sahip vasküliti olmakla beraber bazı hastalarda ağır seyrederek sekel bırakabilmektedir. Kötü prognozu açıklayacak faktörler için çok merkezli prospektif çalışmalar gereklidir.

Objectives: Our study was planned to investigate the effect of Clinical Pilates exercises in children and adolescents with Juvenile Idiopathic Arthritis (JIA). Methods: Fifteen participants diagnosed with JIA (mean age = 12.00 ±3.40 years) were included. The participants were randomly divided into two groups as Clinical Pilates exercise group (n = 6), and home exercise group (n = 9). Clinical Juvenile Arthritis Disease Activity Score (cJADAS), Wong Baker Face Scale, Brunininks-Oseretsky Test of Motor Proficiency Second Edition Short Form (BOT-2 SF), Juvenile Arthritis Biopsychosocial Scale (JAB-Q scale), and Pediatric Quality of Life Inventory (PedsQL) 3.0 Arthritis Module was used for evaluation before and after treatment. Exercises were performed by both groups 3 times a week for 6 weeks. Results: In the analysis of the before and after treatment results, a significant difference was observed in cJADAS (p = .027), manual dexterity (p = .020), running speed and agility (p = .027) subtests of BOT-2 SF, total score of BOT-2 SF (p = .042) and daily activity (p = .043) subtests of PedsQL child form in the Clinical Pilates exercise group. While there was statistically significant differences in manual dexterity (p = .024), running speed and agility (p = .041) and upper limb coordination (p = .034) subtests of BOT-2, and parent form of JAB-Q (p = .041) in home exercise group. When the delta values were compared, the difference was significant in the upper limb coordination subtest of BOT-2 SF (p = .008), and daily activities subtest of PedsQL child form (p = .003) in favour of the Clinical Pilates exercise group. Conclusions: Clinical Pilates exercises are a safe and useful method for children/adolescent with JIA. RESUMEN Objetivos: Nuestro estudio fue planeado para investigar el efecto de los ejercicios de pilates clínico en niños y adolescentes con artritis idiopática juvenil (AIJ). Métodos: Se incluyeron 15 participantes diagnosticados con AIJ (edad media = 12,00 ±3,40 años). Los participantes fueron divididos al azar en 2 grupos como grupo de ejercicio de pilates clínico (n = 6) y grupo de ejercicio en el hogar (n = 9). Para la evaluación antes y después del tratamiento se utilizó la puntuación de actividad de la artritis juvenil clínica (cJADAS), la escala Wong-Baker FACES®, la prueba Brunininks-Oseretsky de proficiencia motora en su segunda edición (BOT-2 SF), la escala biopsicosocial de artritis juvenil (escala JAB-Q) y el inventario de calidad de vida pediátrica (módulo PedsQL® 3.0) de artritis. Se realizaron ejercicios a ambos grupos, 3 veces por semana, durante 6 semanas. Resultados: Cuando se analizaron los resultados, antes y después del tratamiento, se observó una diferencia significativa en las subpruebas de cJADAS (p = 0,027), destreza manual (p = 0,020), velocidad y agilidad en la carrera (p = 0,027) de BOT-2 SF, puntuación total de BOT-2 SF (p = 0,042) y actividad diaria (p = 0, 043) subpruebas de PedsQL® forma infantil en el grupo de ejercicio de pilates clínico, mientras que hubo una diferencia estadísticamente significativa en la destreza manual (p = 0,024), la velocidad y la agilidad en la carrera (p = 0,041) y la coordinación de las extremidades superiores (p = 0,034) subpruebas de BOT-2 SF y forma parental de JAB-Q (p = 0,041) en el grupo de ejercicio en casa. Cuando se compararon los valores delta, la diferencia fue significativa en la subprueba de coordinación de los miembros superiores de BOT-2 SF (p = 0,008) y en la subprueba de actividades diarias de la forma infantil de PedsQL® (p = 0,003) a favor del grupo de ejercicio de pilates clínico. Conclusiones: Los ejercicios de pilates clínico son un método seguro y útil para niños/adolescentes con AIJ.

Juvenile Behçet's disease is a rare and severe disease of childhood characterized by a chronic inflammatory vasculitis. The aim of the present study is reporting demographic, clinical and therapeutic outcomes of juvenile Behçet's disease in a tertiary center.The retrospective study included patients who were diagnosed Behçet's disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded.Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone.In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death. Key Points • In the present study, there were acceptable permanent neurological involvements as morbidity and no mortality. • It is important noticing and managing jBD in early phase in order to prevent the devastating results. • The awareness of jBD provides timely treatment of patients. • The positivity of family history and HLA B51 should alert the clinician about the incomplete cases.

Amaç: Bu çalışmada, juvenil dermatomiyozit (JDM) tanısı ile takip edilen çocukların klinik özelliklerinin, laboratuvar bulgularının ve prognozunun değerlendirmesi amaçlanmıştır.Gereç ve Yöntemler: Ocak 2005-Nisan 2021 tarihleri arasında merkezimiz Çocuk Romatoloji Kliniği’nde JDM tanısı alan, en az 6 ay izlemine devam edilen, 17 çocuğun tıbbi kayıtları geriye dönük olarak değerlendirildi. Bulgular: JDM tanılı 17 hastanın 10’u (%59) kızdı. Hastaların ortanca yaşı 8 (1.5-14)’dü. Tanıdan önceki semptom süresi ortanca 2.5 (1-36) ay; ortalama takip süresi ortanca 24 (6-156) aydı. İlk başvuruda hastaların 15’inde (%88.2) cilt bulgusu, 14’ünde (%82.4) proksimal kas güçsüzlüğü tespit edildi. Başvuruda hastaların 8’inde (%47) eritrosit sedimantasyon hızı ve C-reaktif proteinde yükseklik saptanırken 12’sinde (%70.6) laktat dehidrogenaz, 12’sinde (%70.6) aspartat aminotransferaz, 13’ünde (%76.5) alanin aminotransferaz, 9’unda (%53) kreatinin kinaz yüksekliği saptandı. Miyozit spesifik antikorlar; 5/17 hastada çalışıldı; 4 hastada pozitif bulundu. Kas biyopsisi 6 hastaya yapıldı ve inflamatuar miyozit ile uyumlu bulundu. On üç hastaya ekstremite manyetik rezonans görüntüleme yapıldı ve 10 hastada aktif miyozit gösterildi. Tüm hastalara başlangıç tedavisi olarak steroid ve eş zamanlı olarak metotreksat başlandı. Üç hastada metotreksat yan etkisi nedeni ile mikofenolat mofetil/ siklosporin A verildi. İki hastada kalsinozis gelişti. Son takipte 13 hastanın steroid tedavisi kesilebildi. Bir hasta immünsüpresif ajan olmaksızın, 16 hasta immünsüpresif ajanlarla remisyondaydı. İki hasta 18 yaşın üzerinde olduğu için erişkin romatoloji kliniğine devredildi.Sonuç: JDM çocukluk çağında nadir görülen bir hastalık olmasına rağmen kalsinozis ve vaskülopatiye ikincil ciddi komplikasyonlara neden olabilir. JDM’li tüm çocuklar yakından takip edilmelidir., Objective: The aim of this study is to evaluate the clinical features, laboratory findings and prognosis of children followed up with a diagnosis of juvenile dermatomyositis (JDM).Material and Methods: The medical records of 17 children who were diagnosed with JDM in the Pediatric Rheumatology Clinic of our center between January 2005 and April 2021 and were followed up for at least 6 months were evaluated retrospectively. Results: Ten of the 17 patients (59%) with a diagnosis of JDM were girls. Median age was 8 (1.5-14). Median duration of symptoms before diagnosis was 2.5 (1-36) months; the median follow-up time was 24 (6-156) months. Skin findings were detected in 15 (88.2%) patients and proximal muscle weakness in 14 (82.4%) patients at the first admission. Erythrocyte sedimentation rate and C-reactive protein elevation were detected in 8 (47%) patients at admission, while 12 (70.6%) had lactate dehydrogenase, 12 (70.6%) aspartate aminotransferase, and 13 (76.5%), alanine aminotransferase and 9 (53%) increased creatine kinase. Myositis-specific antibodies; assessed in 5/17 patients; found positive in 4 patients. Muscle biopsy was performed on 6 patients and was found to be compatible with inflammatory myositis. Magnetic resonance imaging of the limbs was performed in 13 patients and active myositis was demonstrated in 10 patients. Steroid and simultaneous methotrexate were started as initial therapy in all patients. Mycophenolate mofetil/cyclosporine A was given to three patients because of the side effect of methotrexate. Calcinosis developed in two patients. Steroid therapy could be discontinued in 13 patients at the last follow-up. One patient was in remission without an immunosuppressive agent, and 16 patients were in remission with immunosuppressive agents. Two patients were transferred to the adult rheumatology clinic because they were over 18 years old.Conclusion: Although JDM is a rare disease in childhood, it can cause serious complications secondary to calcinosis and vasculopathy. All children with JDM should be closely monitored.

AMAÇ: İmmünglobulin A vasküliti (IgAV), çocukluk döneminin en sık görülen küçük damar vaskülitidir. Alt ekstremitelerde görülen palpabl purpura karakteristik özelliğidir. Aynı zamanda eklem ağrısı, artrit, gastrointestinal sistem (GİS) ve üriner sistem etkilenmesi oldukça sıktır. Özellikle hastaneye yatışın büyük kısmı GİS tutulumu nedeni ile olmaktadır. Bu çalışmada IgAV’nde GİS tutulumu için risk faktörleri belirlenmeye çalışıldı. GEREÇ ve YÖNTEM: Eylül 2015-Ağustos 2020 yılları arasında hastanemizin çocuk romatoloji kliniğinde IgAV tanısı ile izlenen GİS tutulumu olan ve olmayan toplam 100 hastanın dosyası retrospektif olarak incelendi. Klinik, demografik, laboratuar parametreleri ve uygulanan tedaviler kaydedildi. BULGULAR: 100 (49 K/51 E) hastanın yarısı GİS tutulumu olan ve diğer yarısı GİS tutulumu olmayan hastalardan seçildi. Ortanca tanı yaşı 8 (6,1-9,8) yıldı. Tüm hastalarda palpabl purpura mevcuttu. En sık görülen diğer bulgular artrit (%34) ve artralji (%52) idi. GİS tutulumu olan hastaların tamamında karın ağrısı mevcuttu. Aktif kanama GİS tutulumu olan hastaların %25’inde (n=12) vardı. GİS tutulumu olan ve olmayan hastalar arasında yaş, cinsiyet, diğer klinik bulgular, mevsimsel farklılıklar, tanı anındaki laboratuar parametreleri arasında herhangi bir ilişki saptanmadı. Ancak MEFV gen mutasyonu varlığı, ağır GİS tutulumu olan grupta daha sık tespit edildi (p=0,009). Renal tutulum hastaların %18’inde gelişti. Hastaların tamamı komplikasyonsuz iyileşti. SONUÇ: IgAV’nde GİS tutulumu oldukça sıktır. Bu çalışmada GİS tutulumunda yaş, cinsiyet, diğer klinik bulguların dağılımı, mevsimler gibi farklılıkların GİS tutulumu için belirleyici faktör olmadığını gösterdik. Ancak ağır GİS tutulumu olan hastalarda MEFV gen mutasyonlarının katkısı olabileceği göz önünde bulundurulmalıdır. Abstract AİM: Immunoglobulin A vasculitis (IgAV) is the most common small vessel vasculitis in childhood. It is characteristic of palpable purpura seen in the lower extremities. Also, joint pain, arthritis, gastrointestinal system (GI) and urinary system involvement are quite common. Especially most hospitalizations are due to GI involvement. In this study, it was aimed to determine the risk factors for GI involvement in IgAV. MATERIALS AND METHODS: The files of 100 patients with and without GI involvement who were followed up with a diagnosis of IgAV in the pediatric rheumatology department of our hospital between September 2015 and August 2020 were retrospectively analyzed. Clinical, demographic, laboratory parameters and applied treatments were recorded. RESULTS: Half of the 100 (49 F/51 M) patients were selected from patients with GI involvement and the other half from patients without GI involvement. The median age of diagnosis was 8 (6.1-9.8) years. All patients had palpable purpura. The other most common findings were arthritis (34%) and arthralgia (52%). All patients with GI involvement had abdominal pain. Active bleeding was present in 25% (n = 12) of the patients with GI involvement. No relationship was found between patients with and without GIS involvement, between age, gender, other clinical findings, seasonal differences, and laboratory parameters at the time of diagnosis. However, presence of MEFV gene mutation was detected more frequently in the group with severe GI involvement (p = 0.009). Renal involvement developed in 18% of the patients. All the patients recovered without any complications. CONCLUSION: GI involvement is very common in IgAV. In this study, we showed that differences in GI involvement such as age, gender, distribution of other clinical findings, and seasons are not determinative factors for GI involvement. However, it should be kept in mind that MEFV gene mutations may contribute to patients with severe GI involvement. Key words: IgA vasculitis, gastrointestinal system, risk, MEFV gene

OBJECTIVES: The aims of this study were to evaluate the role of biological agents in the treatment of severe multisystem inflammatory syndrome in children (MIS-C) and to assess the current application, outcomes, and adverse effects in patients who are followed up in a pediatric intensive care unit (PICU). PATIENTS AND METHODS: This observational, descriptive, medical records review study was performed on patients with MIS-C admitted to the PICU between September 1 and November 1, 2020. Through medical records review, we confirmed that patients were positive for current or recent SARS-CoV-2 infection or for COVID-19 exposure history within the 4 weeks before the onset of symptoms. RESULTS: A total of 33 patients with severe MIS-C were included (21 male) with a median age of 9 years. The most common signs and symptoms during disease course were fever (100%) and abdominal pain (75.5%). Clinical features of 63.6% patients were consistent with Kawasaki disease/Kawasaki disease shock syndrome, and 36.4% were consistent with secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Myocardial dysfunction and/or coronary artery abnormalities were detected in 18 patients during the PICU stay. Intravenous immunoglobulin and corticosteroids were given to 33 patients. Anakinra was administered to 23 patients (69.6%). There was a significant increase in lymphocyte and platelet counts and a significant decrease in ferritin, B-type natriuretic peptide, and troponin levels at the end of the first week of treatment in patients who were given biological therapy. Two patients were switched to tocilizumab because of an insufficient response to anakinra. The mortality rate of MIS-C patients admitted in PICU was 6.0%. CONCLUSIONS: Management of systemic inflammation and shock is important to decrease mortality and the development of persistent cardiac dysfunction in MIS-C. The aggressive treatment approach, including biological agents, may be required in patients with severe symptoms and cardiac dysfunction.

Objective Familial Mediterranean fever is the most common auto-inflammatory disease in childhood. The aim of present study is to assess whether familial Mediterranean fever increases the risk of premature atherosclerosis. Materials and methods It is a case-control prospective study. The study included 32 patients in attack-free period and 22 healthy children who did not have any chronic disease. Demographic features, clinical findings, response to colchicine therapy, Mediterranean Fever gene mutations were recorded in familial Mediterranean fever group. Mean platelet volume and intima-media thickness were evaluated as a possible marker of early onset atherosclerosis. Intima media thickness of abdominal aorta and common carotid arteries with color Doppler ultrasound, mean platelet volume and acute phase reactants were measured in both groups. Results No significant difference was documented between the patient and control groups regarding the intima-media thickness (p>0, 05). Familial Mediterranean fever patients had significantly higher mean platelet volume values compared with the controls (8.35±1.0 vs. 7.79±0.78 fl, p=0.026). Intima media thickness and mean platelet volume values of familial Mediterranean fever patients were not correlated with lipid profiles, inflammatory markers. Conclusions The present study found out no evidence that intima media thickness of abdominal aorta and common carotid arteries in familial Mediterranean fever patients could predict early atherosclerosis. However, mean platelet volume value was found higher in children with familial Mediterranean fever. Mean platelet volume may be useful marker to show premature atherosclerosis in familial Mediterranean fever.

BACKGROUND Diffuse fasciitis with psoriatic arthritis on magnetic resonance imaging (MRI) has not been previously described in childhood. Here we present the first case report of a pediatric patient developing fasciitis, beyond plantar fasciitis, with psoriatic arthritis. CASE SUMMARY An 11-year-old female was admitted with the complaints of psoriatic rash on the body associated with severe pain in the lower extremities and arthritis in the right knee. Psoriasis was confirmed by skin biopsy, she diagnosed with juvenile psoriatic arthritis. Diagnostic tests did not indicate any pathology except MRI. MRI of the femur and tibia revealed that high-signal inflammatory changes in the subdermal fascia. These findings led to a diagnosis of psoriatic fasciitis. Methotrexate was given for 3 mo but the patient showed no response to therapy; therefore, etanercept was added. However, there was no response to treatment. Etanercept was switched to adalimumab at the sixth month of therapy. Clinical improvement started with therapy of adalimumab within one month. Fasciitis finding in MRI disappeared at seventh months on adalimumab therapy. She has no complaint for two years with adalimumab. CONCLUSION The most effective imaging method is MRI and adalimumab may be the best choice of treatment for psoriatic fasciitis. © The Author(s) 2019.

AIMS: Recently, multisystem inflammatory syndrome in children (MIS-C) has been recognized in association with coronavirus disease 2019 as a cytokine storm syndrome. MIS-C presents with symptoms similar to Kawasaki disease and macrophage activation syndrome (MAS). We aimed to better understand this cytokine storm syndrome by comparing the initial laboratory findings of MIS-C and MAS. METHODS: Patients who were diagnosed with MAS due to systemic juvenile idiopathic arthritis in our clinic between March 2002 and November 2020 and with MIS-C between 20 September and 20 October 2020 were enrolled into the study. The medical files of all patients were reviewed retrospectively. RESULTS: A total of 13 MAS (9 boys, 4 girls) and 26 MIS-C (16 boys,10 girls) patients were included in the study. Hemoglobin, absolute neutrophil and lymphocyte counts, C-reactive protein (CRP), ferritin, fibrinogen and lactate dehydrogenase (LDH) levels showed significant differences between the two groups (P

TYPE OF THE ARTICLE: LETTERTITLE: BIOLOGICAL THERAPIES IN CHILDREN WITH RHEUMATIC DISEASES DURING THE COVID-19 PANDEMIC: A SINGLE CENTER EXPERIENCERUNNING TITLE: BIOLOGICAL THERAPIES DURING THE COVID-19 PANDEMIC