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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.

Pediatric mixed connective tissue disease (MCTD) is a subgroup of overlap syndromes. We aimed to compare the characteristics and outcomes in children with MCTD and other overlap syndromes. All MCTD patients met either Kasukawa or Alarcon-Segovia and Villareal criteria. The patients with other overlap syndromes had the features of ≥ 2 autoimmune rheumatic diseases but did not meet MCTD diagnostic criteria. Thirty MCTD (F/M = 28/2) and thirty (F/M = 29/1) overlap patients were included (disease onset < 18 years). The most prominent phenotype at disease onset and the last visit was systemic lupus erythematosus (SLE) in the MCTD group; juvenile idiopathic arthritis and dermatomyositis/polymyositis, respectively, in the overlap group. At the last visit, systemic sclerosis (SSc) phenotype was more frequent among MCTD than overlap patients (60% vs. 33.3%; p = 0.038). The frequency of the predominant SLE phenotype had decreased (60% to 36.7%), while predominant SSc phenotype had increased (13.3% to 33.3%) during follow-up in MCTD patients. Weight loss (36.7% vs. 13.3%), digital ulcers (20% vs. 0), swollen hands (60% vs. 20%), Raynaud phenomenon (86.7% vs. 46.7%), hematologic involvement (70% vs. 26.7%), and anti-Sm positivity (29% vs. 3.3%) were more common, while Gottron papules (16.7% vs. 40%) were less frequent among MCTD than overlap patients (p < 0.05). A higher percentage of overlap patients achieved complete remission than MCTD patients (51.7% vs. 24.1%; p = 0.047). The disease phenotype and outcome differ between pediatric MCTD and other overlap syndromes where MCTD may be regarded as a more severe disease. Analyzing these patients could pave the way for early and effective treatment.

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.

ObjectivesBehçet’s disease (BD) is a multisystem disease and frequently occurs during the second–fourth decades of life, although disease onset may be seen at any age. This study aimed to analyze the influence of the age of onset on clinical manifestations of BD.Materials and methodsThis retrospective study analyzed two cohorts (paediatric and adult) to determine the association between the age of onset and clinical features in BD. Patients were classified into four groups to analyze the clinical characteristics according to the age of fulfilling the BD diagnostic criteria as follows: childhood onset (40 years).ResultsThe study included 801 patients with BD. Male predominance, pathergy test positivity, aphthosis (oral or genital), and skin and ocular involvements were higher among adult patients than paediatric patients. The presence of positive family history for BD, neuro-BD, and epididymitis were observed significantly common in the paediatric group.ConclusionThere may be differences in clinical manifestations with regard to the age of disease onset. Disease presentations may differ from adult patients, and clinicians should be aware of the high familial aggregation rate of BD, especially in countries where the disease is endemic.

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.

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.

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.

Cardiac involvement is a common and serious problem in multisystem inflammatory syndrome in children (MIS-C). Echocardiographic evaluation of systolic and diastolic function by traditional, tissue Doppler and three-dimensional (3D) echocardiography was performed in consecutive 50 MIS-C patients during hospitalization and age-matched 40 healthy controls. On the day of worst left ventricular (LV) systolic function (echo-1), all left and right ventricular systolic function parameters were significantly lower (p

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.

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.

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.

A 16-year-old girl was admitted to the hospital for the absence of a menstrual bleeding over 10 mounts. She has had regular menstrual periods since she was 13 years old and no history of systemic disease. Laboratory tests showed low estrogen (

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.

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.

Intra-articular corticosteroid injection (IACI) is generally used in the management of juvenile idiopathic arthritis (JIA) to obtain rapid relief of active synovitis and functional recovery and to prevent the need for regular systemic therapy. The aim of this study was to investigate the outcome of IACI treatment and the factors associated with remission of synovitis.The clinical records of JIA patients who received IACI between January 2014 and December 2020 in two pediatric rheumatology centers were reviewed. The procedure was evaluated in terms of efficacy, factors that may affect the duration of remission, procedural and drug-related complications.During the study period, 134 patients received 227 injections and 37 joints were injected more than once. One hundred and six (79%) patients had persistent oligoarticular disease. At the time of injection, all patients were receiving non-steroidal anti-inflammatory drugs, 74 patients were on methotrexate, and 14 patients were on biologics. The median duration of remission without exacerbation of synovitis treated with IACI was 15 (range 1-64) months. The inactivity rate was 81% at the 6th month after the injection. It has been shown that being less than 7 years old at disease onset and low initial CRP levels were correlated with a long remission period (p 0.05). Despite the differences were not statistically significant, the duration of remission was longer in boys, in ANA positives, in HLA-B27 negatives, in patients with concurrent methotrexate treatment and in patients not receiving biologic therapy (p 0.05). Only two patients (1.5%, 95% CI - 0.6 to 3.5) developed cutaneous hypopigmentation and subcutaneous atrophy as side effects of injection.Intra-articular corticosteroid injection was more effective especially in patients with low initial CRP levels and younger than 7 years of age. The duration of remission was longer in these patients. Key Points • Intra-articular corticosteroid injection is an effective method for controlling joint inflammation and achieving long-term remission without significant side effects. • Intra-articular corticosteroid injection can be preferred in all forms of juvenile idiopathic arthritis as primary therapy or to relieve the patient while waiting for the effect of systemic agents or to avoid increasing the dose of systemic drugs. • It can be recommended as a treatment option at any stage of the disease, especially in young patients and patients with low initial CRP values.

Immunoglobulin A vasculitis with nephritis (IgAVN) is the most serious complication affecting long-term prognosis. Understanding the risk factors and markers for the development of IgAVN is essential. The aim of this study is to identify IgAVN-associated factors and to evaluate the usability of Pediatric Vasculitis Activity Score (PVAS) at diagnosis as an early marker for the development of IgAVN.We conducted a retrospective case-control study of 314 patients divided into two groups: those with nephritis (IgAVN) and without nephritis (non-IgAVN). The groups were compared in terms of clinical symptoms, laboratory values, and PVAS values.In total, 18.5% of the patients had IgAVN; they were older than the non-IgAVN patients (median age was 8.8, p 0.05). Arthritis/arthralgia, abdominal pain, and intestinal bleeding were more common, systolic and diastolic BP were higher in IgAVN (p 0.05). CRP, serum creatinine, and urine protein/Cr, PVAS were higher, while serum albumin was lower in IgAVN (p 0.05). The receiver operator characteristic curve (ROC) analysis showed that IgAV patients with a determined cut-off PVAS value greater than 3 had 70.7% sensitivity in predicting whether or not they would develop IgAVN. Logistic regression analysis found that PVAS 3 and low serum albumin at the time of diagnosis were independent risk factors for IgAVN.Our study revealed that PVAS 3 at diagnosis is an independent predictor of IgAVN. Patients with PVAS 3 should be followed more closely to ensure early diagnosis and management of IgAVN. A higher resolution version of the Graphical abstract is available as Supplementary information.

Background Although not validated fully, recommendations are present for diagnosis, screening and treatment modalities of patients with familial Mediterranean fever (FMF). Objective To review the current practices of clinicians regarding FMF and reveal their adherence to consensus guidelines. Methods Fifteen key points selected regarding the diagnosis and management of FMF were assessed by 14 paediatric rheumatologists with a three-round modified Delphi panel. Results Consensus was reached on the following aspects: genetic analysis should be ordered to all patients when clinical findings support FMF, but its result is not decisive alone. In the absence of clinical features, colchicine should be commenced when two pathogenic alleles and family history of amyloidosis are present. Serum amyloid A testing at each visit is recommended in patients resistant to colchicine, with subclinical inflammation and family history of amyloidosis. Consensus was reached on both the definition of colchicine resistance and starting biologic in resistant cases. Cost, efficiency, ease of use, treatment adherence, accessibility and emergence of adverse events are the factors affecting the choice of biologic agents. In patients without any attack and evidence of subclinical inflammation within the last 6 months following initiation of biologics, treatment dose intervals can be prolonged. Conclusion A consensus was achieved regarding the routine diagnosis and screening and treatment of FMF patients. The definition of colchicine resistance was made and a protocol was created for prolongation of treatment intervals of biologic agents. We anticipate that the results of the study reveal real-life data on the approach to patients in clinical practice. Supplementary Information The online version contains supplementary material available at 10.1007/s00296-020-04776-1.

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.

OBJECTIVE: Although the initial reports of coronavirus disease (COVID-19) cases in children described that children were largely protected from severe manifestations, clusters of pediatric cases of severe systemic hyperinflammation and shock related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection began to be reported in the latter half of April 2020. A novel syndrome called "multisystem inflammatory syndrome in children" (MIS-C) shares common clinical features with other well-defined syndromes, including Kawasaki disease (KD), toxic shock syndrome (TSS), and secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (MAS). Our objective was to develop a protocol for the evaluation, treatment, and follow-up of patients with MIS-C. METHODS: The protocol was developed by a multidisciplinary team. We convened a multidisciplinary working group with representation from the departments of pediatric critical care, cardiology, rheumatology, surgery, gastroenterology, hematology, immunology, infectious disease, and neurology. Our protocol and recommendations were based on the literature and our experiences with multisystem inflammatory syndrome in children. After an agreement was reached and the protocol was implemented, revisions were made on the basis of expert feedback. CONCLUSION: Children may experience acute cardiac decompensation or other organ system failure due to this severe inflammatory condition. Therefore, patients with severe symptoms of MIS-C should be managed in a pediatric intensive care setting, as rapid clinical deterioration may occur. Therapeutic approaches for MIS-C should be tailored depending on the patients' phenotypes. Plasmapheresis may be useful as a standard treatment to control hypercytokinemia in cases of MIS-C with severe symptoms. Long-term follow-up of patients with cardiac involvement is required to identify any sequelae of MIS-C.

Öz: Amaç: Bu çalışmada, immunglobulin A (IgA) vasküliti tanısı ile takip edilen çocukların klinik özelliklerinin, laboratuvar bulgularının, tedavilerinin ve prognozunun değerlendirmesi amaçlanmıştır.Gereç ve Yöntem: 2018-2020 tarihleri arasında merkezimiz Çocuk Romatoloji Kliniği'nde IgA vasküliti tanısı alan, en az 6 ay izlemine devam edilen, 139 çocuğun tıbbi kayıtları geriye dönük olarak değerlendirildi. Sonuç: 139 hastanın 75’i kızdı (%53,9). Kız/erkek oranı 1,17 olarak saptandı. Hastaların ortalama yaşı 7,6±2,9 yıl olarak tespit edildi. Hastalığın başlamasından önce hastaların 118’inde (%84) geçirilmiş üst solunum yolu enfeksiyon öyküsü mevcuttu. Klinik bulgulardan döküntü, hastaların tamamında mevcuttu. Hastaların 91’inde (%65,5) cilt altı ödem, 89’unda (%64) artralji; 68’inde (%48,9) artrit, 54’ünde (%38,8) karın ağrısı, 30’unda (%21,5) renal tutulum, 6’sında (%4.3) testis tutulumu tespit edildi. Laboratuar bulguları değerlendirildiğinde hastaların 28’inde (% 20,1) lökositoz ve trombositoz, 29’unda (%20,8) akut faz reaktanlarında yükseklik tespit edildi. İzlemde 30 hastada renal tutulum gelişti. Renal tutulum olan 9 hasta (%30) tedavisiz izlenirken, 18’ine diğer sistem tutlumları nedeni ile nonsteroid antiinflamatuvar ilaç, 3’üne (%10) renal tutulum için steroid tedavisi verildi. Dört hasta (1 makroskobik hematüri, 3 nefrotik proteinüri) çocuk nefroloji bölümüne yönlendirildi. Hastaların 30’u (%21,5) tedavisiz izlenirken 56 hastaya (%40,2) non-steroidal antiinflamatuar ilaç (NSAİİ), 51 hastaya (%36,6) steroid tedavisi verildi. Takipte 17 (%12,2) hastada hastalık tekrarladı. Sonuç: IgA vasküliti cilt ve eklemler başta olmak üzere birçok organ ve sistemi tutabilen bir vaskülittir, hastaların çoğu sekelsiz iyileşmektedir. Bununla beraber erken ve geç dönemde gelişebilecek gastrointestinal ve böbrek tutulumu gibi komplikasyonlar açısından hastaların yakın takip edilmesi gerekmektedir., The aim of this study was to evaluate the clinical characteristics, laboratory findings, treatment and prognosis of children with immunoglobulin A (IgA) vasculitis diagnosis. The medical records of 139 children who were diagnosed with IgA vasculitis in our center's Pediatric Rheumatology Clinic between 2018-2020, who continued follow-up for at least 6 months, were retrospectively evaluated. 75 of 139 patients were girls (53.9%). The male / female ratio was 1.17. The average age of the patients was 7.6±2.9 years. Prior to the onset of the disease, 118 (84%) of patients had a history of upper respiratory tract infection. From clinical findings, the rash was present in all patients. Subcutaneous edema in 91 (65.5%), arthralgia in 89 (64%); arthritis was detected in 68 (48.9%), abdominal pain in 54 (38.8%), renal involvement in 30 (21.5%), testicular involvement in 6 (4.3%) of the patients. In the laboratory evaluation, leukocytosis and thrombocytosis were detected in 28 (20.1%), and increased in acute phase reactants in 29 (20.8%) of the patients. During the follow-up, renal involvement developed in 30 patients. Nine patients (30%) with renal involvement were followed without treatment, 18 were given nonsteroidal anti-inflammatory drugs for other system involvement, and steroid treatment was given to 3 (10%) for renal involvement. Four patients (1 macroscopic hematuria, 3 nephrotic proteinuria) were referred to the pediatric nephrology department. Thirty (21.5%) of the patients were followed without treatment, 56 patients (40.2%) were given non-steroidal anti-inflammatory drugs, and 51 (36.6%) patients were treated with steroids. The disease recurred in 17 (12.2%) patients during follow-up. IgA vasculitis is a vasculitis that can involve many organs and systems, especially the skin and joints, and most of the patients recover without sequelae. However, patients should be followed up closely in terms of complications such as gastrointestinal and kidney involvement that may develop in the early and late stages.

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: Sinus vein thrombosis often presents to the emergency department with headache and is a rare disease among all strokes. Epidemiological data on sinus vein thrombosis are insufficient. Our aim in this study is to examine the findings of patients with sinus vein thrombosis who applied to our emergency department. Material and Method: 267 patients with suspected sinus vein thrombosis in patients who came to our emergency department between 2013-2018 were included in the study. Patients were divided into 2 groups as present and without sinus vein thrombosis. Women were divided into 3 subgroups as not pregnant, pregnant and postpartum period. Demographic data, comorbid diseases, hemogram parameters and radiological data of the patients were recorded in a form. The relationship of the data with sinus vein thrombosis was examined. Results: Sinus vein thrombosis was detected in 19.48% of the patients. 76.3% of the patients were women. 65.4% of them were between the ages of 18-40. The most common complaint was headache with 51.9%. And most of the patients’ neurological examinations were normal. We found that hemoglobin

Objective In the second half of April 2020, a new syndrome associated with SARS-CoV-2 infection,”multisystem inflammatory syndrome in children” (MIS-C), was defined by the World Health Organization.However, the risk factors that predispose some children to develop this inflammatory response are poorly understood .Determining the clinical risk factors of MIS-C is important in preventing undesirable complications such as death in children. Methodology In this study, we aimed to investigate the effect of ABO blood groups, hematological parameters (white blood cell, absolute neutrophil, absolute lymphocyte, platelet count, prothrombin time, activated partial thromboplastin time), cardiac parameters (troponin, brain natriuretic factor, electrocardiography) of patients diagnosed with MIS-C in Ankara City Hospital during the pandemic shortening fraction, ejection fraction), infectious parameters (c-reactive protein, interleukin-6, sedimentation) were analyzed retrospectively. Results Of our 89 cases, 49 (55.1%) were group A, 3 (3.4%) were group AB (3.4%), and 11 (12.4%) were group B. 60 of our patients presented with cardiac involvement, 14 with acute abdomen, 1 with seizure, and 1 with acute kidney injury. In clinically severe cases, MPV was higher and platelet count was lower. O blood group were diagnosed with MISC at a later age. Patients with A blood group have a statistically significantly less serious course compared to other blood groups. Conclusion In our study, we found that individuals with A blood group had MISC more frequently than other blood groups, and MISC was less severe in these patients compared to other blood groups.