Search

To evaluate the efficacy and safety of anti-interleukin (IL)-6 receptor antibody tocilizumab (TCZ) as a treatment option of juvenile systemic sclerosis (JSS). Nine JSS patients were assigned to a TCZ, additionally to conventional treatment (steroids, methotrexate, mycophenolate-mofetil). The modified Rodnan skin score (mRSS), carbon-monoxide diffusion capacity (DLCO), thorax high-resolution tomography (HRCT), patient global assessment (PGA) and Juvenile Systemic Sclerosis Severity (J4S) score were used to explore the efficacy of treatment. Nine JSS patients were treated with TCZ with a median treatment duration of 10 (1-21) months. Nine patients (77.8%) had radiologically confirmed improvement on thorax HRCT, 7 (77.8%) had decreased PGA (mean pre-treatment PGA 3.7 vs. 2.3 post-treatment PGA 2), 6 (66.7%) had increased DLCO (mean pre-treatment DLCO 69.14% vs. post-treatment DLCO 79.50%) after the TCZ treatment. In all patients mRSS and the J4S decreased: 26.1 vs. 19.7 and 8.2 vs. 4.7, respectively. Changes in mRSS, DLCO, PGA and J4S were statistically significant: p = 0.012, 0.04, 0.026 and 0.007, respectively. All patients tolerated well TCZ treatment. JSS is a rare condition characterized with skin fibrosis and internal organ involvement. Tocilizumab represents a potential treatment option for patients unresponsive to conventional treatment. Long-term prospective studies with higher number of patients are needed to provide more relevant data.

Objectives: Multi-system inflammatory syndrome in children (MIS-C) is a less understood and a rare complication of coronavirus disease-2019 (COVID-19). Given the scarce data regarding this novel disease, we aimed to describe the clinical features and outcomes of our patients with MIS-C and to evaluate the associated factors for the pediatric intensive care unit (PICU) admission. Methods: The MIS-C patients under 18 years old diagnosed and treated in three referral centers between July 2020 and March 2021 were included. Data of the patients were retrospectively obtained from their medical records. Results: Overall, 76 subjects (24 females) with a mean age of 8.17 ± 4.42 years were enrolled. Twenty-seven (35.5%) patients were admitted to the PICUs. The two most common systemic involvement patterns were cardiac and gastrointestinal. There was only one lethal outcome in a patient with underlying acute lymphoblastic leukemia. Those with higher procalcitonin levels at admission were found to stay longer in the hospital (r = 0.254, p = 0.027). The risk of PICU admission increased with age (aOR: 1.277; 95% CI: 1.089–1.498; p = 0.003) and with decreased initial serum albumin levels (aOR: 0.105; 95% CI: 0.029–0.378; p = 0.001). Conclusion: Although there is a wide clinical variability among the patients with MIS-C, we suggest that those with older age and lower initial serum albumin levels merit close monitoring due to their higher risk for PICU admission.Key Points• Although there is a wide variability regarding the management process among clinicians, MIS-C is a rare, severe, less understood complication of COVID-19 that may cause rapid clinical deterioration in the patients.• Clinicians should be aware of this condition in children with persistent fever and a family history of COVID-19.• Older age and low serum albumin levels are the independent predictors for the pediatric intensive care unit admission among MIS-C patients. © 2021, International League of Associations for Rheumatology (ILAR).

Background In the pediatric age, anti-nuclear antibody (ANA) testing is most commonly ordered by general pediatricians to evaluate children with musculoskeletal system complaints. Given the limited utility of the test, we aimed to estimate the effectivity of ordering ANA testing in childhood. Methods Children referred to our department to be examined due to ANA positivity between 2008 and 2020 were included in the study. Those with less than one-year follow-up period, those with previously known rheumatic or autoimmune disease, and those diagnosed as an autoimmune and/or rheumatic disease at the first visit were excluded. Data were obtained from their medical records, retrospectively. The parents of all of the patients were called via phone, data were verified, and missing information was collected. Results Three hundred and fifty-eight patients (230 females) were eligible for the study. The median age at ANA positivity was found is 9.31 (1.3-17.86) years old and the median follow-up duration was 4.85 (1-11.91) years. Most of the patients had no underlying disease (n=337, 94.1%). The most common reason for ordering ANA testing was to evaluate musculoskeletal system symptoms (n=225, 62.8%). None of our patients referred to us due to ANA positivity developed any autoimmune conditions or ANA associated rheumatic disease. Hypermobility syndrome is the most common final diagnosis among our ANA-positive patients CONCLUSION: We suggest that instead of using it as a screening tool, ANA testing should be performed if only there is a strong suspicion of autoimmune diseases or certain rheumatic conditions such as systemic lupus erythematosus.

YILDIZ, Mehmet/0000-0002-7834-4909; Sahin, Sezgin/0000-0002-5365-3457; Kasapcopur, Ozgur/0000-0002-1125-7720; Haslak, Fatih/0000-0002-6963-9668; Barut, Kenan/0000-0001-8459-2872 WOS:000548231000002 PubMed ID: 32661928 Concerns regarding the comorbidity as a significant risk factor for Coronavirus Disease-2019 (COVID-19), gave rise to an urgent need for studies evaluating patients with chronic conditions such as autoinflammatory diseases (AIDs). We prepared a web-based survey investigating the clinical findings and contact histories among pediatric patients with AIDs. Confirmed COVID-19 cases, patients with contact history and those with symptoms which were highly suggestive of COVID-19 were called via phone or recruited to a video or face to face appointment. Data of AIDs were obtained from their medical records, retrospectively. Laboratory and screening findings were confirmed by our national health registry website. There were 404 patients (217 female) eligible for the enrollment. During pandemic, 375 (93%) were on colchicine treatment and 48 (11.8%) were receiving biologic treatment. Twenty-four out of 404 patients were admitted to hospital due to COVID-19 suspicion. Severe acute respiratory syndrome coronavirus-2 (SARS CoV-2) was identified through rhinopharyngeal swabs in seven patients, six of whom were only on colchicine treatment. Only one patient with no finding of any severe respiratory complications was hospitalized. All of seven patients recovered completely. Among patients on biologic drugs, neither a symptom nor a positive polymerase chain reaction test for COVID 19 was detected. In conclusion, pediatric patients with AIDs, those receiving biologic treatment and/or colchicine, may not be at increased risk for neither being infected nor the severe disease course.

Since concerns have been raised that comorbidity is a significant risk factor for Coronavirus Disease-2019 (COVID-19), there is an urgent need to perform studies which evaluate patients with chronic diseases such as autoinflammatory diseases (AIDs). We prepared a web-based survey investigating the clinical findings and contagion histories. Patients with AIDs, were included in the study. Confirmed COVID-19 cases, patients with contact history and patients with symptoms which were highly suggestive of COVID-19 were called via phone or recruited to a video or face to face appointment. Data of AIDs were obtained from their medical records, retrospectively. Laboratory and screening findings were confirmed by using our national health registry website and they were re-examined, if required. There were 404 patients (217 female) eligible for the enrollment. During pandemic, 375 (93%) were on colchicine treatment, and 48 (11.8%) were receiving biologic treatment. 24 out of 404 patients had admission to hospital due to COVID-19 suspicion. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) were identified through rhino-pharyngeal swabs in 7 patients, 6 of whom were only on colchicine treatment. Only one patient with no finding of any severe respiratory complications has been hospitalized. All of them recovered completely. Among patients on biologic drugs, neither a symptom nor a positive polymerase chain reaction test was detected that would have been suggestive of COVID-19. In conclusion, pediatric patients with AIDs, those receive either biologic treatment or colchicine may not be at increased risk for either being infected or having worse disease course.

Background:Familial Mediterranean Fever (FMF) is the most common periodic fever syndrome in childhood with an autosomal recessive inheritance pattern and is characterized by unprovoked fever attacks, serositis episodes. The causative gene of the disease is MEFV that encodes pyrin protein. The pyrin protein takes a role in pathways related to inflammation, and mutations of it lead to increased inflammation. It is already shown that frequencies of some certain diseases like PAN, HSP increase in patients with FMF. Nevertheless, frequencies of inflammatory diseases in families of patient with FMF haven’t been investigated.Objectives:In this study, we have aimed to evaluate the comorbid disorders in a large cohort of families of patients with FMF.Methods:Four hundred and ninety-eight children with FMF, one hundred and forty patients with JIA and ninety-two healthy children were interviewed between December 2019 and January 2020. In JIA group and healthy control group, patients who have family history for FMF were excluded from the study. Patients were asked about characteristics of their disease attacks and if there is a relative with any inflammatory diseases who does not have FMF in patient’s 1stand 2th degree relatives.Results:Demographic features of study group have shown in Table 1. The most common MEFV mutations in patients with FMF were: M694V homozygotes (13.2 %), M694V heterozygotes (12 %), M694V homozygotes and R202Q homozygotes (6,8 %). Type II diabetes, asthma and hypothyroidism were the most commonly detected diseases in all cohorts. Frequency of Behçet’s disease, allergic rhinitis and type II diabetes were significantly higher in families of patients with FMF than other groups (pTable 1.Demographic features of study population.FMF†JIA††Healthy Controln: 498 (%)mean +/- SDn: 140 (%)mean +/- SDn: 92 (%)mean +/- SDFemale284 (57)91 (65)55 (59.8)Age (years)12.9 ± 8.211.7 ± 5.17.4 ± 4.6Age at Onset (years)4.3 ± 3.35.4 ± 4.1-Age at Diagnosis (years)6.3 ± 3.66.3 ± 4.5-Delay in Diagnosis (months)23.8 ± 29.211.3 ± 28.2-Follow-up Duration (years)6.9 ± 8.35.3 ± 4.0-Consanguinity100 (20)25 (17.8)8 (8.6)Family History of FMF282 (56.6)0 (0)0 (0)JIA subgroup-- Oligoarticular72 (51.4) Polyarticular (RF negative)16 (11.4) Polyarticular (RF positive)3 (2.1) Enthesitis Related Arthritis14 (10) Psoriatic Arthritis7 (5) Systemic23 (16.4) Other5 (3.5)Clinical Findings-- Fever392 (78.1) Abdominal Pain429 (86.1) Chest Pain102 (20.5) Arthralgia334 (67.1) Arthritis157 (31.5) Extremity Pain64 (12.8) Heel Pain44 (8.8) Myalgia43 (8.6) *ELE13(2.6) Serositis10 (2)†Familial Mediterranean Fever††Juvenile Idiopathic Arthritis*Erysipelas like erythemaTable 2.Comparison of frequencies of diseases detected among families of patient groups (shortened).DiseasesFMFJIAHealthy Controlp1Type II Diabetes284 (57)64(45.7)44 (47.8)0.02Asthma139 (27.9)30 (21.4)20 (21.7)0.19Hypothyroidism122 (24.4)27 (19.2)14 (15.2)0.09Eczema68 (13.6)14 (10)5 (5.4)0.06Psoriasis49 (9.8)6 (4.2)7 (7.6)0.10Allergic Rhinitis49 (9.8)3 (2.1)1 (1)0.001Hyperthyroidism40 (8)9 (6.4)3 (3.2)0.24Behçet’s Disease31 (6.2)1 (1)2 (2.1)0.01Rheumatic Fever30 (6)10 (7.1)2 (2.1)0.25Conclusion:In this study, we have reported increased frequencies of Behçet’s disease, allergic rhinitis and type II diabetes in families of patients with FMF. Our results suggest that possible increased mutation load among families of patients with FMF may cause increased inflammatory diseases.References:[1]Yildiz M, Adrovic A, Tasdemir E, et al. Evaluation of co-existing diseases in children with familial Mediterranean fever.Rheumatol Int. 2020;40(1):57–64. doi:10.1007/s00296-019-04391-9Disclosure of Interests:None declared

Background:Anti-nuclear antibodies (ANA) are a group of the antibodies that develop against intracellular components of the cells. It is usually useful for diagnosing some of the connective tissue diseases like systemic lupus erythematosus, mixed connective tissue disease. But it is reported that its positivity rate is about %20 in healthy individuals. Therefore, it can be confusing to check ANA test, if there is not really high suspicion for connective tissue diseases or juvenile idiopathic arthritis.Objectives:We aimed to evaluate results of long-term follow-up of the patients with ANA positivity who had initially no identifiable rheumatic diseases.Methods:Six hundred and ninety-four patients with ANA positivity who did not diagnosed as any of the rheumatic diseases at the first examination were found in database. Two hundred and eighty- two patients of them were called so far and questioned about their demographic features and symptoms that are related with rheumatic diseases.Results:Mean age of the patients at the time of study and at the time of testing were 13.4± 4.5 and 9.1±4.0 years. The female: male ratio was 1.05. Mean follow-up duration was 4.3±2.8 years. Most common reasons for the request for ANA test were arthralgia and skin eruptions. ANA testing was most commonly requested by a general pediatrists. Demographic features of the patients were summarized in Table 1.Table 1.Demographic features of the patients.n (%)Age (years)13.4± 4.5Female145 (51.4)Age at the time of testing (years)9.1±4.0Follow-up Duration (years)4.3±2.8Reason for testing Arthralgia99 (44.1)Skin Eruption54 (24.1)Check-Up20 (8.9)Arthritis13 (5.8)Gait abnormalities7 (3.1)Hair Loss6 (2.6)Fever5 (2.2)Uveitis2 (0.8)Recurrent abdominal pain2 (0.8)Who suggested testing?Pediatrician196 (87.5)Parents13 (5.8)Dermatologist7 (3.1)Ophthalmologist3 (1.3)Rheumatologist2 (0.8)Other3 (1.3)Positivity of acute phase reactants15 (5.3)History of infection before testing56 (24.3)History of drug-using before testing39 (17)Most of the diseases were diagnosed in patients with ANA positivity were not related with autoimmune mechanisms that associated with ANA positivity therefore, these diseases are thought to be coincidence. Only in 1 patients, systemic lupus erythematosus that has certain association with ANA positivity were diagnosed. All diseases that are diagnosed were shown in Table 2.Table 2.All diseases that are diagnosed in patients during the follow-up period.n (%)Hypermobility Syndrome29 (10.2)Urticaria7 (2.4)Hypothyroidism6 (2.1)Transient synovitis4 (1.4)Chronic ITP*4 (1.4)Scoliosis4 (1.4)Familial Mediterranean Fever3 (1)Cryopyrin associated periodic Syndrome2 (0.7)PFAPA syndrome**2 (0.7)Celiac Disease2 (0.7)Acute Rheumatic Fever2 (0.7)Fibromyalgia1 (0.3)Bone Tumor1 (0.3)Juvenile Idiopathic Arthritis1 (0.3)Henoch-Shöenlein Purpura1 (0.3)Myastenia Graves1 (0.3)Sever Disease1 (0.3)Vitiligo1 (0.3)Systemic Lupus Erythematosus1 (0.3)*Idiopathic Thrombocytopenic Purpura, **Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome.Conclusion:We are reporting that in only 0.3% of patients with ANA positivity who don’t have any diseases diagnosed initially, were diagnosed as rheumatologic diseases during to the follow-up period. Since positivity of ANA is also common in the healthy population, requesting this test in only patients with high suspicion for connective tissue disease will reduce confusion in terms of diagnosis.References:[1]Kasapcopur O, Ozbakir F, Arisoy N, Ingol H, Yazici H, Ozdogan H. Frequency of antinuclear antibodies and rheumatoid factor in healthy Turkish children. Turk J Pediatr 1999;41:67-71.Disclosure of Interests:None declared