[Clinical and imaging features of infective sacroiliitis in children].

Objective: To summarize the clinical, radiological characteristics, and prognosis of infectious sacroiliitis in children. Methods: A case-control study was conducted, including 12 cases of infectious sacroiliitis diagnosed in the Rheumatology and Immunology Department of the Children's Hospital affiliated with the Capital Institute of Pediatrics from June 2018 to June 2023. These cases comprised the case group. Concurrently, 28 cases of pediatric idiopathic arthritis involving the sacroiliac joint in the same department served as the control group. Basic patient information, clinical features, laboratory parameters, and clinical treatment outcomes for both groups were collected and analyzed. Independent sample t-tests and chi-squared tests were used for inter-group comparisons. Results: Among the 12 cases in the case group, there were 5 males and 7 females, with a disease duration of 0.8 (0.5, 1.2) months. Nine patients presented with fever, and 1 patient had limping gait. Human leukocyte antigen (HLA)-B27 positivity was observed in 1 case, and there was no family history of ankylosing spondylitis. In the control group of 28 cases, there were 19 males and 9 females, with a disease duration of 7.0 (3.0, 17.0) months. One patient (4%) had fever, and 14 cases (50%) exhibited limping gait. HLA-B27 positivity was found in 18 cases (64%), and 18 cases (64%) had a family history of ankylosing spondylitis. The case group had higher white blood cell count (WBC), neutrophil ratio, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) levels, as well as a higher proportion of unilateral involvement on magnetic resonance imaging and bone destruction on CT compared to the control group ((11.1±6.2)×109 vs. (7.3±2.3)×109/L, 0.64±0.10 vs. 0.55±0.12, 72 (34, 86) vs. 18 (5, 41) mm/1 h, 24.6 (10.1, 67.3) mg/L vs. 3.6 (0.8, 15.0) mg/L, 11/12 vs. 36% (10/28), 9/12 vs. 11% (3/28), t=2.90, 3.07, Z=-2.94, -3.28, χ2=10.55, 16.53, all P<0.05). Conclusions: Pediatric infectious sacroiliitis often presents as unilateral involvement with a short disease history. Elevated WBC, CRP, and ESR, as well as a high rate of bone destruction, are also common characteristics.

[Clinical features and prognosis of 118 children with histiocytic necrotizing lymphadenitis].

Objective: To explore the clinical features and prognosis of children with histiocytic necrotizing lymphadenitis (HNL). Methods: The clinical data of 118 children with HNL diagnosed and treated in the Department of Rheumatology and Immunology of Children's Hospital, Capital Institute of Pediatrics from January 2014 to December 2021 were retrospectively analyzed. The clinical symptoms, laboratory examination, imaging examination, pathological findings, treatment and follow-up were analyzed. Results: Among the 118 patients, 69 were males and 49 were females. The age of onset was 10.0 (8.0, 12.0) years, ranging from 1.5 to 16.0 years. All the children had fever lymph node enlargement, blood system involvement in 74 cases (62.7%), skin injury in 39 cases (33.1%). The main manifestations of laboratory examination were increased erythrocyte sedimentation rate in 90 cases (76.3%), decreased hemoglobin in 58 cases (49.2%), decreased white blood cells in 54 cases (45.8%) and positive antinuclear antibody in 35 cases (29.7%). Ninety-seven cases (82.2%) underwent B-mode ultrasound of lymph nodes, showing nodular lesions with low echo in the neck; 22 cases (18.6%) underwent cervical X-ray and (or) CT; 7 cases (5.9%) underwent cervical magnetic resonance imaging. Lymph node biopsy was performed in all 118 cases, and the pathological results did not support malignant diseases such as lymphoma or Epstein-Barr virus infection, suggesting HNL. Fifty-seven cases (48.3%) recovered without treatment, 61 cases (51.7%) received oral steroid therapy, and 4 cases (3.4%) received indomethacin as anal stopper. The 118 cases were followed up for 4 (2, 6) years, ranging from 1 to 7 years, 87 cases (73.7%) had one onset and did not develop into other rheumatological diseases, and 24 cases (20.3%) had different degrees of recurrence, 7 cases (5.9%) had multiple system injuries, and all of the tested autoantibodies were positive for medium and high titers. All of them developed into other rheumatic immune diseases, among which 5 cases developed into systemic lupus erythematosus and 2 cases developed into Sjogren's syndrome; 7 cases were given oral steroid therapy, including 6 cases plus immunosuppressant and 2 cases receiving methylprednisolone 20 mg/kg shock therapy. Conclusions: The first-onset HNL portion is self-healing, hormone-sensitive and has a good prognosis. For HNL with repeated disease and multiple system injury, antinuclear antibody titer should be monitored during follow-up, and attention should be paid to the possibility of developing into other rheumatological diseases, with poor prognosis.

[Treatment of 11 cases of juvenile idiopathic arthritis by intra-articular injection of adalimumab].

Objective: To evaluate the efficacy and safety of intra-articular injection of adalimumab (ADA) in the treatment of refractory oligoarticular juvenile idiopathic arthritis (JIA). Methods: This was a retrospective study. Clinical data on age, gender, and symptoms of joint swelling and pain were collected from 11 children with refractory oligoarticular JIA involving only knee joints admitted to Department of Rheumatism and Immunology of Children's Hospital, Capital Institute of Pediatrics from November 2019 to October 2020. The physician and parent-child evaluation of disease activity, the number of active joints, and the level of erythrocyte sedimentation rate (ESR) at different treatment time points were analyzed at every 4-week observation point after drug administration, and the non-parametric Kruskal-Wallis test was used to compare the differences in clinical evaluation indicators and changes in laboratory tests at different treatment times. The follow-up period was 6 months. Results: Among the 11 children, 5 were boys and 6 were girls. The age was 3.0 (2.8) years. All 11 children had symptoms of joint swelling and pain as well as limitation of movement. After 3 intra-articular injections of ADA, the joint symptoms of 11 children were better than before treatment; the joint symptoms of 7 children disappeared completely, and no recurrence occurred during the 6-month follow-up period. At different treatment times, physician and parent-child evaluation of disease activity, a gradual decrease in the number of active joints in the children, ESR, and juvenile arthritis disease activity score with 27 joints were all statistically significant (χ2=53.99, 59.37, 32.87, 40.07, 54.00, all P<0.001).No significant adverse drug reactions were observed in any of the 11 children during treatment and follow-up. Conclusion: Intra-articular injection of ADA in the treatment of refractory oligoarticular JIA has a significant effect in controlling joint symptoms and is relatively safe.

[Analysis of 13 cases with pediatric rheumatic disease combined with endocrine disorder].

Objective: To summarize the clinical characteristics of children with rheumatic disease combined with endocrine disorder. Methods: A retrospective analysis was performed on the clinical data, including sex, age, clinical presentation, laboratory tests, treatment and outcome, of 13 patients with rheumatic diseases combined with endocrine disorder, who were admitted to our department in Children's Hospital, Capital Institute of Pediatrics from January 2014 to December 2020. Results: Among the 13 cases, 3 were males and 10 were females, without family history. Their age was (10±4) years. And the average course of disease was 4.1 months. Eight of them were diagnosed with systemic lupus erythematosus (JSLE), 2 with juvenile idiopathic arthritis (JIA), 1 with childhood vasculitis, 1 with juvenile-onset systemic sclerosis (JSSc) and 1 had juvenile dermatomyositis (JDM). Regarding the initial presentation, 10 cases had symptoms of rheumatic disease, 2 had polydipsia and polyuria, and 1 had goiter. All the 13 patients had multiple system involvement. Regarding endocrine disorder, 10 had thyroiditis or subclinical thyroiditis, 4 had diabetes mellitus and one had both thyroid and pancreas involvement. Thyroid stimulating hormone in 10 patient with thyroid involvment was 19.6 (5.2-34.0) mU/L, and their total thyroxine was 75.3 (45.2-105.4) nmol/L. Besides, thyroid peroxidase antibody or thyroglobulin antibody was positive in 7 cases. The blood glucose of 4 children with pancreatic injury was 25.0 (17.0-33.0) mmol/L, and C-peptide was 0.4 (0.3-0.5) mg/L. Glutamate dehydrogenase antibody, protein tyrosine phosphatase antibody and zinc transporter 8 antibody were positive in two cases. After treatement with immunosuppressant or immunoglobulin combined with glucocorticoid or nonsteroidal antiinflammatory drugs for rheumatic symptoms, and levothyroxine or insulin for endocrine diseases, they were all followed up for more than 6 months and maintained clinical stability. Conclusions: Rheumatic diseases in children can be complicated with endocrine disorders, and the involved organs are usually thyroid and pancreas. In children with rheumatic disease, thyroid injury usually has subtle onset, whereas pancreas injury develops rapidly, even life-threatening. Insulin should be used persistently under the instruction of endocrinologist.

[Infliximab in infantile Takayasu arteritis: a case report and literature review].

Objective: To analyze the clinical characteristics of infantile Takayasu arteritis and the efficacy of infliximab (IFX). Methods: Clinical manifestations, laboratory investigations and infliximab intervention of a case with infantile Takayasu arteritis, who was admitted to Department of Rheumatism and Immunology, Children's Hospital, Capital Institute of Pediatrics in January 2018, were reviewed and analyzed. The related literature published from the beginning to March 2020 were retrieved from CNKI, Wanfang, SinoMed and PubMed with the keywords of"Takayasu arteritis","Infant" in both Chinese and English. Results: This case was a 70-day-old boy admitted due to recurrent fever for 20 days. On admission, his blood pressure were 104/90, 95/59, 125/80, and 152/125 mmHg (1 mmHg=0.133 kPa) in the right arm, left arm, right leg, and left leg, respectively. The complete blood cell count showed leukocytosis (22.6×109/L), thrombocytosis (858×109/L) and mild anemia (80 g/L). He also had elevated erythrocyte sedimentation rate (119 mm/1h), serum ferritin (598 µg/L) and C-reactive protein (112 mg/L). Computed tomographic angiography (CTA) showed narrowing of the thoracic and abdominal aorta, with thickening and heterogenous enhancement of the vessel wall. Coronary artery ultrasound detected dilatation and wall thickening of the bilateral coronary arteries, and uneven dilatation of the middle segment of the right coronary artery, showing bead-like change. Vessel wall thickening was also found in the other main arteries, including both femoral arteries, axillary arteries, carotid arteries, and subclavian arteries, and both superficial femoral arteries were slightly narrowed in the distal segments. The diagnosis of TA was confirmed, and the boy was treated with infliximab monotherapy (5 mg/(kg·every time), a total of 13 times). Then his body temperature and all inflammatory markers were normalized, and the vascular pathology was resolved according to the radiography. No side effects such as allergy or infection were noted during the treatment. During the 2 years and 6 months of follow-up, the boy maintained normal growth and development. Literature review found 8 related articles, and one of them was in Chinese but had limited information. In the other 7 papers, a total of 7 infants with TA were reported. The most common symptom was fever (5 cases), and inflammatory markers usually elevated, and the most common affected artery was abdominal aorta (6 cases). Most cases were treated with glucocorticoid. Conclusions: TA is a rare disease in infants, usually presents with fever and increased inflammatory markers. At the early stage, infliximab monotherapy could effectively control the symptoms and ensure normal growth and development.

[Tocilizumab for refractory systemic juvenile idiopathic arthritis].

Objective: To evaluate the efficacy and side effects of tocilizumab for the treatment of systemic juvenile idiopathic arthritis. Method: In this prospective self case-control study, the children diagnosed with refractory systemic juvenile idiopathic arthritis admitted to Department of Rheumatism and Immunology of Children's Hospital Affiliated to Capital Institute of Pediatrics from December 2013 to June 2016 were enrolled and information before and after treatment of tocilizumab was analyzed. The tocilizumab was introvenously guttae in a dose of 8-12 mg/kg every 2 weeks. Complete blood count, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) were tested before and after the application of tocilizumab. Detailed clinical manifestations were recorded. All results were analyzed by χ(2) test and t test. Result: Forty patients with a median age of (6.6±3.7) years were enrolled, including 15 males and 25 females. All of the patients presented with fever and 38 patients got normal temperature 24-48 hours after treatment with tocilizumab. Symptoms disappeared in 13 and improved in 4 patients after treatment among the 17 patients who presented with arthritis. Within the 10 patients who manifested with rashes, 9 patients' rashes disappeared without relapse accompanied by the normalization of temperature after the treatment of tocilizumab. One patient got normal temperature but intermittently emerged rashes after symptoms of arthritis improved. In the 40 patients, 38 well tolerated tocilizumab while 2 showed rashes and chill which disappeared shortly after antianaphylaxis treatment. No severe treatment-related infection was found in any patients. According to the study, the white blood cell counts(×10(9)/L), CRP(mg/L) and ESR(mm/1h) tested 2 weeks after the treatment with tocilizumab were significantly lower than that before treatment(12.1±1.2 vs. 16.5±1.8, 47±8 vs. 67±9, 21±5 vs. 57±6, t=2.75, 3.98, 5.22, P=0.009, 0, 0, respectively). No significant changes were found in concentration of IL-6 and TNF-α (65(207) vs. 45(137) ng/L, and 14(6) vs. 17(19)ng/L, Z=-1.247 and-1.285, P=0.212 and 0.199 respectively). Conclusion: Tocilizumab is a treatment with good efficacy and safety for refractory systemic juvenile idiopathic arthritis. Adverse effects would be found in some patients.

[Characteristics of the joint involvement in fibrodysplasia ossificans progressiva and follow up].

Objective: To summarize the clinical data of 15 patients with fibrodysplasia ossificans progressiva (FOP), follow up and analyze the characteristics of the joint involvement in FOP. Method: From May 2005 to December 2016, fifteen FOP cases had been diagnosed in the Children's Hospital Capital Institute of Pediatrics. All medical records and follow-up data were collected and a retrospective analysis was made on the joint involvement in FOP. Pearson correlation analysis was used for data, P<0.05 for the difference was statistically significant. Result: There were 8 males and 7 females in 15 cases. The age of onset was 2(1-6)years. The age at diagnosis was 6 (4-9) years. All cases had hallux valgus deformity and bone mass formation. Twelve cases had joints involvement on enrollment into this study: 8 cervical vertebra, 7 shoulder joint, 5 hip joint, 4 elbow joint, 3 wrist joint, 2 temporomandibular joint, 2 lumbar vertebra. The age of diagnosis and duration of disease were positively correlated with the number of the involved joints (r=0.523, 0.628; P=0.045, 0.012); mild changes were found in joint imaging. Thirteen cases received telephone follow-up, the average duration of follow-up was 6(3-7)years, no change in 11 cases, disease progress in 2 cases. Conclusion: Joint involvement is a common complication of FOP, especially the cervical vertebra.Multiple joints involvement, dominant functional impairment, and mild imaging changes are the characteristics of joint lesions caused by FOP.The number of involved joints gradually increases with increase of age of the patients and the prolonged course of the disease.

[Prediction of occupational allergic contact dermatitis induced by formaldehyde by IL17/IL22 secretion cell rest combined with patch test].

Objective: To investigate the possible role of IL17-and IL22-secreting cells combined with patch test for the prediction of formaldehyde-induced occupational allergic contact dermatitis(OACD). Methods: From October 2014 to October 2016, totally 131 formaldehyde-exposed workers(49 cases with inflammatory skin lesions,82 ones without inflammatory skin lesions)and 63 non-exposed health controls were recruited. Patch-test was performed in 49 cases of formaldehyde-exposed workers with inflammatory skin lesions. Circulating IL17+and IL22+Tcell subsets were assessed by flow cytometry(FCM). Results: Among 49 cases of formaldehyde-exposed workers with inflammatory skin lesions,32 cases were with positive patch-test while 17 cases with negative patch-test. The proportions of circulating CD3+CD8-IL17+ and CD3+CD8-IL22+ cells from patch-test(+) formaldehyde-exposed workers were significantly higher than that of patch-test(-)group, formaldehyde-exposed workers without skin lesions and non-exposed controls(P<0.05). The proportions of circulating CD3+CD8-IL17+ and CD3+CD8-IL22+cells from patch-test(-)group and formaldehyde-exposed workers without skin lesions were also higher than that of non-exposed controls(P<0.05). But there was no significant difference between patch-test(-)group and formaldehyde-exposed workers without skin lesions(P>0.05). Peripheral CD3+CD8+IL17+and CD3+CD8+IL22+cells were also detected in spite of small amounts. The percentages of CD3+CD8+IL17+and CD3+CD8+IL22+ cells inperipheral blood from patch-test(+)formaldehyde-exposed workers were enhanced significantly, compared to patch-test(-)group, formaldehyde-exposed workers without skin lesions and non-exposed controls(P<0.05). The proportions of circulating CD3+CD8+IL17+ and CD3+CD8+IL22+ cells from patch-test(-)group and formaldehyde-exposed workers without skin lesions were significantly higher than that of non-exposed controls(P<0.05). But there was no significant difference between patch-test(-) group and formaldehyde-exposed workers without skin lesions(P>0.05). Conclusion: The proportions of circulating IL17+ and IL22+T cells(both CD8-and CD8+)are enhanced in formaldehyde-exposed workers at proposed OEL, possibly involved in the development of formaldehyde-induced OACD.The detection of IL17-and IL22-secreting cells combined with formaldehyde patch test help to screen the workers with allergy property and prevent OACD.

[Role of magnetic resonance imaging in the diagnosis of juvenile dermato-myositis and polymyositis in Chinese children].

Objective: To evaluate the utility of magnetic resonance imaging (MRI) in diagnosis of juvenile dermatomyositis and polymyositis (JDM-PM) in children. Method: Fifty-four patients with JDM-PM in the active stage were enrolled in the study group. Twelve patients with benign acute childhood myositis and forty patients with juvenile idiopathic arthritis (JIA) complicated with myositis were enrolled as controls. MRI imaging of thighs was performed in all patients, fast spin echo T1WI, T2WI, and STIR were obtained in all patients.Muscle biopsy was performed in 41/54 patients with JDM-PM. We compared the value of MRI in diagnosis of JDM-PM with muscle biopsy, electromyography and serum aspartate transaminase (AST), alanine transaminase (ALT), creatine kinase (CK), isoenzyme of creatine kinase (CKMB), lactate dehydrogenase (LDH), hydroxybutyrate dehydrogenase (HBDH) levels. Continuous normally distributed variables were reported as means and continuous non-normally distributed variables as median. Chi-square test and Fisher exact test were used to test differences between MRI and other categorical variables. Result: A total of 54 patients were included. Twenty-seven patients were male and the others were female. Average age of the patients was (7.1±3.5) years (2-13 years); 45(83%) paitests were JDM cases and 9(17%) patients had JPM. All patients had MRI examination. Of the 54 patients, 53 had multiple myositis; 10 out of 50 (19%) patients received second MRI after treatment, 6 out of 10 patients had normal findings, 4 patients showed obviously improved images; 41 out of 54 patients underwent muscle biopsy; 22 out of 41 patients had inflammatory cells infiltration and muscle fiber degeneration. The results of the muscle enzyme tests are as follows: 27 (50%) patients had elevated AST, 24 (44%) patients had elevated ALT, 22 (41%) patients had elevated CK, 18(33%) patients had elevated CKMB, and LDH rose in 30 (56%) patients, HBDH rose in 28(52%) patients. These results suggested that muscle MRI was more sensitive than muscle biopsy and muscle enzyme tests in diagnosis of JDM-PM. Conclusion: Patients with JDM-PM showed diffuse patchy hyperintense signals on T2WI of their thighs. MRI may be a sensitive, reliable, and noninvasive tool for clinical diagnosis and theraputic evaluation of JDM-PM.

[The value of brain magnetic resonance imaging in the early diagnosis of neuropsychiatric systemic lupus erythematosus].

OBJECTIVE: To investigate the value of brain magnetic resonance imaging (MRI) in evaluating the intracranial injuries in patients with juvenile onset systemic lupus erythematosus (SLE). METHOD: Data of brain MRI, CT, electroencephalogram (EEG), cerebrospinal fluids analysis and clinical features of the central nervous system of 44 patients from March 2007 to March 2015 with juvenile onset SLE who were not treated with glucocorticoids (Gcs) and immunosuppressive agents (Is) were retrospectively analyzed and compared. RESULT: Twenty-seven out of 44 patients demonstrated abnormal signs on brain MRI, including encephalatrophy, cerebral infarction, demyelination, encephalorrhagia, vertebral arteriostenosis and abnormal signals on the brain diffusion-weighted imaging (DWI). Sixteen patients had clinical features of the central nervous system involvement, fifteen had continuous headache, nine had continuous dizziness, seven had convulsions, three had hemiplegia, one had blurred vision. Physical examination of the nervous system: ten patients had abnormal signs, all had cervical rigidity, five showed pyramidal sign, three showed loss of muscle tone, two with cranial neuropathies and one had paresthesia. EEG: Ten patients showed abnormal waves on EEG, all showed diffused slow-waves, and five showed sharp waves and spikes. Cerebrospinal fluids analysis: six patients had abnormal results, five of them had cell count elevation and one had cell count and protein elevation, while there was glucose and chloride degression. Brain CT: Eight patients received CT scan, two showed cerebral infarction. χ(2) test was used to compare the differences among head MRI, EEG, cerebrospinal fluid analysis, physical examination of the nervous system, clinical features of the nervous system, the difference was significant(χ(2)=12.055, P=0.001; χ(2)=19.627, P=0.001; χ(2)=3.859, P=0.049; χ(2)=12.055, P=0.001). CONCLUSION: Brain MRI may be a better method in early diagnosis of intracranial injuries than CT, EEG, cerebrospinal fluid analysis and physical examination of the nervous system. Patients with juvenile onset SLE should receive brain MRI after diagnosis in order to investigate the intracranial injuries. Abnormal signals on the DWI are the signs of active disease.