Juvenile Idiopathic Arthritis

This project intends to explore and validate the utility of new MRI pulse sequence, 3D DL oZTEo, in the detection of osseous erosions of the hand in patients with inflammatory arthritis.

The purpose of this study is to evaluate long-term safety of subcutaneous guselkumab in pediatric participants with moderately to severely active ulcerative colitis, or moderately to severely active Crohn's disease, or juvenile psoriatic arthritis (jPsA).

The Takayasu arteritis (TAK) is a rare chronic inflammatory arteritis, which lacks an effective well-accepted intervention strategy. Previous studies have revealed that methotrexate, tofacitinib, adalimumab, and tocilizumab were effective in controlling disease activity and preventing disease relapse in some TAK patients, especially the latter two biological agents. However, we believed that different patients should be prescribed different drug combinations, i.e. personalized medicine, to obtain the optimal intervention effect. Here we tried to classify the TAK patients into three levels including mild, moderate, and severe, and prescribe different drug interventions to discover which biological agent is better in severe TAK patients. 1\. Patients were classified as mild, moderate, and severe groups according to the disease severity of TAK patients. 1.1 Severe 1. Severe hypertension 1. Continuously upper limb systolic blood pressure ≥180mmHg or diastolic blood pressure ≥110mmHg; 2. OR upper limb blood pressure cannot be measured if lower limb systolic blood pressure ≥200mmHg or diastolic blood pressure ≥120mmHg; 3. With target organ damage due to hypertension; 2. Aortic arch and its branches involved a. Multiple branches involved (two or more) and severe stenosis (stenosis rate ≥70%); b. stenosis rate ≥50%, accompanied by nervous system ischemic symptoms and/or signs; c. stenosis rate ≥50%, accompanied by a recent history of cerebrovascular events; 3. The carotid artery and its branches involved 1. Multiple branches (two or more) involved and severe stenosis (stenosis rate ≥70%); 2. stenosis rate ≥50%, accompanied by nervous system ischemic symptoms and/or signs; 3. stenosis rate ≥50%, accompanied by the recent history of cerebrovascular events; 4. Pulmonary artery involvement a. Chest tightness, hemoptysis, dyspnea, radionuclide pulmonary ventilation/blood perfusion imaging or CTA suggesting pulmonary artery thrombosis with respiratory failure (type I); b. Chest tightness, shortness of breath, and cardiac ultrasound suggesting severe pulmonary artery hypertension with cardiac function abnormality (NYHA class III and above); 5. The coronary artery involved 1. The onset of unstable angina pectoris or myocardial infarction; 2. Cardiac ultrasound indicating ischemic cardiomyopathy, NYHA class III and above; 6. The aortic valve and aortic root involved 1. Severe reflux of the aortic valve; 2. OR aortic valve leakage, annulus tearing; 3. OR aneurysm in the aortic root and/or ascending aortic (≥2 times in diameter); 4. OR dilation of the aortic root and/or ascending aorta (≥5cm in diameter); 5. OR dissection of the aortic root and/or ascending aorta; Any item of the above a - e is accompanied by abnormal cardiac function (NYHA III and above); 7. Renal artery involved a. Severe stenosis of renal artery secondary to malignant hypertension (blood pressure is still 180 / 120mmHg after treatment with three or more antihypertensive drugs) b. Severe renal artery stenosis accompanied by a progressive increase in serum creatinine or a reduction in glomerular filtration rate (GFR) of ≥25%; 8. Glucocorticoids and the traditional synthetic chemical immunosuppressants were of no use. And the disease is not well controlled with the severe injury of the organs. 1.2 Moderate 1. Hypertension a. Upper limb systolic blood pressure ≥160-180mmHg or / and diastolic blood pressure ≥100-110mmHg; b. OR upper limb blood pressure is unmeasurable or lower limb systolic blood pressure is ≥180mmHg or diastolic blood pressure is ≥100-110mmHg; 2. Aortic arch and its branches involved a. 1-2 vessels are involved with moderate stenosis (stenosis rate ≥50%-\<70%); b. Dizziness occurs during physical activity, and symptoms disappear in the resting state; 3. The carotid artery and its branches involved a. Unilateral or bilateral vascular stenosis rate ≥50% -70%, with dizziness during light physical activity; 4. Pulmonary artery involved a. Radionuclide pulmonary ventilation/blood perfusion imaging or CTA indicates pulmonary vascular disease; chest tightness after activity; Cardiac ultrasound indicates moderate pulmonary artery hypertension (pressure\> 40-60mmHg), with abnormal cardiac function (NYHA class Ⅱ); 5. Coronary artery involvement a. Chest tightness and chest pain after moderate activities, CTA showed 50% or more in coronary artery stenosis, abnormal cardiac function (NYHA class Ⅱ); 6. The aortic valve and aortic root involved 1. Moderate aortic regurgitation; 2. Aortic root and/or ascending aortic aneurysm (diameter \<2 times); 3. Dilation of the aortic root and/or ascending aorta (diameter \<5cm); Each item of a - c is accompanied by abnormal cardiac function (NYHA Class II); 7. Renal artery involvement a. Renal artery stenosis rate ≥50%, and blood pressure 160-180 / 110-120 (excluding 120) mmHg after treatment, or with left ventricular myocardial hypertrophy, hypertension and heart disease, CKD-II; 1.3 Mild 1. Hypertension 1. Upper limb systolic blood pressure ≥140mmHg or diastolic blood pressure ≥90mmHg; 2. OR upper limb blood pressure is unmeasurable or lower limb systolic blood pressure is ≥140mmHg or diastolic blood pressure is ≥90mmHg; 2. Aortic arch and its branches involved a. Single or multiple lesions with mild stenosis (stenosis rate \<50%), and without neurological ischemic symptoms and/or signs in daily activities; 3. The carotid artery and its branches involved a. Single or multiple lesions with mild stenosis (stenosis rate \<50%) and no neurological ischemic symptoms and/or signs during daily activities; 4. The pulmonary artery involved 1. Cardiac ultrasound indicates mild or normal pulmonary artery pressure (pressure 30-40mmHg); 2. Imaging shows pulmonary arteritis or pulmonary artery stenosis, occlusion a and b, with chest tightness, shortness of breath, and hemoptysis without activity; cardiac function (NYHA I), normal blood gas analysis; 5. The coronary artery involved a. Chest tightness, shortness of breath, chest pain after inactivity; cardiac function (NYHA I); 6. The aortic valve and aortic root involved 1. Mild aortic regurgitation; 2. Aortic root and / or ascending aortic aneurysm-like expansion (diameter \<1.5 times); a and b, each with cardiac function (NYHA I); 7. Renal artery involved a. Renal artery stenosis rate \<50%, without/with mild hypertension (see 1), or normal serum creatinine, normal or slightly impaired glomerular filtration rate (GFR); 1.2. Based on the TAK patients in the ECTA cohort (Clinical trials. No: NCT03893136), we tried to compare the treatment efficacy of biological agents between tocilizumab (TCZ) and adalimumab (ADA) in severe TAK patients, with a pilot study. Other important detailed description of the study are listed as follows: 1. Basic treatment of prednisone: The initial prednisone dose is 40mg.qd.po, and maintained for 1 month. After 1 month's treatment, the dose is gradually tapered to 15mg by 5mg per 2 weeks. Subsequently, the dose is decreased to 5mg by 2.5mg per 3 months. The 5mg is the final target maintained dose. In the treatment, if the relapse occurs, the dose of prednisone returned to the last dosage. For example, if the patient gets a relapse at the dose of 15mg of prednisone, then the dose of prednisone returned to 20mg. The relapse of TAK is defined according to the "2018 Update of the EULAR recommendations for the management of large vessel vasculitis". Relapse includes major relapse or minor relapse. Major relapse: Recurrence of active disease with either of the following: a. Clinical features of ischemia\* (including jaw claudication, visual symptoms, the visual loss attributable to TAK, scalp necrosis, stroke, limb claudication). b. Evidence of active aortic inflammation resulting in progressive aortic or large vessel dilatation, stenosis or dissection. Minor relapse Recurrence of active disease, not fulfilling the criteria for the major relapse. 2. ADA: 40mg bim IH. 3. TCZ: 8mg/kg qm ivgtt. 4. Treatment shift: if the TAK patients in ADA group failed to reach clinical remission at the end of 24th week, they would be shifted to TCZ group starting a new round of induction and complete the rest 24-week follow-up; and vice versa; In the follow-up, the disease remission and related markers are monitored.

1. To understand the severity and nature of participants experiences during rheumatic immune-related adverse events following immune checkpoint inhibitor immunotherapy, including their functional impact 2. To explore domains relevant to participants experiencing rheumatic immune-related adverse events following immune checkpoint inhibitor immunotherapy, and attitudes to domains identified from the literature

The total study duration per patient will be 166 weeks that will consist of a 4- week screening, a 12-week core treatment phase, a 144-week extension phase, and a 6-week post-treatment follow-up.

Approval of the study was obtained from the hospital's ethical committee. The study design and methodology followed the tenets of Declaration of Helsinki. All patients were provided with written informed consent and received a thorough explanation of the use of adalimumab, its potential risks and benefits. This is a monocenter, cohort, observational study evaluating patients with acute VKH divided into two groups: adalimumab therapy group and traditional therapy group. For adalimumab therapy group, an initial dose of 80 mg adalimumab was administered subcutaneously followed by a maintenance dose of 40 mg every two weeks, and data will be collected prospectively with regard to ophthalmologic outcomes. For the traditional therapy group, patients were treated with glucocorticoids alone or glucocorticoids combined with immunosuppressants. Study participants will be followed for up to one year to determine efficacy and side effects. According to best corrected visual acuity (BCVA), anterior chamber and vitreous inflammation, optical coherence tomography (OCT), change in corticosteroid dose during the study period and so on. The investigators evaluate the anti-inflammatory and immunosuppressive effects of adalimumab in treatment of acute VKH.

The primary objective of this study is to evaluate the long-term safety of apremilast in subjects 2 years of age or older with oral ulcers associated with Behçets disease or 5 years of age or older with active juvenile psoriatic arthritis that have completed Study 20190530 or Study 20190529.

The reason for this study is to see if the study drug baricitinib is safe and effective in participants from 1 year to less than 18 years old with systemic juvenile idiopathic arthritis (sJIA). Participants are assigned to 1 of 2 cohorts. In cohort 1, participants will receive baricitinib or tocilizumab reference. In cohort 2, participants will receive baricitinib.

The reason for this study is to see if the study drug baricitinib is safe and effective in the treatment of JIA in participants ages 1 to 17. This study is for participants that have been enrolled in studies I4V-MC-JAHV (NCT03773978) or I4V-MC-JAHU.

Psoriatic arthritis (PsA) is a type of arthritis that happens when the body's immune system attacks healthy cells and tissues causing joint pain, stiffness, and swelling. Symptoms can get worse and go away for periods of time. PsA that begins before a patient's 16th birthday is called juvenile PsA (jPsA).This study will evaluate how safe risankizumab is for the treatment of psoriatic arthritis and to assess change in disease symptoms. Risankizumab is being studied for the treatment of jPsA and adalimumab is approved for the treatment of jPsA. Participants are placed in 1 of 2 groups, called treatment arms. Each group receives a different treatment. There is a 1 in 4 chance that participants will be assigned to receive adalimumab. Approximately 40 juvenile participants with jPsA will be enrolled at approximately 30 sites worldwide. Participants will receive risankizumab and adalimumab as subcutaneous (SC) injections based on body weight. At the start of Period 1, participants are randomized to receive risankizumab or adalimumab for 24 weeks. Participants who respond to the study treatment received in Period 1, will continue to receive the same treatment in Period 2 for another 100 weeks. Those with worsening jPsA symptoms in Period 2 will be withdrawn from the study. Participants who receive adalimumab are followed for safety for 70 days after the last study treatment. Participants who receive risankizumab are followed for 140 days after the last study treatment. There may be higher treatment burden for participants in this trial compared to their standard of care (due to study procedures). Participants will attend regular visits during the study at a hospital or clinic. The effect of the treatment will be checked by medical assessments, blood tests, checking for side effects and completing questionnaires.