Childhood rheumatic diseases: bites not only the joint, but also the heart.

Cardiovascular involvement in juvenile rheumatic diseases is the primary manifestation in paediatric vasculitis and a major organ manifestation in paediatric connective tissue diseases. Though coronary vasculitis is the prototypical manifestation of Kawasaki disease, it can also be seen in patients with polyarteritis nodosa. Pericarditis is the most common manifestation seen in juvenile rheumatic diseases like systemic onset JIA, and lupus. Cardiac tamponade, valvular insufficiency, aortic root dilatation and arrhythmias are seen rarely. Cardiac involvement is often recognized late in children. The development of cardiac disease in juvenile systemic sclerosis is associated with a poor outcome. In long term, childhood onset of rheumatic diseases predisposes to diastolic dysfunction and premature atherosclerosis during adulthood. Key Points • Pericarditis is the most common cardiac manifestation in SLE and can lead to tamponade. • Conduction defects are common in juvenile mixed connective tissue disease and systemic sclerosis. • Pulmonary hypertension is a significant contributor to mortality in juvenile systemic sclerosis. • In Kawasaki disease, early treatment can reduce risk of coronary artery aneurysms.

MRI and Sonography of the Knee in Acute Reactive Arthritis: An Observational Cohort Study.

INTRODUCTION: Reactive arthritis (ReA) is a unique subgroup of spondyloarthritis with acute presentation and tendency to develop chronicity. Magnetic resonance imaging (MRI) has enabled identification of sensitive markers of response to therapy. METHODS: A longitudinal pilot study of acute ReA with knee joint involvement satisfying the Braun's criteria was undertaken. Magnetic resonance imaging of the knee was assessed at baseline, and agreement with ultrasonography was assessed. Clinical details were recorded using a detailed and structured case record form. Patients were followed up, and MRI predictors of transition to chronic arthritis were looked for. RESULTS: In 25 patients with ReA, synovial thickening was the most common feature. Enthesitis was observed on MRI in 20%. Urethritis-related and HLA-B27-positive ReA had higher synovial thickening scores (p = 0.007). Agreement was poor between MRI and ultrasonography (synovial hypertrophy: k = 0.04). On follow-up, 34% (n = 7/21 for >12 months) continued to have active disease. None of the clinical or radiological features were predictive of chronicity. CONCLUSIONS: Posturethritis and B27-positive ReA was more severe than postenteritis ReA and RA on MRI. One third develop chronic disease on follow-up. Magnetic resonance imaging is superior to sonography, although baseline imaging is not predictive of chronicity. The results of this pilot exploratory study argue for larger studies on MRI in ReA.

Mechanisms of thrombosis in ANCA-associated vasculitis.

Patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have a two- to threefold greater risk of developing venous as well as arterial thrombotic events. Although such thrombotic events are more commonly seen during phases of active AAV, they are also recognized to occur during AAV in remission. Endothelial injury is a key pathogenic event in AAV. Endothelial injury can be caused by neutrophil activation and release of thrombogenic tissue factor into the circulation. Neutrophil activation further results in the formation of neutrophil extracellular traps (NETs). NETs contribute to thrombosis by expressing tissue factor. NETs have also been detected in cutaneous thrombi from patients with AAV induced by hydralazine. Activated neutrophils in AAV patients release thrombogenic microparticles loaded with tissue factor which further enhances clotting of blood. Antiphospholipid antibodies (APLs) have been detected in up to a third of AAV and might also be induced by drugs such as cocaine adulterated with levamisole and propylthiouracil, which are known to trigger AAV. Such APLs further drive the thrombosis in AAV. Once thrombogenesis occurs, the homeostatic mechanisms resulting in clot dissolution are further impaired in AAV due to anti-plasminogen antibodies. The ongoing pandemic of coronavirus disease 2019 (COVID-19) is associated with endothelial injury and NETosis, mechanisms which are in common with AAV. Reports of new-onset AAV following COVID-19 have been described in the literature, and there could be shared mechanisms driving these processes that require further evaluation.

18F-florbetaben whole-body PET/MRI for evaluation of systemic amyloid deposition.

BACKGROUND: Florbetaben, a 18F-labeled stilbene derivative (Neuraceq®, formerly known as BAY-949172), is a diagnostic radiopharmaceutical developed to visualize ß-amyloid plaques in the brain. Here, we report a pilot study evaluating patients with suspected cardiac amyloidosis for systemic extent of disease. METHODS: We prospectively enrolled nine patients, 61-86 year old (mean ± SD 69.4 ± 8.6), referred from the cardiac amyloid clinic. First, dynamic imaging of the heart was acquired immediately after injection of 222-318.2 MBq (mean ± SD 270.1 ± 33.3) of 18F-florbetaben using the GE SIGNA PET/MRI. This was followed by a whole-body PET/MRI scan 60-146.4 min (mean ± SD 98 ± 33.4) after injection. Cardiac MRI sequences included ECG-triggered cine SSFP, T2-weighted, and late gadolinium-enhanced imaging. Whole-body MRI sequences included MRAC and axial T1-weighted imaging. RESULTS: High early uptake and delayed high uptake in the left ventricle correlated with amyloid deposition in five patients, while low uptake on early and delayed cardiac imaging was noted in four patients. Cardiac function measurements were successfully obtained in all participants. Areas of increased abnormal 18F-florbetaben accumulation were noted on delayed whole-body imaging in the bone marrow (seven patients), stomach (diffuse in five patients and focal in one patient), brain (five patients), salivary glands (three patients), tongue (three patients), spleen (three patients), skeletal muscles (three patients), ocular muscles (two patients), thyroid (two patients), pleura (two patients), kidneys (two patients), and lungs (two patients). CONCLUSIONS: Whole-body 18F-florbetaben PET/MRI is promising for localization of systemic amyloid deposition. This technique may provide important structural and functional information regarding the organs involved by disease, with potential to guide biopsy and evaluate response to treatment. TRIAL REGISTRATION: Clinicaltrials.gov registration: NCT03119558 .