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Concept: Rheumatism

29

More than 14 years of clinical practice in rheumatology led the author to discover the prognostic role of anti-citrullinated protein antibody (ACPA) as well as the erosions found by MRI, in detecting the RA patients resulting in establishing a new set of criteria by revising the 1987 ACR classification-Iran Criteria for Rheumatoid Arthritis. Medical records of 243 patients at the outpatient Rheumatology Clinic of the author (private sector) were reviewed for the data on the criteria of the 1987 ACR, 2010 ACR/European League against Rheumatism (EULAR), and Iran Criteria for RA. In addition to modifying the 1987 ACR classification, Iran Criteria for RA adds some additional information to the ACR criteria (including ACPA and bony erosions detected by MRI), and any patient who satisfies 6 out of 12 points is considered as a definite RA patient. Sensitivity of the three classifications was calculated considering the clinical diagnosis by a single rheumatologist as the gold standard. A total of 63 male and 180 female patients with a mean follow-up duration of 28.24 ± 50.19 months were considered. Mean age at diagnosis and mean disease duration were 49.16 ± 15.38 years and 7.04 ± 6.87 months, respectively. The sensitivity for Iran Criteria for RA, 1987 ACR classification, and 2010 ACR/EULAR criteria were calculated as 98.4, 59.7, and 66.3%, respectively. Comparing Iran Criteria for RA with ACR and ACR/EULAR criteria, it was concluded that our newly introduced criteria is a more sensitive instrument in determining RA patients in the early stages of the disease.

Concepts: Medical terms, Rheumatoid arthritis, Rheumatology, Vasculitis, Gout, Arthritis, Rheumatism, European League Against Rheumatism

28

Rheumatic heart disease (RHD) is a chronic condition characterized by fibrosis and scarring of the cardiac valves and damage to the heart muscle, leading to congestive heart failure and death. This prospective cohort study was conducted to investigate the possible relation between the levels of serum adhesion molecules and acute rheumatic fever (ARF) carditis, valvular insult severity, and residual valvular lesion after improvement of rheumatic activity. Serum levels of intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), and E-selectin were assayed by enzyme-linked immunoassay (ELISA) for 50 children with ARF carditis during activity and after improvement and for 50 healthy children as control subjects. After the acute attack, patients were followed up regularly to detect residual valvular lesion. The serum levels of these adhesion molecules were significantly higher in the patients than in the control group (p < 0.001). In addition, the levels of serum adhesion molecules were significantly higher in the patients with severe carditis than in the patients with mild to moderate carditis (p < 0.001). Among the severe carditis group, the level of serum adhesion molecules was significantly higher among the patients with heart failure than among the patients without heart failure (p < 0.001). Furthermore, the pretreatment serum levels of ICAM-1 and VCAM-1 were significantly higher among the patients with residual valve lesion (p = 0.002) than among those without the lesion (p < 0.001). The cutoff values were obtained for the prediction of residual valvular lesion (ICAM-1, >1,032.3 μg/ml; VCAM-1, >3,662.3 μg/ml; E-selectin, >104.8 μg/ml). Finally, by combining the three adhesion molecules in a single prediction model, the highest area under the curve (AUC) ± standard error (SE) was obtained (0.869 ± 0.052), and the positive likelihood ratio for having a residual valvular lesion was increased (17.33). Levels of serum adhesion molecules could predict residual valvular lesions in RHD patients. The authors recommend that the serum level of adhesion molecules be measured in all cases of ARF carditis.

Concepts: Cardiology, Heart, Rheumatic fever, Cell adhesion molecule, Rheumatism, Intercellular adhesion molecule, VCAM-1, Heart valve

28

To compare the diagnostic accuracy of the 2010 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) and 1987 ACR criteria for rheumatoid arthritis (RA) in a cohort of patients with recent-onset arthritis followed-up for 10 years.

Concepts: Medical terms, Rheumatoid arthritis, Rheumatology, Vasculitis, Arthritis, Rheumatism, European League Against Rheumatism

27

Anti-TNF-α are used in the treatment of rheumatic diseases not responsive to first-line regimens. Data on the safety of anti-TNF-α in HIV-infected patients are scarce and conflicting. We describe a case of septic shock and multiorgan failure occurred after etanercept initiation and influenza vaccination in a HIV-infected-woman with rheumatoid arthritis.

Concepts: Rheumatoid arthritis, Rheumatology, Influenza, Rheumatism, Septic shock

21

We retrospectively analyzed electronic medical records of patients with Ehlers-Danlos Syndrome hypermobility type (HEDS), including demographic information, workup, rheumatological diagnoses in order to determine its association with rheumatological conditions. HEDS Patients were stratified according to level of workup received (no additional work (physical exam only) = NWU, limited workup = LWU, comprehensive workup = CWU)). HEDS patients were predominantly female (21:4, F:M). The percentage of patients with at least one rheumatological condition was significantly correlated with level of workup (NWU, 9.2%; LWU, 33.3%, CWU, 67.1%; p-value < 0.0001). The HLA-B27 antigen was more prevalent (p-value < 2.2 × 10(-8)) in the CWU HEDS patients (23.9%) than in the general population of the United States (6.1%). HEDS with CWU were associated with more rheumatological conditions (i.e. psoriasis, ankylosing spondylitis, rheumatoid arthritis, fibromyalgia) than those with NWU or LWU. In conclusion, HEDS is associated with complicated rheumatological conditions, which are uncovered by comprehensive workup. These conditions require different clinical management strategies than HEDS, and left untreated could contribute to the pain or even physical disability (i.e. joint erosions) in HEDS patients. While the mechanisms underlying these associations are unknown, it is important that all HEDS patients receive adequate workup to ensure a complete clinical understanding for the best care strategy possible.

Concepts: Rheumatic fever, Rheumatoid arthritis, Rheumatology, Syndromes, Arthritis, Rheumatism, Ankylosing spondylitis, Psoriatic arthritis

18

A European League Against Rheumatism (EULAR) task force was established to define points to consider on use of antirheumatic drugs before pregnancy, and during pregnancy and lactation. Based on a systematic literature review and pregnancy exposure data from several registries, statements on the compatibility of antirheumatic drugs during pregnancy and lactation were developed. The level of agreement among experts in regard to statements and propositions of use in clinical practice was established by Delphi voting. The task force defined 4 overarching principles and 11 points to consider for use of antirheumatic drugs during pregnancy and lactation. Compatibility with pregnancy and lactation was found for antimalarials, sulfasalazine, azathioprine, ciclosporin, tacrolimus, colchicine, intravenous immunoglobulin and glucocorticoids. Methotrexate, mycophenolate mofetil and cyclophosphamide require discontinuation before conception due to proven teratogenicity. Insufficient documentation in regard to fetal safety implies the discontinuation of leflunomide, tofacitinib as well as abatacept, rituximab, belimumab, tocilizumab, ustekinumab and anakinra before a planned pregnancy. Among biologics tumour necrosis factor inhibitors are best studied and appear reasonably safe with first and second trimester use. Restrictions in use apply for the few proven teratogenic drugs and the large proportion of medications for which insufficient safety data for the fetus/child are available. Effective drug treatment of active inflammatory rheumatic disease is possible with reasonable safety for the fetus/child during pregnancy and lactation. The dissemination of the data to health professionals and patients as well as their implementation into clinical practice may help to improve the management of pregnant and lactating patients with rheumatic disease.

Concepts: Pregnancy, Rheumatoid arthritis, Rheumatology, Rheumatism, Immunosuppressants, Mycophenolic acid, Disease-modifying antirheumatic drug, Teratology

14

Rheumatic heart disease (RHD) prevalence and mortality rates remain especially high in many parts of Africa. While effective prevention and treatment exist, coverage rates of the various interventions are low. Little is known about the comparative cost-effectiveness of different RHD interventions in limited resource settings. We developed an economic evaluation tool to assist ministries of health in allocating resources and planning RHD control programs.

Concepts: Medicine, Epidemiology, Diseases and disorders, Africa, Rheumatic fever, Rheumatology, Rheumatism, Resources

12

Fatigue is a frequent symptom in several inflammatory diseases, particularly in rheumatic diseases. Elements of disease activity and cognitive and behavior aspects have been reported as causes of fatigue in patients with rheumatoid arthritis. Fatigue could be associated with activity of inflammatory rheumatism. Indeed, biologic agents targeting inflammatory cytokines are effective in fatigue. Fatigue is also associated with pain and depressive symptoms. Different pathways could be involved in fatigue and interact: the immune system with increased levels of pro-inflammatory cytokines (interleukin-1 and -6 and tumor necrosis factor alpha), dysregulation of the hypothalamic-pituitary-adrenal axis and neurological phenomena involving the central and autonomic nervous systems. A pro-inflammatory process could be involved in pain and behavioral symptoms. Inflammation could be a common link between fatigue, pain, and depression.

Concepts: Immune system, Inflammation, Cancer, Infection, Rheumatoid arthritis, Rheumatology, Rheumatism, Tumor necrosis factor-alpha

9

In 2012, a European initiative calledSingle Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and uveitis is possibly its most devastating extra-articular manifestation. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. Consequently, treatment practices differ widely, within and between nations.

Concepts: Medicine, Rheumatic fever, Rheumatoid arthritis, Rheumatology, Arthritis, Juvenile idiopathic arthritis, Rheumatism, Specialty

7

The involvement of people of all ages including young people in research is now widely advocated but prioritisation of research topics is still driven largely by professional agendas. Evidence from adult literature has reported a mismatch between a researcher and patient generated list of research topics. There have been no studies to date exploring the priorities of young people with long term conditions other than in SLE. The study aimed to explore the research priorities of young people across the UK with respect to rheumatic conditions.

Concepts: Rheumatic fever, Rheumatology, Research, United Kingdom, Term, Cultural studies, Rheumatism, Explorer