JCR: Journal of Clinical Rheumatology最新文献

Background/objective: Despite advances in clinical care and treatment options, adolescents with lupus continue to experience adverse health outcomes. Poor adherence to medication regimens is a major contributor to these negative outcomes. The utility of short message service (SMS) in tracking barriers to adherence prospectively remains untested for adolescents with lupus. Our objectives were (1) feasibility of incorporating a Health Insurance Portability and Accountability Act (HIPAA)-compliant SMS text messaging application into REDCap and (2) acceptability of using SMS text messaging to track barriers to medication adherence in adolescents with lupus.

Methods: This study is a 12-week pilot cohort study of adolescents with SLE per the 1997 revised American College of Rheumatology. A REDCap-embedded HIPAA-compliant text messaging application was used to send biweekly messages with survey link to track medication adherence. Measures were completed. Descriptive statistics were used to summarize demographics, medical, and acceptability data. Response to text messages and survey completion rates were reported as a measure of feasibility.

Results: Most eligible adolescents approached agreed to participate (n = 17, 71% enrollment rate). The cellphone ownership rate among adolescents eligible for participation was 92%. Nine subjects responded to all text messages sent (53% response and completion rate). Eleven subjects (65%) responded to two thirds of the text messages. Overall, 77% of enrolled subjects completed at least half of the surveys sent. Reminders to complete surveys were sent to 30% of enrolled adolescents.

Conclusions: This study shows that embedding a HIPAA-compliant SMS text message application in REDCap is feasible and can be used to engage adolescents with chronic conditions in monitoring between clinic visits.

Abstract: Women with systemic chronic inflammatory disease, such as those with scleroderma, systemic vasculitis, and Sjögren syndrome, need preconception evaluation by a multidisciplinary team. Counseling and pregnancy management should be tailored to patients' needs, considering specific disease features, organ involvement, treatment options, and risk factors to minimize risks of maternal-fetal complications during pregnancy.Additionally, considerations regarding fertility, assisted reproductive techniques, and contraception also need to be addressed for these women.In this narrative review, we integrate the current published literature with our expert opinion to address the issues faced by patients with the aforementioned inflammatory conditions.

Abstract: Autoimmune rheumatic diseases (ARDs) often affect women during their reproductive years, and early studies of pregnancy in these patients reported high rates of adverse outcomes. Continuation or initiation of safe and effective medications in the preconception period is beneficial for maintaining or achieving disease quiescence throughout pregnancy thereby improving both maternal and pregnancy outcomes. The European Alliance of Associations for Rheumatology, the American College of Rheumatology, and the British Society for Rheumatology have published recommendations and guidelines regarding management of ARDs during pregnancy. The American College of Obstetricians and Gynecologists and the American Gastroenterological Association have also provided guidance statements with relevant recommendations. This review provides an overview of available recommendations for medication use in ARD pregnancy, with discussion of safety considerations for maternal and fetal well-being. Medications considered compatible with pregnancy include hydroxychloroquine, sulfasalazine, azathioprine, cyclosporine, tacrolimus, and TNF inhibitors. Methotrexate, mycophenolate, leflunomide, and cyclophosphamide should be avoided before and during pregnancy. Other medications, most of them newer, are largely discouraged for use in pregnancy due to inadequate data or concerns for neonatal immunosuppression, including non-TNF biologics and small molecule therapies. Further investigation is needed regarding effects of non-TNF biologics, biosimilars, and small molecules in pregnancy. Important efforts for the future will include improved methodologies to gather critical safety data, with consideration of inclusion of pregnant women in clinical trials, a complex and controversial issue. Long-term information on outcomes in offspring of treated women is lacking for many of these medications.

Introduction: Audiovestibular dysfunction has been reported in many autoimmune connective tissue diseases, including systemic sclerosis (SSc).

Objective: To assess the prevalence and features of audiological and vestibular disturbances in SSc patients and evaluate their relationship with disease duration, clinical features, nailfold videocapillaroscopy pattern, and immunologic profiles.

Method: A matched case-control study was conducted in a rheumatology clinic of a second-level hospital over 24 months. All patients underwent a detailed ear, nose, and throat examination, as well as audiometric and vestibular assessments, including pure tone audiometry, speech audiometry, immittance tests, and the Video Head Impulse Test.

Results: Thirty-five SSc patients and 24 healthy controls were included in the study. In the SSc group, subjective hearing loss was reported by 17.1% of patients, vertigo by 14.3%, tinnitus by 11.4%, and dizziness by 5.7%. Sensorineural hearing loss was identified in 42.9% of SSc patients, significantly higher than in the control group ( p = 0.013). There was no correlation between audiological manifestations and clinical symptoms, organ involvement, immunologic characteristics, and treatment. Vestibular dysfunction was detected in 60% of SSc patients, significantly higher than the control group ( p = 0.05). A significant correlation was found between abnormal Video Head Impulse Test and the presence of anti-RNA polymerase III and anti-Th/To antibodies ( p = 0.05 and p = 0.034, respectively).

Conclusion: Our study revealed an increased prevalence of sensorineural hearing loss and vestibulopathy in SSc patients.

Objective: ​​This study aimed to characterize the profile of myositis-specific and myositis-associated autoantibodies (MSAs/MAAs) in an Indian cohort of juvenile dermatomyositis (JDM) patients and correlate them with clinical features and outcomes.

Methods: Forty-three children diagnosed with JDM were enrolled for this observational study. Clinical details (presentation, course, and outcome) were noted in a predesigned proforma. Serum samples were tested for 16 MSAs/MAAs by line immunoassay. MSAs/MAAs were correlated with clinical features and outcome (defined as a complete clinical response [≥6 months' disease inactivity on medication] or complete remission [≥6 months' inactivity off all drugs]).

Results: Thirty-five subjects (81.4%) had at least 1 MSA/MAA detected. The most common antibodies were anti-NXP2 (n = 13, 30.2%), anti-TIF1γ (n = 10, 23.2%), and anti-MDA-5 (n = 8, 18.6%). No patient had anti-Ku, anti-Pm Scl-100, anti-PL-12, anti-EJ, anti-OJ, or anti-Ro52. Thirty-two patients (74.4%) attained a complete clinical response over a median follow-up duration of 14 months, among which 6 (13.9%) achieved complete remission over a median follow-up duration of 30 months. Anti-TIF1γ was associated with younger age at onset (≤3 years) (odds ratio [OR], 6.25; 95% confidence interval [CI], 1.15-34.12; p = 0.034) and disease flares after attaining complete response (OR, 10.18; 95% CI, 1.64-70.93; p = 0.013). Patients with anti-NXP2 had higher odds of severe muscular weakness (OR, 3.73; 95% CI, 0.95-14.59; p = 0.058) and truncal weakness (OR, 3.89; 95% CI, 0.97-15.64; p = 0.056). One child with anti-MDA-5 positivity had interstitial lung disease. We found no association between the MSA/MAA profile and the achievement of complete clinical response or remission.

Conclusions: MSAs/MAAs were identified in 81% of children with JDM in our study, which is higher than most other studies. The most frequently observed antibodies displayed a pattern consistent with other studies. Anti-TIF1γ was associated with a younger age at onset and disease flares even after attaining a complete clinical response. Anti-NXP2 had higher odds of severe muscular weakness. These observations suggest consistency in certain phenotypic associations observed across geographic boundaries.