• International Journal of Stem Cells
• /
• 제15권3호
• /
• pp.233-246
• /
• 2022

Background and Objectives: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease mainly affecting young women of childbearing age. SLE affects the skin, joints, muscles, kidneys, lungs, and heart. Cardiovascular complications are common causes of death in patients with SLE. However, the complexity of the cardiovascular system and the rarity of SLE make it difficult to investigate these morbidities. Patient-derived induced pluripotent stem cells (iPSCs) serve as a novel tool for drug screening and pathophysiological studies in the absence of patient samples. Methods and Results: We differentiated CMs from HC- and SLE-iPSCs using 2D culture platforms. SLE-CMs showed decreased proliferation and increased levels of fibrosis and hypertrophy marker expression; however, HC-and SLE-monolayer CMs reacted differently to SLE serum treatment. HC-iPSCs were also differentiated into CMs using 3D spheroid culture and anti-Ro autoantibody was treated along with SLE serum. 3D-HC-CMs generated more mature CMs compared to the CMs generated using 2D culture. The treatment of anti-Ro autoantibody rapidly increased the gene expression of fibrosis, hypertrophy, and apoptosis markers, and altered the calcium signaling in the CMs. Conclusions: iPSC derived cardiomyocytes with patient-derived serum, and anti-Ro antibody treatment could serve in effective autoimmune disease modeling including SLE. We believe that the present study might briefly provide possibilities on the application of a combination of patient-derived materials and iPSCs in disease modeling of autoimmune diseases.

• Journal of Preventive Medicine and Public Health
• /
• 제56권2호
• /
• pp.154-163
• /
• 2023

Objectives: Systemic lupus erythematosus (SLE) or lupus patients usually experience various physical and psychological challenges. Since the coronavirus disease 2019 pandemic, these challenges have become even harsher. Using the participatory action research approach, this study evaluated how an e-wellness program (eWP) impacted SLE-related knowledge and health behaviors, mental health, and quality of life among lupus patients in Thailand. Methods: A 1-group, pretest-posttest design study was conducted among a purposive sample of lupus patients who were members of Thai SLE Foundation. The 2 main intervention components were: (1) online social support, and (2) lifestyle and stress management workshops. Sixty-eight participants completed all the study requirements, including the Physical and Psychosocial Health Assessment questionnaire. Results: After being in the eWP for 3 months, participants' mean score for SLE-related knowledge increased significantly (t=5.3, p<0.001). The increase in sleep hours was statistically significant (Z=-3.1, p<0.01), with the percentage of participants who slept less than 7 hours decreasing from 52.9% to 29.0%. The percentage of participants reporting sun exposure decreased from 17.7% to 8.8%. The participants also reported significantly lower stress (t(66)=-4.4, p<0.001) and anxiety (t(67)=-2.9, p=0.005). The post-eWP quality of life scores for the pain, planning, intimate relationship, burden to others, emotional health, and fatigue domains also improved significantly (p<0.05). Conclusions: The overall outcomes showed promising results of improved self-care knowledge, health behaviors, mental health status, and quality of life. It is recommended that the SLE Foundation continues to use the eWP model to help the lupus patient community.

• IMMUNE NETWORK
• /
• 제14권3호
• /
• pp.138-148
• /
• 2014

MicroRNAs (miRNAs) are endogenous small RNA molecules best known for their function in post-transcriptional gene regulation. Immunologically, miRNA regulates the differentiation and function of immune cells and its malfunction contributes to the development of various autoimmune diseases including systemic lupus erythematosus (SLE). Over the last decade, accumulating researches provide evidence for the connection between dysregulated miRNA network and autoimmunity. Interruption of miRNA biogenesis machinery contributes to the abnormal T and B cell development and particularly a reduced suppressive function of regulatory T cells, leading to systemic autoimmune diseases. Additionally, multiple factors under autoimmune conditions interfere with miRNA generation via key miRNA processing enzymes, thus further skewing the miRNA expression profile. Indeed, several independent miRNA profiling studies reported significant differences between SLE patients and healthy controls. Despite the lack of a consistent expression pattern on individual dysregulated miRNAs in SLE among these studies, the aberrant expression of distinct groups of miRNAs causes overlapping functional outcomes including perturbed type I interferon signalling cascade, DNA hypomethylation and hyperactivation of T and B cells. The impact of specific miRNA-mediated regulation on function of major immune cells in lupus is also discussed. Although research on the clinical application of miRNAs is still immature, through an integrated approach with advances in next generation sequencing, novel tools in bioinformatics database analysis and new in vitro and in vivo models for functional evaluation, the diagnostic and therapeutic potentials of miRNAs may bring to fruition in the future.

• 대한약침학회지
• /
• 제3권2호
• /
• pp.245-255
• /
• 2000

After observing a patient diagnosed with Systemic Lupus Erythematosus from the September 21, 2000 to the January 13, 2001, the following results are obtained. Method and Result: We treated one case of Systemic Lupus Erythematosus with the Korean Bee Venom Therapy and the other Oriental Medical treatments. In acupuncture therapy, Korean Bee-venom Therapy is believed to be outstanding way to treat Systemic Lupus Erythematosus. Korean Bee Venom Therapy is treated on the following acupuncture points: ST36(B23(Shinsu:腎兪), BL26(Guanyuanshu:關元兪), ST36(Chok-Samni:足三里), LI4(Hapkok:合谷), LV3(Taechung), SP10(Hyolhae:血海) SP6 (Samumgyo;三陰交). In addition, CFC(Carthami Flos;紅花 and Cervi Pantotrichum Cornu;) Herbal-Acupuncture was applied on the acupucture points of GB20(Pungji: 風池), GB21(Kyonjong:), as well as BUM(It was made of Boviscalculus(牛黃), Moschus and Fel ursi Herbal-Acupuncture was given on CV17(Chonjung). In herbal medication, based on the Sasang Constitution, Taeyumin Chungsimyunja-Tang was given to the patient. As the results of these treatments, general conditions of this patient improved drastically. Conclusion: Based on the clinical results, traditional Korean Medical treatment is believed to be effective for treating Systemic Lupus Erythematosus, and futher studies should be carried out to provide more valuable information.

• Journal of Yeungnam Medical Science
• /
• 제15권2호
• /
• pp.371-380
• /
• 1998

저자들은 최근 루푸스가 뇌 혈관계를 침범하여 신경 정신과적 이상 증상을 보였으며 면역 검사, 뇌파 검사 및 뇌 자기공명사진에서 중추 신경 루푸스의 소견에 부합되는 양상을 보였고 고용량의 부신피질 호르몬 충격 요법에 반응이 좋았던 2례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
• /
• 제24권2호
• /
• pp.107-114
• /
• 2020

Purpose: The purpose of this study was to evaluate the prevalence, clinical characteristics, and long-term clinical effects of hypertension in Korean childhood-onset systemic lupus erythematous (SLE) patients. Methods: The medical records of SLE patients, diagnosed by 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, who visited Samsung Medical Center from January 2009 to May 2019 were reviewed. Disease activity and long-term damage were evaluated using the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Pediatric Systemic Lupus International Collaborating Clinics/ACR Damage Index (Ped-SDI), respectively. The sex-, age- and height-blood pressure standards recommended by the American Academy of Pediatrics 2017 guideline was used to define hypertension. Results: A total of 32 patients were enrolled in this study. The median follow-up duration was 7.3 years and females were predominant. The median ages at SLE and hypertension diagnoses were 14.2 and 14.3 years, respectively. The biopsy-proven lupus nephritis was detected in 90.6% and 37.5% were class IV. During the follow-up, 12 patients (37.5%) had hypertension. Among them, 2 patients had 3 episodes of posterior reversible encephalopathy syndrome and 5 patients had left ventricular hypertrophy (LVH). Univariate analysis showed baseline hypertension was significantly correlated with a lower estimated glomerular filtration rate, higher body mass index and SLEDAI at baseline. The development of hypertension during the follow-up was significantly correlated with obesity, LVH, and higher Ped-SDI. Conclusion: Our study revealed that hypertension in pediatric SLE is associated with obesity and renal function at SLE diagnosis and could affect long-term damage.

• Journal of Yeungnam Medical Science
• /
• 제27권1호
• /
• pp.78-84
• /
• 2010

Adult-onset Still's disease (AOSD) is an inflammatory disorder that's characterized by daily, spiking high fever, arthritis and an evanescent, salmon-pink rash. AOSD is diagnosed purely on the basis of the typical clinical features of the illness. The symptoms commonly include swelling of the lymph nodes, enlargement of the spleen and liver, and a sore throat. AOSD is difficult to differentiate from systemic lupus erythematosus (SLE) due to the similar clinical manifestations. We report here on a case of a 16-year-old female patient with autism and epilepsy and who complained of daily spiking fever for 20 days. The patient had maculopapular skin rashes on the face and whole body and lymphadenopathy. The liver function tests were elevated mildly. The initial rheumatoid factor (RF) and antinuclear antibody (ANA) tests were negative. We diagnosed her as having adult-onset Still's disease according to the criteria of Yamaguchi. We successfully treated her with oral prednisolone. But her antinuclear antibody test was changed to positive after discharge. So we finally diagnosed her as having SLE.

• Investigative Magnetic Resonance Imaging
• /
• 제17권2호
• /
• pp.144-148
• /
• 2013

대부분의 가역적 후두부 뇌병증 증후군 (PRES)는 가역적인 경과를 보이나 출혈 또는 경색을 동반한 비가역적 경과들도 보고되고 있다. 전신성 홍반성 낭창 (SLE)과 관련된 PRES는 고혈압이나 면역억제제와 관련이 있다. 저자들은 최초 PRES발병 시 확산강조영상에서 확산제한을 보인 부위에서, PRESS의 반복 발생 시 출혈성 변환을 보인 증례를 보고한다.

• Pediatric Infection and Vaccine
• /
• 제22권3호
• /
• pp.210-215
• /
• 2015

대식세포 활성 증후군(MAS, Macrophage activation syndrome)은 전신 홍반성 루푸스(SLE, systemic lupus erythematous) 환자에서 감염에 의해 나타날 수 있는 드문 합병증이다. MAS는 기저의 자가면역질환의 임상양상과 유사하게 나타나거나 혹은 감염성 합병증과 혼돈될 수 있어 감별에 주의 하여야 한다. 14세 여환이 2주간 지속되는 발열과 통증을 동반하는 얼굴의 피부 발진을 주소로 내원하였다. 피부 발진과 간비대, 범혈구 감소증, aspartate aminotransferase, lactate dehydrogenase, 혈청 ferritin이 상승하여, MAS를 의심하였다. 피부 병변과 항핵체 양성, C3와 C4의 감소, 간접 쿰스검사 양성으로 SLE를 진단하였다. 따라서 본 증례는 MAS가 HSV에 의하여 촉발된 것을, SLE의 첫 증상으로서 나타낸 증례로서, 촉발 요인 및 기저질환을 치료함으로써 중증의 합병증 없이 호전되었다.

• Journal of Chest Surgery
• /
• 제37권7호
• /
• pp.613-616
• /
• 2004

전신성 홍반성 낭창은 자가면역질환의 일종으로 피부, 신장 및 심장에 빈번히 침범하는 질환으로 알려져 있으며, 심장에서는 심외막염, 심근염 및 심장 판막 질환을 발생시킬 수 있다. 전신성 홍반성 낭창에서의 심장 판막 질환은 주로 승모판막 및 대동맥 판막 폐쇄부전증인 것으로 알려져 있으며, 대동맥 판막의 협착증이 보고는 극히 적었다. 또한, 판막성 심장 질환이 발생하여도 다른 장기의 손상으로 인하여 수술을 받는 경우는 매우 드물며 국내보고도 확인되지 않았다. 저자들은 전신성 홍반성 낭창으로 받아오던 59세 여자 환자에게서 대동백 판막 협착증이 발생하였으며 이를 수술 치험하였기에 보고하는 바이다.