• Title/Summary/Keyword: Lupus

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CD72 is a Negative Regulator of B Cell Responses to Nuclear Lupus Self-antigens and Development of Systemic Lupus Erythematosus

  • Takeshi Tsubata
    • IMMUNE NETWORK
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    • v.19 no.1
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    • pp.1.1-1.13
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    • 2019
  • Systemic lupus erythematosus (SLE) is the prototypic systemic autoimmune disease characterized by production of autoantibodies to various nuclear antigens and overexpression of genes regulated by IFN-I called IFN signature. Genetic studies on SLE patients and mutational analyses of mouse models demonstrate crucial roles of nucleic acid (NA) sensors in development of SLE. Although NA sensors are involved in induction of antimicrobial immune responses by recognizing microbial NAs, recognition of self NAs by NA sensors induces production of autoantibodies to NAs in B cells and production of IFN-I in plasmacytoid dendritic cells. Among various NA sensors, the endosomal RNA sensor TLR7 plays an essential role in development of SLE at least in mouse models. CD72 is an inhibitory B cell co-receptor containing an immunoreceptor tyrosine-based inhibition motif (ITIM) in the cytoplasmic region and a C-type lectin like-domain (CTLD) in the extracellular region. CD72 is known to regulate development of SLE because CD72 polymorphisms associate with SLE in both human and mice and CD72-/- mice develop relatively severe lupus-like disease. CD72 specifically recognizes the RNA-containing endogenous TLR7 ligand Sm/RNP by its extracellular CTLD, and inhibits B cell responses to Sm/RNP by ITIM-mediated signal inhibition. These findings indicate that CD72 inhibits development of SLE by suppressing TLR7-dependent B cell response to self NAs. CD72 is thus involved in discrimination of self-NAs from microbial NAs by specifically suppressing autoimmune responses to self-NAs.

Two Cases of Lupus Nephritis (Lupus Nephritis 2례)

  • Lee, Ki-Hyuck;Park, Young-Hoon;Hah, Jeong-Ok
    • Journal of Yeungnam Medical Science
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    • v.4 no.2
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    • pp.185-191
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    • 1987
  • The systemic lupus erythematosus is a self-perpetuating disease with multisystem involvement, ie ; skin, kidney, serous membrane, nervous system and other organs. The mortality in SLE is determined primarily by the extent of renal involvement ana the degree of immunosuppression resulting from the therapy. We experienced two cases of lupus nephritis in SLE with clinical, serologic, immunologic and pathologic evaluations. Renal biopsy revealed focal and segmental proliferative glomerulonephritis and mesangial proliferative glomerulonephritis. Both patients have been improving with prednisolone on follow-up studies.

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A case of Reversible Posterior Leukoencephalopathy Syndrome in Lupus Nephritis Treated with Cyclosporine (루프스 신염 치료로 Cyclosporine 투여 후 발생한 가역성후백질뇌병증 증후군)

  • Na, Sang-Jun;Lee, Kee-Ook
    • Annals of Clinical Neurophysiology
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    • v.11 no.1
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    • pp.24-27
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    • 2009
  • Reversible posterior leukoencephalopathy syndrome (RPLS) is an acute encephalopathy, usually characterized by seizures, visual disorder, mental change and reversible posterior white and gray matter lesions on brain magnetic resonance imaging (MRI). Conditions commonly associated with RPLS include acute renal failure, eclampsia, hypertension, immunosuppresion state, and use of intravenous immunoglobulin or chemotherapy, and additional contributing factors. We report the case of RPLS associated with cyclosporine use in lupus nephritis. In this case, it was not clearly elucidated which was main cause of RPLS. We could think RPLS developed maybe due to combined effect of cyclosporine and lupus nephritis.

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A Case of Neonatal Lupus with Abnormal Liver Function Test and Skin Lesion (피부 병변과 간기능 이상이 동반된 신생아 루푸스 1례)

  • Chung, Ju-Young;Chey, Myoung Jae
    • Clinical and Experimental Pediatrics
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    • v.48 no.1
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    • pp.85-87
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    • 2005
  • Neonatal lupus(NL) is characterized by typical clinical features and the presence of maternal autoantibodies. The principal serologic markers of NL are anti-Ro/SSA or anti-La/SSB maternal autoantibodies, which are transferred across the placenta and can be detected for the first few months of the affected child. The major clinical manifestations are cardiac disease, notably congenital heart block, and cutaneous lupus lesions. Hepatobiliary disease is relatively rare clinical manifestation of NL. We experienced a case of NL with abnormal liver function test and skin lesion.

Combination therapy of cyclosporine and prednisolone in a dog with systemic lupus erythematosus

  • Kim, Yeon-Hee;Kang, Min-Hee;Park, Hee-Myung
    • Korean Journal of Veterinary Research
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    • v.56 no.1
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    • pp.47-49
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    • 2016
  • An 11-year-old, spayed female poodle presented with fever and shifting lameness. Physical examination revealed hyperthermia ($40.6^{\circ}C$), and proteinuria was detected upon urinalysis. Increased neutrophils (83%) and decreased viscosity were revealed upon synovial fluid analysis. Serum antinuclear antibody was positive at 1 : 80. Based on these findings, the dog was diagnosed with systemic lupus erythematosus. Immunosuppressive therapy was initiated with prednisolone and cyclosporine, and the condition was markedly improved after the treatments. This case report describes the clinical and laboratory findings, imaging characteristics and successful outcomes after prednisolone plus cyclosporine therapy in a canine systemic lupus erythematosus case.

Two Cases of Systemic Lupus Erythematosus with Cerebrovascular Involvement (뇌 혈관계를 침범한 전신성 홍반성 루푸스 2례)

  • Kim, Bong-Jun;Lee, Eun-Young;Hong, Young-Hoon;Park, Ki-Do;Song, Young-Doo;Lee, Choong-Ki;Shim, Young-Ran
    • Journal of Yeungnam Medical Science
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    • v.15 no.2
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    • pp.371-380
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    • 1998
  • Systemic lupus erythematosus(SLE) is an autoimmune disease which may affect many different organs and disclose various clinical manifestations. Recently central nervous system(CNS) involvement has been recognized as an increasingly significant contributor to morbidity and mortality of SLE. The clinical manifestations of CNS-lupus are highly variable and range from mild cognitive dysfunction, movement disorder, headache, psychosis to life-threatening stroke and coma. Among the neuropsychiatric disorders encountered in patients with SLE, cerebrovascular disease has been a relatively rare complication. The diagnosis and management of CNS-lupus is difficult because of the lack of useful diagnostic methods. If cerebrovascular involvement is suspected, aggressive treatment such as high dose steroid, immunosuppressive therapy, plasma exchange may be required to reduce high mortality rate. We experienced 2 cases cerebrovascular dis eases occurring in SLE patients which presented with various neuropsychiatric manifestations. They were diagnosed as CNS-lupus by neuropsychiatric symptoms, brain MRI, and BEG, and showed good response to high dose steroid pulse therapy.

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One Case of Systemic Lupus Erythematosus treated with traditional Korean Medicine (전신성 홍반성 낭창(SLE)환자 1예(例)에 대한 증례 보고)

  • An, Chang-Suk;Kang, Kye-Seong;Kwon, Gi-Rok
    • Journal of Pharmacopuncture
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    • v.3 no.2
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    • pp.245-255
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    • 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.

Effect of Hypertension on Childhood-onset Systemic Lupus Erythematous in a Tertiary Medical Center in Korea

  • Kim, Jeong Yeon;Cho, Heeyeon
    • Childhood Kidney Diseases
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    • v.24 no.2
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    • pp.107-114
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    • 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.

A Case of Neonatal Lupus Erythematosus Associated with Anti-U1RNP Antibodies (항-U1RNP 항체 양성인 신생아 홍반성 루푸스 1례)

  • An, Byung-Hoon;Lee, Gu Chang;Yoon, Tae Young;Kim, Mi-Jung
    • Clinical and Experimental Pediatrics
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    • v.48 no.3
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    • pp.342-345
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    • 2005
  • Neonatal lupus erythematosus(NLE) is a distinct subset of lupus characterized by cutaneous findings, cardiac conduction defects, hepatic or hematologic abnormalities. These manifestations are associated with the presence of maternal auto-antibodies such as anti-SSA/Ro, anti-SSB/La, and rarely anti-RNP($U_1RNP$) antibodies. Cases of $U_1RNP$ antibody-positive NLE have somewhat atypical cutaneous manifestation without cardiac or systemic abnormalities. We report a case of cutaneous NLE associated with $U_1RNP$ antibodies.

Brain meningioma in a patient with systemic lupus erythematosus

  • Yoo, Byung-Woo;Ahn, Sung-Soo;Pyo, Jung Yoon;Byun, Se-Jin;Song, Jason Jungsik;Park, Yong-Beom;Lee, Soo-Kon;Song, Jung-Soo;Lee, Sang-Won
    • Journal of Yeungnam Medical Science
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    • v.33 no.2
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    • pp.159-161
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    • 2016
  • Brain meningioma, the most common benign brain tumor, has been reported to account for 13-26% of all intracranial tumors, with a crude incidence rate of 2.3 per 100,000 persons for all types of meningiomas. The prevalence of neuropsychiatric lupus erythematosus is 15-91% and its clinical manifestations are diverse: from mild cognitive dysfunction to serious neurological or psychiatric symptoms. Here, we report the first Korean patient with brain meningioma and systemic lupus erythematosus who had undergone surgical tumor resection.