• 대한영상의학회지
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• 제82권5호
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• pp.1281-1286
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• 2021

척추기저동맥의 혈류공급감소로 인한 뇌간 경색은 매우 드문 전신성 루푸스 환자의 초기 증상으로, 경색의 직접적인 원인으로 매우 작은 기저동맥 분지인 교뇌공급혈관의 박리성 동맥류는 보고된 사례가 없다. 이에 저자들은 디지털감산 혈관조영술과 고해상도 혈관벽 자기공명영상를 이용하여 작은 교뇌공급혈관의 박리성 동맥류의 진단과 추적관찰 중 치유된 20세 여성의 사례를 보고하고자 한다. 전신성 루푸스의 진단은 신경학적장애의 유무와 혈액화학검사 결과를 바탕으로 하였다. 추적 고해상도 혈관벽 자기공명영상에서 환자의 교뇌천공지의 박리성동맥류는 폐색되어 있었고 우측 척추동맥의 박리성동맥류는 치유되어 보이지 않았다. 환자는 퇴원 시 수정랭킨척도 점수가 1점으로 증상 개선을 보였으며 3개월과 12개월 추적관찰에서도 증상이 악화되지 않고 1점을 유지하였다.

• Journal of Ginseng Research
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• 제48권2호
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• pp.190-201
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• 2024

Background: Deposition of immune complexes drives podocyte injury acting in the initial phase of lupus nephritis (LN), a process mediated by B cell involvement. Accordingly, targeting B cell subsets represents a potential therapeutic approach for LN. Ginsenoside compound K (CK), a bioavailable component of ginseng, possesses nephritis benefits in lupus-prone mice; however, the underlying mechanisms involving B cell subpopulations remain elusive. Methods: Female MRL/lpr mice were administered CK (40 mg/kg) intragastrically for 10 weeks, followed by measurements of anti-dsDNA antibodies, inflammatory chemokines, and metabolite profiles on renal samples. Podocyte function and ultrastructure were detected. Publicly available single-cell RNA sequencing data and flow cytometry analysis were employed to investigate B cell subpopulations. Metabolomics analysis was adopted. SIRT1 and AMPK expression were analyzed by immunoblotting and immunofluorescence assays. Results: CK reduced proteinuria and protected podocyte ultrastructure in MRL/lpr mice by suppressing circulating anti-dsDNA antibodies and mitigating systemic inflammation. It activated B cell-specific SIRT1 and AMPK with Rhamnose accumulation, hindering the conversion of renal B cells into plasma cells. This cascade facilitated the resolution of local renal inflammation. CK facilitated the clearance of deposited immune complexes, thus reinstating podocyte morphology and mobility by normalizing the expression of nephrin and SYNPO. Conclusions: Our study reveals the synergistic interplay between SIRT1 and AMPK, orchestrating the restoration of renal B cell subsets. This process effectively mitigates immune complex deposition and preserves podocyte function. Accordingly, CK emerges as a promising therapeutic agent, potentially alleviating the hyperactivity of renal B cell subsets during LN.

• 한국임상수의학회지
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• 제41권1호
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• pp.49-53
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• 2024

A two-year-old, spayed female, 22.5 kg Pungsan was referred with chronic crusts and erosion on the nose. A referring veterinarian prescribed an anti-inflammatory dose (0.5-1 mg/kg/day) of oral glucocorticoids for 5 months, but skin lesions showed no meaningful improvement. A dermatological evaluation revealed a crust, depigmentation, erosion, and erythematous lesion over the nasal planum with a loss of the normal cobblestone texture. Also, firm and multifocal plaques over the thigh, groin, axilla, and dorsum were detected. A cytology examination on the nose, thigh, groin, axilla, and dorsum revealed moderate neutrophilic inflammation and bacterial infection. Abdominal radiography and ultrasonography revealed subcutaneous calcified materials along the thigh, groin, axilla, and dorsum. Calcinosis cutis was suspected because of the adverse effect of previous prolonged corticosteroid therapy. A histopathology examination of the nose lesion revealed moderate to severe degenerative or apoptotic changes of the basal layer and lymphoplasmacytic interface dermatitis. Facial discoid lupus erythematosus (FDLE) was diagnosed based on the history and the clinical, cytological, and histopathological results. Minocycline (7 mg/kg PO q 12 h) and niacinamide (500 mg/dog PO q 12 h) were prescribed as initial treatment. Glucocorticoids were not administered due to the presence of calcinosis cutis induced by previous corticosteroid treatment. After 6 weeks of treatment, the clinical signs on the nose were mildly improved. At this time, topical 1% pimecrolimus cream (twice daily) was initiated, while minocycline and niacinamide were continued at the same dose. The nasal planum markedly improved after 6 weeks of additional treatment, hence minocycline and niacinamide were prescribed for an additional 2 weeks and stopped, and the patient was continued solely on topical pimecrolimus. The dog's skin lesion has been maintained in clinical remission with topical 1% pimecrolimus twice daily for more than 5 months.

• Clinical and Experimental Pediatrics
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• 제49권4호
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• pp.381-387
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• 2006

목 적 : 신생아 루푸스는 전신성 홍반성 루푸스 산모의 자가 항체가 태반을 통해 태아에게로 넘어가 임상 증상을 보이는 질환으로 선천성 완전 방실 블록, 피부 병변, 간질환, 혈구 감소증을 특징으로 하는 질환이다. 이에 저자들은 전신성 홍반성 루푸스 산모의 출산아에서 선천성 완전 방실 블록의 빈도 및 출산아의 임상적 특징을 알아보고자 본 연구를 시행하였다. 방 법 : 1997년 1월부터 2005년 1월까지 한양대학교병원 류마티스 병원과 산부인과에서 진료받은 산모 55명의 57회의 임신결과와 태어난 49례의 신생아에 대한 임상기록을 후향적으로 검토하였다. 결 과 : 57례의 임신 중 5례(8.8%)의 자연유산, 1례(1.8%)의 사산이 있었고 15례(26.3%)의 조산, 8례(12.3%)의 부당 경량아의 빈도를 확인할 수 있었다. 57례의 임신 중 산전 초음파 검사에서 선천성 방실 블록 1례(1.8%)가 있었고 보호자 자의에 의해서 인공 유산이 행해졌다. 49례의 신생아 심전도 소견상 선천성 완전 방실 블록은 없었으며 모두 정상이었고 임상 기록상 피부병변이 있는 신생아는 없었다. 49례의 출산아 중 39례에서 혈액검사가 시행되었고 10례(25.6%)에서 혈액학적 이상이 있었다. 이 중 빈혈이 8례(20.5%), 중성구 감소증이 2례(5.1%), 혈소판 감소증이 3례(7.7%) 있었다. 자가 항체 anti-SSA(Ro)가 양성인 산모와 antiphospholipid 항체(aPL 항체)가 양성인 산모에서 미숙아 출산 빈도가 높았다(P=0.003, P=0.049). 항cardiolipin 항체(aCL 항체) 양성인 산모의 출산아가 인공환기요법을 받은 빈도가 높았다(P=0.018). 결 론 : 전신성 홍반성 루푸스 산모의 임신 중 선천성 완전 방실 블록의 빈도는 1.8%로 그 빈도가 낮았고 출산아 중 혈액학적 이상은 20% 이상에서 나타났다. 소홀히 하기 쉬운 피부병변에 대한 주의 깊은 추적 관찰로 신생아 홍반성 루푸스 진단에 관심을 가져야 할 것이다.

• 한국임상수의학회지
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• 제17권2호
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• pp.443-449
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• 2000

A three-year-old male jindo with generalized skin lesions (including seborrhea, hyperk- eratosis, alopecia, papules, and ecchymoses), pruritus lymph node enlargement, and fever was brought to Veterinary Teaching Hospital, College of Veterinary Medicine, Chungbuk National Uni- versity. There were no laboratory findings for parasites and fungi in the hair and skin But, the com- plete blood counts (CBC) showed leukocytosis and severe cosinophilia, It was suspected to be an inflammatory and allergic dermatitis. Thus, prednisoIone (0.5 mg/kg PO, BID for 1 week) and ampi- cillin (10 mg/kg PO, BID for 1 week given. One week later, pruritus and ecchymoses were reduced. These treatments were repeated for 7 day again. Three months later, the dog was presented again due to the relapse and exacerbation of the clinical signs. The signs were as follows; severe pru- ritus, vesicobullous skin lesions, anorexia, emaciation, lameness, and welling of carpal joints that showed inflammatory skin lesion and draining of synovia-like fluid. The values of WBC counts were returned to normal ranges. In contrast, eosinophilia was still observed. Coombs test for patient RBC and serum were negative. Hypoalbuminemia (2.5g/dl) was shown by serum chemistry. The uri- nalysis revealed and presence of leukocytes. Luxation finding of right radial carpal joint by polyarthritis was shown in radiography of affected joints.Lupus eryhematosus(LE) cells also appeared in peripheral blood and synovial fluid of affected joints. Definitely, antinuclear antibody (ANA) of patient serum using feline peripheral blood mononuclear cells was detected by all immu- nofluorescence. Based on these findings such as sedum ANA-Positive. major signs (skin disease, non- erosive polyarthritis with soft tissue swelling. and proteinuria), minor signs (fever), and LE cell-pos- itive, a diagnosis of systemic lupus crythematosus (SLE) was mad\ulcorner in this dog. The dog with SLE was administered with Pre(2.0 mg/kg PO, BID for first 4 week and then QOD) to inhibit the production of autoantibodies and with ampicillin (10 mg/kg PO, BID for first 4 weeks and then QOD) to prevent the secondary infection. The condition of this dog was monitored every 2 weeks by physical examinations, radiography, CBC, serum chemistry and urinalyais. At 8th week of treat- ment, the state of SLE evaluated by physical examinations and laboratory findings was markedly improved except for proteinura.

• Tuberculosis and Respiratory Diseases
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• 제52권1호
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• pp.76-85
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• 2002

BALT 림프종은 대부분 비호지킨스 림프종의 저등급의 B-세포 림프종으로서 쇼그렌씨 증후군 및 류마티스 관절염 등과 같은 자가면역질환과 관계가 있다고 알려져 있으나 전신성 홍반성 낭창에서 BALT 림프종의 발생에 대한 보고는 없는 것 같다. 저자들은 흉막성 통증을 주소로 내원한 54세 남자환자에서 전신성 홍반성 낭창으로 인한 흉막염의 원인규명과정에서 진단된 BALT 림프종에 대하여 문헌고찰과 함께 보고하는 바이다.

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

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

• BMB Reports
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• 제51권8호
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• pp.371-372
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• 2018

Cardiovascular disease such as atherosclerosis is caused by imbalanced lipid metabolism and represents a leading cause of death worldwide. Epidemiological studies show that patients with systemic autoimmune diseases exhibit a higher incidence of atherosclerosis. Conversely, hyperlipidemia has been known to accelerate the incidence of autoimmune diseases in humans and in animal models. However, there is a considerable gap in our understanding of how atherosclerosis impacts the development of the autoimmunity in humans, and vice versa. The atherosclerosis-related autoimmune diseases include psoriasis, rheumatoid arthritis, systemic lupus erythematosus (SLE) and diabetes mellitus. By using animal models of atherosclerosis and SLE, we have recently demonstrated that hyperlipidemia significantly accelerates the development of autoantibodies, by inducing autoimmune follicular helper T ($T_{FH}$) cells. Mechanistic studies have identified that hyperlipidemia induces IL-27 production in a TLR4-dependent manner, likely via downregulating LXR expression in dendritic cells. In this case, mice lacking IL-27 do not develop enhanced antibody responses. Thus it is noted that these findings propose a mechanistic insight responsible for the tight association between cardiovascular diseases and SLE in humans.

• Clinical and Experimental Reproductive Medicine
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• 제25권3호
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• pp.277-280
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• 1998

Antiphospholipid antibody is an immunoglobulin which may be of any class and which reacts with any phospholipid. For clinical use the definition of the term anti-phospholipid antibody is usually restricted to IgG and/or IgM antibody directed against the negatively charged phopholipids, cardiolipin, phosphatidyl inositol, phosphatidyl serine, or phosphatidic acid. The antigen of the serological test for syphilis is cardiolipin; negatively charged phopholipids are understood to be antigens to which lupus anticoagulants are directed. The term 'anticardiolipin' antibody syndrome, 'antiphospholipid' antibody syndrome, and 'lupus anticoagulant' syndrome are often, imprecisely, used interchangeably. We have experienced a case of recurrent spontaneous abortion with antiphospholipid antibody. So we report this case with a brief review of literatures.