• Tuberculosis and Respiratory Diseases
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• v.46 no.1
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• pp.96-102
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• 1999

A 40-year-old female presented with fever and cough which had lasted for 3 months. Three years previously systemic lupus erythematosus had been diagnosed, and at that time 10mg of prednisone per day was prescribed. Crackles were heard at the base of the lung and her chest roentgenogram revealed diffuse infitrates in both lower lung fields. Despite of empirical antibiotics and antituberculous medication consisting of isoniczid, rifampicin and ethambutol, the patients condition deteriorated. We performed open lung biosy and numerous nodules with necrosis as well as acid fast bacilli were observed. After biochemical tests and sequencing, the organism was identified as Mycobacterium abscessus. The patient was treated with imipenem and amikacin ; patient's condition, as indicated by both symptoms and roentogenogram, subsequently improved.

• Tuberculosis and Respiratory Diseases
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• v.65 no.4
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• pp.323-327
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• 2008

Systemic lupus erythematosus (SLE) is a multisystem disorder where the etiology is not clearly known. Symptomatic chronic interstitial pneumonitis is an uncommon manifestation, with a reported prevalence of 3~13%. Achalasia is rare disease that presents with failure in the relaxation of the esophagus sphincter. A 22-year-old woman was admitted to our hospital because of fever, cough and dyspnea. The patient had a history of pericardial effusion and Raynaud's phenomenon. The results of laboratory tests indicated the presence of lymphopenia and included positive antibody tests for antinuclear antibody and anti Sm antibody. A chest X-ray demonstrated the presence of peribronchial infiltration on both lung fields. A Chest CT image showed interlobar septal thickening, ground-glass opacity and a honeycomb appearance in both lung fields and esophageal dilatation with air fluid level. An esophagogram showed the presence of dilated esophagus ends that represented the non-relaxed lower esophageal sphincter. Manometry demonstrated incomplete sphincter relaxation. The case was diagnosed as systemic lupus erythematosus associated with interstitial pneumonia and achalasia.

• IMMUNE NETWORK
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• v.19 no.1
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• pp.7.1-7.11
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• 2019

Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disorder that affects mainly salivary and lacrimal glands, but its cause remains largely unknown. Clinical data indicating that SS occurs in a substantial proportion of patients with lupus points to common pathogenic mechanisms underlying the two diseases. To address this idea, we asked whether SS develops in the lupus-prone mouse strain sanroque (SAN). Owing to hyper-activation of follicular helper T (Tfh) cells, female SAN mice developed lupus-like symptoms at approximately 20 wk of age but there were no signs of SS at that time. However, symptoms typical of SS were evident at approximately 40 wk of age, as judged by reduced saliva flow rate, sialadenitis, and IgG deposits in the salivary glands. Increases in serum titers of SS-related autoantibodies and numbers of autoantibody-secreting cells in cervical lymph nodes (LNs) preceded the pathologic manifestations of SS and were accompanied by expansion of Tfh cells and their downstream effector cells. Thus, our results suggest that chronic dysregulation of Tfh cells in salivary gland-draining LNs is sufficient to drive the development of SS in lupus-prone mice.

• IMMUNE NETWORK
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• v.24 no.4
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• pp.25.1-25.14
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• 2024

Lupus is characterized by the autoantibodies against nuclear Ags, underscoring the importance of identifying the B cell subsets driving autoimmunity. Our research focused on the mitochondrial activity and CXCR4 expression in CD11c⁺ B cells from lupus patients after ex vivo stimulation with a TLR9 agonist, CpG-oligodeoxyribonucleotide (ODN). We also evaluated the response of CD11c⁺ B cells in ODN-injected mice. Post-ex vivo ODN stimulation, we observed an increase in the proportion of CD11c^hi cells, with elevated mitochondrial activity and CXCR4 expression in CD11c⁺ B cells from lupus patients. In vivo experiments showed similar patterns, with TLR9 stimulation enhancing mitochondrial and CXCR4 activities in CD11c^hi B cells, leading to the generation of anti-dsDNA plasmablasts. The CXCR4 inhibitor AMD3100 and the mitochondrial complex I inhibitor IM156 significantly reduced the proportion of CD11c⁺ B cells and autoreactive plasmablasts. These results underscore the pivotal roles of mitochondria and CXCR4 in the production of autoreactive plasmablasts.

• Archives of Plastic Surgery
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• v.43 no.5
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• pp.481-482
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• 2016

• The Journal of the Korean life insurance medical association
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• v.31 no.1
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• pp.19-28
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• 2012

Nowadays, Rheumatic diseases are increasing more and more. So, it's important knowing the pathophysiology and extra-risk of each rheumatic disease so as to do sound underwriting. Here is brief review and long-term prognosis of some rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, Sj$\ddot{o}$gren syndrome, antiphospholipid syndrome, systemic sclerosis, ankylosing spondylitis, Takayasu's arteritis, and Behcet syndrome.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.381-387
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• 2006

Purpose : Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. Methods : The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. Results : There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). Conclusion : The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.

• Toxicological Research
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• v.33 no.3
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• pp.205-209
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• 2017

Transmembrane Protein 39A (TMEM39A) is a member of TMEM family. The understanding about this protein is still limited. The earlier studies indicated that TMEM39A was a key mediator of autoimmune disease. TMEM39A seems to be involved in systemic lupus erythematosus and multiple sclerosis in numerous of populations. All of these works stop at insufficient information by using gene functioning methods such as: Genome-wide association studies (GWASs) and/or follow-up study. It is the fact that the less understood of TMEM39A actually is the attraction to the scientist in near future. In this review the current knowledge about TMEM39A and its possible roles in cell biology, physiology and pathology will be described.

• Journal of Korean Public Health Nursing
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• v.16 no.1
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• pp.105-114
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• 2002

This study was to investigate the level of fatigue and its associated variables in patients with systemic lupus erythematosus(SLE) in Korea. From March to June, 2001, 100 patients, registered at one university hospital rheumatology clinic in Seoul, were accepted as subjects for this study. The sampling method was adopted a non-probability, purposive technique. The instruments used for this study were The Multidimensional Assessment Fatigue scale developed by Tack and Beck Depression Instrument develped by Beck. The collected data were analyzed by SAS program using t-test, ANOVA with Scheffe-test, Pearson correlation coefficients and stepwise multiple regression. The results were as follows: 1. Total scores of fatigue of the subjects averaged $24.46(\pm10.85)$, degree of fatigue was $5.08(\pm2.29)$, and influence of fatigue was $3.52(\pm2.12)$. 2. Regarding characteristics, more depressive(p=.0001) and more painfuI(p=.0122) patients revealed more fatigue. Also, the subjects with spouse(p=.0337) and having poor quality of sleep(p=.0445) revealed more fatigue. 3. The subjects' total fatigue score, depression, pain and age was correlated positively(r=.53; r=.48; r=.24), and total fatigue score, and exercise time, quality of sleep was correlated negatively(r=-.45; r=-.21). 4. The main influencing factors on the fatigue were depression$(52.92\%)$ and quality of sleep $(8.10\%)$. These two main variables made it possible to explain $61.02\%$ of the varience in fatigue. In conclusion, this study revealed depression and quality of sleep is an important factor that can improve quality of life in patients with SLE. It is recommended that nursing intervention for SLE patients would be focused to decrease depression and to enhance quality of sleep.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.155-168
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• 2002

Purpose; Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. Methods: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. Results: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1(2-18) years old. FANA(95.0%), anti-ds DNA antibody(87.5%), malar rash(80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria(75.0%), nephrotic syndrome(55.0%), and microscopic hematuria alone(15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was $51.8{\pm}40.5$ months. During $51.8{\pm}40.5$ months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. Conclusion: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.