• IMMUNE NETWORK
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• 제3권3호
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• pp.227-234
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• 2003

Background: Immunoglobulin (Ig) light chain repertoire has been implicated as a critical determinant in regulation of autoreactive B cells and production of pathogenic anti-DNA antibodies in systemic lupus erythematosus (SLE). We analyzed the impact of Ig ${\lambda}$ chain repertoire on development of autoimmunity in patients with SLE. Methods: We obtained genomic DNA from individual peripheral CD19+ B cells of 3 untreated active SLE patients, and amplified $V{\lambda}$ rearrangements from each single cell by polymerase chain reaction. Results: A total number of 208 $V{\lambda}J{\lambda}$ rearrangements were analyzed. Analyzed sequences included 158 productive rearrangements and 50 nonproductive rearrangements. The differences in $V{\lambda}$ gene usage in the productive and nonproductive repertoire of SLE patients were found compared to the non-autoimmune individuals. $V{\lambda}$ gene, 9A was significantly overrepresented in nonproducative repertoire of SLE patients (P=0.016). In the productive repertoire, $V{\lambda}$ genes, 3L and 1E were found more often in the SLE patients (P=0.001, P=0.043). When the productive and the nonproductive repertoires were compared, 9A was found significantly less in the productive repertoire in the SLE patients (P=0.000). There were no significant differences in the $J{\lambda}$ gene usage between SLE patients and non-autoimmune individuals, but $J{\lambda}2/3$ gene was the most frequently used in SLE, whereas $J{\lambda}7$ gene was the most frequently used in the normal subjects. In the productive SLE $V{\lambda}$ repertoire, 9.4% of the total sequences employed identical CDR3. It was particularly striking to find 7 identical versions of the 1G-$J{\lambda}2/3$ $V{\lambda}J{\lambda}$ rearrangements from one patient and 3 of the same sequence from another patient. Notably, identical $V{\lambda}$ junctions in the SLE patients utilized significantly more homologous joining compared to $V{\lambda}$ junctions of the normal adults (P=0.044). Conclusion: These data demonstrate regulation of ${\lambda}$ light chain expression in the SLE patients by selection of unique $V{\lambda}$ genes. Also, biased selection and clonal expansion of particular $V{\lambda}$ rearrangements are apparent in the SLE ${\lambda}$ repertoire.

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

• Annals of Clinical Neurophysiology
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• 제9권1호
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• pp.29-32
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• 2007

Recurrent transverse myelitis is a rare manifestation of systemic lupus erythematosus. Recurrent transverse myelitis presents the biggest diagnostic problem, since it is common manifestation of multiple sclerosis. But it can also be the only feature or first manifestation in systemic lupus erythematosus. Neurological manifestations and magnetic resonance imaging can be indistinguishable, and there are no specific diagnostic tools. Here we describe a 59-year-old female having a systemic lupus erythematosus with recurrent transverse myeltitis. No uniform therapeutic protocol exists for systemic lupus erythematous with transverse myelitis, and the prognosis is usually poor. We suggest that aggressive treatment (usually with pulses of methylprednisolone and cyclophosphamide) might improve the prognosis of systemic lupus erythematosus with transverse myeltis.

• 대한두경부종양학회지
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• 제38권2호
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• pp.23-27
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• 2022

Systemic lupus erythematosus(SLE) is a multisystemic disorder of autoimmune etiology. SLE can occur commonly in young women, and the early symptoms include fever, myalgia, arthralgia, weight loss, lymphadenopathy and these nonspecific symptoms develop into skin rash, splenomegaly, serositis and encephalopathy. Diagnosis of SLE requires clinical and serologic criteria, and treatment choices are hydroxyquinolone and NSAIDs for mild disease, corticosteroids and immunosuppressant for severe disease. In lupus patient, the prevalence of lymphadenopathy is 12~59%. Although lymphadenopathy is common finding in SLE, it is hard to distinguish in early phase of SLE. A 38-year-old woman visited our hospital for cervical lymphadenopathy with polyarthritis and malaise. Multiple cervical lymph nodes enlargement was found on Neck CT, and serologic laboratory test including ANA, antiphospholipid antibody, and anti-dsDNA was positive. For excluding lymphoma, PET-CT and excisional biopsy were performed. The patient finally diagnosed with SLE, and got regular follow-up without complication.

• IMMUNE NETWORK
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• 제2권4호
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• pp.227-232
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• 2002

Background: Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by diverse clinical manifestations and autoantibody production, which is known to be strongly influenced by genetic factors. Previous studies have revealed the associations of SLE with HLA class II alleles and antiphospholipid antibody system (anticardiolipin antibody (aCL) and anti-${\beta}_2$ glycoprotein I antibody (anti-${\beta}_2$ GPI)). Therefore, we studied the associations of HLA class II alleles with SLE and antiphospholipid antibody system. Methods: The genotyping for HLA-DRB1 and DQB1 alleles were performed in 61 SLE patients and 100 controls by the polymerase chain reaction (PCR)-sequence specific oligonucleotide probe method. ELISA tests for aCL and anti-${\beta}_2$ GPI were performed in 39 of the 61 SLE patients. The results were evaluated statistically by Chi-square test. Results: The frequencies of the HLA-$DRB1^*15$ and $DQB1^*06$ in SLE patients were significantly higher than those in controls. HLA-$DRB1^*12$ was significantly lower in SLE patients than controls. Nine of 39 patients were positive for aCL (IgG) and three were positive for aCL (IgM). One of 39 patients were positive for anti-${\beta}_2$ GPI (IgG) and none of them positive for anti-${\beta}_2$ GPI (IgM). Association of aCL with HLA class II alleles was not observed in our study. Conclusion: According to our results, it was found that HLA-$DRB1^*15$ and $DQB1^*06$ were associated with genetic susceptiblility and $DRB1^*12$ was associated with resistance to SLE in Korean population. No Association of aCL with HLA class II alleles was observed and the positive rate for anti-${\beta}_2$ GPI was very low.