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PubMed2004

The study of the participation of basement membrane zone antibodies in the formation of the lupus band in systemic lupus erythematosus.

BACKGROUND: The lupus band test (LBT) is an important auxiliary method in the diagnosis of systemic lupus erythematosus (SLE), but the mechanism of its formation is still unknown. There are many kinds of autoantibodies, such as basement membrane zone autoantibodies (BMZ-Abs), in patients with SLE. AIM: To detect whether skin BMZ-Abs participated in the formation of the lupus band in SLE. Methods Immunoglobulin G (IgG)-type BMZ-Abs in 15 SLE patients were detected by means of immunofluorescence (IF), immunoblotting (IB), and immunoelectron microscopy (IEM). RESULTS: Direct immunofluorescence (DIF) on salt-split skin showed epidermal fluorescence in 12 of the 15 SLE patients. Two of the 12 patients also showed dermal fluorescence. Indirect immunofluorescence (IIF) on salt-split skin revealed that, in 12 of the 15 (80%) sera, antibodies were bound to the epidermal roof of the salt-split skin. IB showed that, in 14 SLE sera, autoantibodies reacted to 230, 200, 180, 130, and 97 kDa epidermal extracts and 75 kDa dermal extracts. Direct (DIEM) and indirect (IIEM) immunoelectron microscopy showed that gold particles were directed to every region of the BMZ, including hemidesmosomes, lamina lucida, lamina densa, and sublamina densa. CONCLUSIONS: BMZ-Abs in SLE sera may participate in the formation of the lupus band.

Autoantibodies↗
PubMed2001

Successful delivery in a pregnant woman with lupus anticoagulant positive systemic lupus erythematosus treated with double filtration plasmapheresis.

The case was a 29 year old female who has suffered from systemic lupus erythematosus (SLE) since 15 years of age. The activity of SLE was low, and she took prednisolone orally. Her first pregnancy failed after 14 weeks. In the second pregnancy, she had thrombocytopenia, prolonged activated partial thromboplastin time (APTT), positive lupus anticoagulant (LAC) and thus was diagnosed with antiphospholipid antibody syndrome (APS). Combination therapy with steroids and aspirin was started, and she underwent treatment of double filtration plasmapheresis (DFPP) in the early stage of pregnancy. Her platelet count increased, and the value of APTT has normalized with DFPP treatment. She delivered successfully on the 32nd week of pregnancy. We think that DFPP is an effective and safe treatment in patients with an LAC positive pregnancy.

Adult↗
PubMed1998

Subacute cutaneous lupus erythematosus versus systemic lupus erythematosus: diagnostic criteria and therapeutic implications.

BACKGROUND: The nosologic position of subacute cutaneous lupus erythematosus (SCLE) is controversial. More than four American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE) are found in a proportion of patients diagnosed as having SCLE; thus such cases could be classified as SLE. OBJECTIVE: Our purpose was to determine whether ARA criteria for SLE are helpful in differentiating SCLE from SLE and whether cutaneous and visceral changes, immunologic findings, and photosensitivity provide a basis for diagnosis of SCLE. METHODS: A cohort of 143 patients (79 with SCLE, 58 with SLE, and six with overlapping features of SCLE and SLE) was studied clinically, histologically, and immunologically as well as by phototesting. The patients were observed for up to 10 years, and the course of the disease and response to therapy were evaluated in each group. RESULTS: SCLE differed from SLE by cutaneous changes, significantly less frequent kidney involvement, serositis and arthritis, and the rare presence of double-stranded DNA, U1RNP, and Sm antibodies characteristic of SLE. Ro(SS-A) and La(SS-B) antibodies were detected with similar frequency, and photosensitivity was not related to the presence of Ro antibody. In contrast, photoreproduction (appearance of LE lesion in irradiated area) was significantly more frequent in patients with SCLE. The course of SCLE in older patients was less severe than in younger patients, and aggressive therapy was usually not required. CONCLUSION: Patients with SCLE (although the majority fulfill more than four ARA criteria for SLE) show significant differences from those with SLE in terms of cutaneous and visceral involvement, immunologic findings, photosensitivity, course of the disease, and the requirement for therapy. Therefore SCLE should be recognized as a separate subset. However, cases of overlapping SLE and SCLE suggest a close relation.

Adult↗
PubMed1990

Lupus anticoagulants in systemic lupus erythematosus: prevalence and clinical associations.

The prevalence of lupus anticoagulant (LAC) and its relation with reported clinical associations has been determined in 55 patients with systemic lupus erythematosus (SLE) from northern India who were studied prospectively. Kaolin clotting time was used to screen for LAC, which was detected in seven (13%) of the patients. Significant associations were found between LAC and thrombotic events, onset of disease at an early age, and disease of shorter duration. No statistically significant association could be found between LAC and recurrent abortions, pulmonary hypertension, thrombocytopenia, and neurological manifestations. It is concluded that LAC is a useful marker for a subset of patients with SLE at risk of thromboembolic events.

Adolescent↗
PubMed1995

Tc-99m DTPA aerosol lung clearance test in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) can affect every organ. Involvement of the lung in systemic lupus erythematosus may be a significant cause of morbidity and mortality. To evaluate alveolar epithelial damage in SLE, we studied lung epithelial permeability by measuring the clearance of inhaled Tc-99m DTPA aerosol. Twenty-three SLE patients without clinical pulmonary manifestations were studied. Of the 23 patients, 16 had normal clearance rates of Tc-99m DTPA (T1/2 > 60mins) and normal chest X-ray findings. A mild increase in the clearance rate was found in 5 cases (T1/2 between 40-60 min) and a significant increase in clearance rate in the remaining 2 (T1/2 < 40mins). Totally, 30% (7/23) of SLE patients have abnormal lung permeability. Of the 7 patients with abnormal clearance of Tc-99m DTPA, 3 had abnormal findings in chest X-rays and four had normal chest X-rays. Our study suggests that the clearance of Tc-99m DTPA aerosol may be a useful complementary study to assess pulmonary involvement in SLE.

Adolescent↗
PubMedDate not supplied

[The role of anti-nucleosome antibodies in systemic lupus erythematosus. Results of a study of patients with systemic lupus erythematosus and other connective tissue diseases].

OBJECTIVE: The aim of our study was to investigate the prevalence and the disease specificity of anti-nucleosome antibodies in systemic lupus erythematosus and their association with disease activity and renal involvement. METHODS: Anti-nucleosome antibodies were measured by ELISA in the sera of patients with systemic lupus erythematosus (SLE) (47), rheumatoid arthritis (RA) (22), mixed connective tissue disease (MCTD) (19), systemic sclerosis (SSc) (11) and Sjögren's syndrome (SS) (10). Anti-dsDNA antibodies were measured by IIF on Crithidia luciliae. In the patients with SLE serum levels of C3 and C4 complement components were also measured. Sera of 22 healthy individuals were assayed as controls. SLE activity was evaluated by the ECLAM score. RESULTS: Anti-nucleosome antibodies were found in 40 patients with SLE (85.1%), in 10 with RA (45.4%), in 8 with MCTD (42.1%), in 4 with SSc (36.3%), in 1 with SS (10%) and in none of the healthy controls. Anti-dsDNA antibodies were found in 23 patients with SLE and were absent in the patients with other CTD and in controls. All the patients with SLE and renal involvement were positive both for anti-dsDNA antibodies and anti-nucleosome antibodies. No significant correlation was observed between anti-nucleosome antibodies and disease activity and renal involvement. CONCLUSION: Anti-nucleosome antibodies are present in a high percentage of the patients with SLE but they don't seem to be specific markers of the disease. Our data don't support a clear correlation between anti-nucleosome antibodies and disease activity and renal involvement.

Adolescent↗
PubMed2003

B cell loss leading to remission in severe systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) pathogenesis is mediated in part by autoantibodies. We describe a patient with central nervous system lupus who developed a loss of B cells with associated hypogammaglobulinemia and sinopulmonary infections requiring intravenous immunoglobulin. The SLE went into complete remission. Of 18 reported patients with SLE developing persistent hypogammaglobulinemia, only 5 patients including ours had a nearly complete loss of circulating B cells. Of those whose SLE and B cell status was reported, 5/5 with B cell loss and 1/10 without B cell loss experienced a durable response of SLE (p = 0.002). These cases illustrate that B cell ablative therapies may have efficacy for SLE.

Adult↗
PubMed2004

High-dose immunosuppression with autologous stem cell transplantation in severe refractory systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an immune-mediated disease that is responsive to suppression or modulation of the immune system. Patients with SLE who experience persistent multiorgan dysfunction, despite standard doses of intravenous cyclophosphamide (Cy), represent a subset of patients at high risk of early death. We investigated the efficacy and toxicity of high-dose immunosuppression and autologous hematopoietic stem cell transplantation (SCT) to treat such patients. Six patients (all female, age 15-29 years) with severe refractory SLE were enrolled in the clinic of our institution from 1998 to 2003. All patients were seriously ill, with SLE disease activity indices (SLEDAI) of 6-30, including two cases with central nervous system lupus, one case with lung vasculitis, and three cases with nephritis and nephrotic syndrome. All patients were registered in the European Group for Blood and Marrow Transplantation (EBMT)/European League Against Rheumatism (EULAR) database. Previous immunosuppression included pulse Cy intravenous, prednisolone (standard doses and pulse therapy), oral Cy and azathioprine, with little or no effect on disease progression. Autologous hemopoietic stem cells were collected from bone marrow (n = 4) or mobilized from peripheral blood with Cy and granulocyte colony-stimulating factor (G-CSF) (n = 2). Pre-transplant conditioning regimens included BEAM +/- ATG (n = 2), melphalan 140 mg/m2 + etoposid 1600 mg/m2 (n = 2) and Cy 200 mg/kg +/- ATG (n = 2). Median time to an absolute neutrophil count (ANC) greater than 0.5 x 10(9)/L and platelet count greater than 50 x 10(9)/L was 13 and 15 days, respectively. Three patients died on days 11, 22 and 63 due to transplant-related complications. The follow-up is now 60 and six months for two patients (complete remission), and 42 months for one other patient (partial response). All patients had experienced multiple and severe episodes of infections pre-SCT and long-term history of corticosteroid therapy (3-14 years). We conclude that achievement of prolonged, corticosteroid-free remissions is a reality. Judicious selection of patients earlier in disease or in remission, but with a high risk of relapse or further progression, will diminish transplantation-related mortality.

Adolescent↗
PubMed2000

Favourable interactions of cytostatic and steroid drugs in treatment of systemic lupus erythematosus.

Systemic lupus erythematosus (referred to below as SLE) is a disorder associated with formation of immune complexes in the circulatory system and their deposition in the internal organs, as well as in the skin. The disease can appear at any age, but the vast majority of cases are women between 15 and 40 years of age. The paper presents the case of a thirty-year-old woman with systemic lupus erythematosus. She was admitted to hospital because of erythematous and haemorrhagic lesions in the skin of her face, hands and feet and febrile states. SLE was diagnosed in the patient on the basis of criteria established by the American Rheumatological Association. The following abnormalities were observed in the patient: skin lesions of erythematous type on the face, hypersensitivity to UV light, erosions of the mucous membranes of the oral cavity, changes in the kidneys confirmed by biopsy, haematological symptoms, immune disorders. Additional criteria to establish the diagnosis included: febrile states. Raynaud's sign, hair loss, presence of immunoglobulin complexes in the skin, decreased complement level. The paper discusses modern therapy of visceral lupus erythematosus, based on corticosteroids and immunosuppressants. The interactions of these drugs were observed in the patient. The woman was treated with combined corticosteroid and cytostatic therapy, which led to the remission of the pathologic process confirmed by the clinical condition of the patient and laboratory tests.

Adrenal Cortex Hormones↗
PubMed2005

The role of host CD4 T cells in the pathogenesis of the chronic graft-versus-host model of systemic lupus erythematosus.

Systemic lupus erythematosus is characterized by production of autoantibodies and glomerulonephritis. The murine chronic graft-vs-host (cGVH) model of systemic lupus erythematosus is induced by allorecognition of foreign MHC class II determinants. Previous studies have shown that cGVH could not be induced in CD4 knockout (CD4KO) mice. We have further explored the role of host CD4 T cells in this model. Our studies now show that B cells in CD4KO mice have intrinsic defects that prevent them from responding to allohelp. In addition, B cells in CD4KO mice showed phenotypic differences compared with congeneic C57BL/6 B cells, indicating some degree of in vivo activation and increased numbers of cells bearing a marginal zone B cell phenotype. The transfer of syngeneic CD4 T cells at the time of initiation of cGVH did not correct these B cell abnormalities; however, if CD4 T cells were transferred during the development and maturation of B cells, then the B cells from CD4KO mice acquire the ability to respond in cGVH. These studies clearly indicate that B cells need to coexist with CD4 T cells early in their development to develop full susceptibility to alloactivation signals.

Adoptive Transfer↗
PubMed2002

Fcgamma receptor IIIA polymorphism in Korean patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is characterized by the presence of various autoantibodies and the deposition of immune complex, which is cleared by Fcgamma receptors. Genotype analysis was done to investigate whether the FcgammaRIIIA-176F/V polymorphism is a risk factor for SLE in Koreans. We genotyped 145 Korean SLE patients and 75 control subjects for FcgammaRIIIA-176F/ V. After amplifying a 1.7-kb fragment containing the Fcgamma/RIIIA-176F/V polymorphic site using two FcgammaRIIIA gene-specific primers, we performed a nested polymerase chain reaction (PCR) for allele-specific genotyping at position 559 in FcgammaRIIIA. FcgammaRIIIA genotype or allele distribution was not significantly different between lupus patients and controls, and also between lupus nephritis patients and healthy controls. Neither creatinine clearance, 24 h urine proteinuria, number of American College of Rheumatology (ACR) criteria, nor the Systemic Lupus International Collaborating Clinics (SLICC)/ACR damage index was different according to the genotype. In conclusion, FcgammaRIIIA-176F/V polymorphism is not associated with SLE in Koreans.

Asian People↗
PubMed2006

CD134 expression on CD4+ T cells is associated with nephritis and disease activity in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is characterized by a deviation of the immune system that involves T cell-dependent autoantibody production. The aim of this study was to investigate the role of co-stimulatory markers on T cells in this disease. Twenty-eight patients with SLE as defined by the American College of Rheumatology (ACR) criteria and 11 healthy controls were included into the study. Eleven patients had biopsy-proven lupus nephritis while 17 patients had no clinical evidence of lupus nephritis. Clinical disease activity was assessed according to the systemic lupus erythematosus disease index (SLEDAI). CD4+ T cell populations in the peripheral blood were analysed for the expression of co-stimulatory markers CD45RO, CD70, CD80, CD86, CD137, CD137L, CD134, CD152, CD154 and ICOS. SLE patients showed an increased frequency of peripheral CD4+ T cells expressing high levels of CD80, CD86 and CD134 compared to healthy controls (7.1 +/- 1.5% versus 1.7 +/- 0.9%; P < 0.005; 2.3 +/- 0.4% versus 1.0 +/- 0.2%; P = 0.008, 20.2 +/- 2.0% versus 10.6 +/- 1.9%; P < 0.005, respectively). Significantly higher levels of CD80 on CD4+ T cells were detected in SLE patients with lupus nephritis compared to patients without nephritis (11.9 +/- 3.3% versus 4.0 +/- 0.7%; P < 0.005). There was an increased presence of CD134+ CD4+ cells in SLE patients with lupus nephritis (27.5 +/- 4.0% versus 15.5 +/- 1.3%; P < 0.005). CD80 and CD134 expression was significantly correlated with SLEDAI (r = 0.42, P = 0.03; r = 0.56, P < 0.005). Co-stimulatory molecules on CD4+ T cells are associated with renal disease and disease activity in patients with systemic lupus erythematosus.

Acute Disease↗
PubMedDate not supplied

The pathogenesis of systemic lupus erythematosus.

Systemic lupus erythematosus is an autoimmune disease that causes inflammation in the tissues of the brain, endothelial cells, gastrointestinal/genitourinary (GI/GU), joints, kidneys, muscles, and skin. Lupus comprises a range of multisystem disorders involving the deposition of aberrant immune complexes into tissues. Inflammation occurs as a result of autoantibodies attacking organ systems. The pathogenesis of systemic lupus erythematosus is presented.

Antigen-Antibody Complex↗
PubMed2001

Genetic epidemiology: systemic lupus erythematosus.

Systemic lupus erythematosus is the prototype multisystem autoimmune disease. A strong genetic component of susceptibility to the disease is well established. Studies of murine models of systemic lupus erythematosus have shown complex genetic interactions that influence both susceptibility and phenotypic expression. These models strongly suggest that several defects in similar pathways, e.g. clearance of immune complexes and/or apoptotic cell debris, can all result in disease expression. Studies in humans have found linkage to several overlapping regions on chromosome 1q, although the precise susceptibility gene or genes in these regions have yet to be identified. Recent studies of candidate genes, including Fcgamma receptors, IL-6, and tumour necrosis factor-alpha, suggest that in human disease, genetic factors do play a role in disease susceptibility and clinical phenotype. The precise gene or genes involved and the strength of their influence do, however, appear to differ considerably in different populations.

Animals↗
PubMed1990

[Central nervous symptoms and findings in patients with systemic lupus erythematosus].

Systemic lupus erythematosus is a disease characterized by multiple autoimmune phenomena, and a broad clinical spectrum. Involvement of the central nervous system is common, and in the majority of patients occurs mainly as an organic brain syndrome or as migraineous headache. Cerebral atrophy as judged by CT scan is common. Cerebral infarction occurs in a minority of patients, mainly those with high disease activity and a high titer of anti-phospholipid antibodies. Systemic lupus erythematosus should be considered as a possible diagnosis in patients with certain central nervous system aberrations, especially young females.

Adult↗
PubMed2004

Global cerebral edema and subarachnoid hemorrhage in a patient with systemic lupus erythematosus.

Systemic lupus erythematosus is a multifactorial autoimmune disease of complex etiology, which may be associated with cognitive dysfunction, seizures, and headache. The authors present an unusual presentation of systemic lupus erythematosus complicated by global cerebral edema and subarachnoid hemorrhage secondary to rupture of a cerebral aneurysm. The complicated patient management issues are discussed.

Adolescent↗
PubMed1992

Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features.

Sixty-seven patients with cutaneous lupus erythematosus (CLE) were followed up as part of a series of 570 lupus erythematosus patients seen in a private practice between 1980 and 1989. Clinical and laboratory features, treatment, and natural course were observed. Findings of interest included (1) a ratio of at least one CLE case for every seven cases of systemic lupus erythematosus (SLE); (2) occurrence of CLE in fewer women and apparently associated with an older age at diagnosis than SLE; (3) similar frequency of cutaneous lupus subsets in CLE and SLE; (4) strong family history for SLE but not CLE in CLE patients; (5) other cutaneous and musculoskeletal features in a majority of CLE patients and constitutional symptoms in 10%; (6) positive ANA titers, high sedimentation rates, and leukopenia common in CLE; (7) anticardiolipin antibody in 31% of CLE patients but not associated with systemic complications; (8) antimalarials required by 75% of patients and systemic steroids by 33%; and (9) an excellent prognosis associated with CLE, organ-threatening disease being rare.

Adult↗
PubMed1969

Systemic lupus erythematosus.

Systemic lupus erythematosus is a polysystemic disease with a high incidence of associated glomerulonephritis. Patients with sle rarely have the destructive arthritis so characteristic of rheumatoid arthritis. An unusual case is presented in which both glomerulonephritis and destructive arthritis occurred simultaneously, justifying the diagnosis of both systemic lupus erythematosus and rheumatoid arthritis.Immunohistochemical studies in lupus glomerulonephritis suggest that the pathogenetic mechanisms involve the deposition of immune complexes containing "nuclear" antigens and antinuclear antibodies in the lesions. The detection of mixed cryoglobulins in the sera of patients with sle suggests that a portion of the circulating immune complexes may precipitate at reduced temperatures and be detected as mixed cryoglobulins. The therapy of lupus glomerulonephritis with combinations of corticosteroids and azathioprine, though still in an investigative state, holds great promise. Similar abnormalities in diseases of minks and mice and in sle suggest similar pathogenetic mechanisms in the three species involved. Since the diseases in the lower animals have been associated with persistent viral infection, the investigation of the role of persistent infection in sle seems warranted.

Adolescent↗