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PubMedDate not supplied

Magnetic resonance imaging (MRI) of the brain in SLE: ECLAM and SLEDAI correlations.

Twenty-one unselected SLE patients were examined with Magnetic Resonance Imaging (MRI) for cerebral areas of high signal intensity (HSI). Nine patients (42.9%) had HSI and 6 (28.6%) had large (> or = 6 mm) lesions suggestive of cerebral involvement. Despite the lack of correlation among these lesions and clinical or immunological features, the SLEDAI and ECLAM indices were correlated to the number of large lesions (p < 0.002). Our findings suggest that brain MRI may be useful in the evaluation of SLE patients, not only those with evident neurologic symptoms, but also asymptomatic subjects with high disease activity indices.

Adolescent↗

PubMed1993

Induction of an anti-Fab, anti-DNA and anti-RNA polymerase I autoantibody response network in rabbits immunized with SLE anti-DNA antibody.

Systemic lupus erythematosus (SLE)-like anti-IgG Fab autoantibodies (autoAb) were induced in rabbits by immunization with either human, mouse or rabbit anti-DNA Ab. In direct-binding radioimmunoassay (RIA), affinity-purified anti-normal rabbit (NR) Fab autoAb cross-reacted with normal mouse (NM) Fab, ssDNA (but not dsDNA), poly(dA,dT), and RNA polymerase I (RPI). Affinity-purified anti-NM IgG Ab isolated from the same antisera cross-reacted with NR Fab, ssDNA and RPI. In inhibition RIA, soluble NR Fab inhibited anti-NR Fab binding to NR Fab and ssDNA, but enhanced binding to RPI. In contrast, ssDNA or RPI inhibitors had no effect upon autoAb binding to NR Fab. Anti-DNA, anti-RPI and anti-RPI 190 kD subunit autoAb, induced by immunization with lupus mouse anti-DNA Ab, also reacted with NM Fab, but were idiotypically specific for lupus mouse anti-DNA Fab. Further, rabbit anti-DNA and anti-RPI IgG autoAb, induced by immunization with rabbit anti-DNA IgG, were each idiotypically specific for homologous and autologous rabbit anti-ssDNA Fab. Together, these data provide evidence that anti-DNA, anti-RPI and anti-Fab autoAb are linked in a complex, multiple-specific and perhaps regulatory, immune response idiotype network in SLE.

Animals↗

PubMedDate not supplied

Thrombosis and thrombocytopenia in antiphospholipid syndrome (idiopathic and secondary to SLE): first report from the Italian Registry. Italian Registry of Antiphospholipid Antibodies (IR-APA).

BACKGROUND: Since 1989 we have been promoting an Italian Registry of patients with APA (IR-APA), and we report here the analysis on 319 cases (80 males and 239 females, aged from 2 to 76 years, median 31 years) from 22 institutions. METHODS: A two-page questionnaire was prepared, divided into 4 parts: 1. personal data, 2. associated conditions, 3. biology (for the diagnosis of APA), 4. history of thromboembolism, haemorrhage and pregnancy. Every information was entered into a computerized registry. RESULTS: The IR-APA included 207 cases of idiopathic APA and 112 cases of APA secondary to SLE. Seventy-one out 207 (34.3%) and 47 out of 112 (42%) cases experienced arterial and/or venous thrombosis, respectively (not significant). Among patients with a thrombotic history, venous thrombosis were experienced by 60.5% vs 57.4%, arterial thrombosis by 26.7% vs 31.9%. Venous thrombosis of the legs and arterial thrombosis of the cerebral vessels were the most common types of thrombosis in both groups. Thrombocytopenia was present in 27.7% of idiopathic APA vs 22.4% of secondary APA (not significant). Four patients experienced major haemorrhagic events. CONCLUSIONS: This retrospective study shows that the prevalence of thrombosis and thrombocytopenia in patients with idiopathic APA is similar to that observed in patients with APA secondary to SLE.

Adolescent↗

PubMed1993

[Two cases of RA-like and SLE-like features similar to remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome)].

In 1985, McCarty et al reported 10 patients with a symmetrical synovitis affecting predominately the wrists and flexor digitorum tendon sheaths associated with marked pitting edema of the dorsum of both hands and both feet. It was insisted on the clinical entity as remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome). These patients were mostly elderly men whose sera revealed negative rheumatoid factor and had a benign clinical course. Patients with RS3PE syndrome remitted completely within 3-36 months and the remission was maintained even after all medications were discontinued. We experienced 2 interesting cases which were similar to RS3PE syndrome. One case with SLE-like conditions evolved into RA-like conditions. On the contrary, the other which had been effectively treated as RA developed into SLE-like conditions. Both cases were seronegative and had the characteristic pitting edema of both hands and feet demonstrating the symmetrical synovitis without bony erosions. They went into complete remission by corticosteroid therapy, although it did not continue for a long time. We should consider that such cases are similar to RS3PE syndrome and must be distinct from it.

Aged↗

PubMed1993

[Systemic lupus erythematosus (SLE) today].

SLE is a complex systemic disease which probably has a multifactorial genesis. The basis alteration takes the form of a severe immunoregulation disorders. From an epidemiological point of view, there are no certain links with country of origin or race; knowledge of the disease's etiology is equally limited. Clinical symptoms are widely variable according to the prevalent organ or apparatus pathology. The symptomatology of SLE is in fact highly polymorphous thus making it complex and sometimes difficult to diagnose. From a prognostic point of view, over recent years the future of these patients has appeared brighter than in the past. The current therapeutic approach uses a number of measures which can be used in combination or sequentially. It is precisely this varying combination of therapies that has led to a marked prolongation of survival and long disease-free periods.

Humans↗

PubMed1995

Age-related differences on clinical and immunological manifestations of SLE.

The clinical and immunological manifestations of 51 children with onset of systemic lupus erythematosus (SLE) before the age of 15 were compared with those of 308 adult patients with disease onset between the age of 15-49 and another 27 elderly lupus patients whose disease onset occurred at or after the age of 50. Overall disease activity determined by mean SLEDAI score was highest in the childhood group followed by the adult and the elderly group respectively. More severe form of cutaneous involvement, adenopathy, hypertension, renal involvement with renal insufficiency and anti-nDNA antibodies occurred predominantly in the childhood lupus. The clinical features distinguishing old-age lupus were chronic disease with a long interval between the time of onset and diagnosis, higher incidence of discoid rash and lower incidence of malar rash and renal involvement. Frequencies of anti-nDNA antibodies and renal involvement gradually decreased from childhood, to adulthood and to elderly lupus respectively. Anti-Sm antibodies were predominant in the adult onset group. Genetic markers, sex hormones and senility of the immune system may play a role in these age-related differences in clinical and immunological manifestations in SLE.

Adolescent↗

PubMed1996

[Analysis of immunoglobulin classes of five anti-phospholipid antibodies in sera from patients with SLE, ITP and recurrent fetal abortion by enzyme linked immunosorbent assay].

Using a panel of phospholipid(PL) antigens, we have established an enzyme linked immunosorbent assay (ELISA) for measuring both IgG and IgM type anti-phospholipid antibodies (APA) in sera from patients with systemic lupus erythematosus (SLE), idiopathic thrombocytopenic purpura (ITP) and recurrent fetal abortion (RFA). The percentage of anticardiolipin antibody (aCL) positive patients was increased in SLE (73% for IgG, 74% for IgM), ITP (24% for IgG, 8% for IgM) and RFA (20% for IgG, 52% for IgM) as compared with normal controls. The percentage of other APAs in each disease was significantly different from one another, suggesting the existence of disease-specific APA in these autoimmune disorders. We consider that it is important to analyze these APAs for the investigation of pathogenesis of autoimmune disorders.

Abortion, Habitual↗

PubMed1996

What's new in the treatment of pediatric SLE.

Therapy in pediatric systemic lupus erythematosus (SLE) is reviewed by examining the pediatric experience, including uncontrolled retrospective observations and case reports. In addition, I extrapolate from the adult experience and interpret findings in light of pediatric SLE.

Anticoagulants↗

PubMed1996

[Neuro-SLE. Models of its clinical expression].

In this paper we considered different models concerning the clinical expression of neuropsychiatric involvement in course of systemic lupus erythematosus (SLE). These models describe pathological conditions as multifocal cerebropathy, transverse myelitis, peripheral neuropathy and panic attacks. We have chosen these cases as clinical example of different pathogenic mechanisms responsible of CNS-lupus, as hypercoagulation due to antiphospholipid syndrome, immune-complex vasculitis, complement-mediated autoantibody damage and antibody-induced cytotoxicity. The prevalence of neuropsychiatric manifestations in 122 SLE patients is also reported. Finally, the paper reports some guidelines about diagnostic and therapeutic behaviour in course of CNS-lupus.

Adolescent↗

PubMedDate not supplied

International survey on the management of patients with SLE. I. General data on the participating centers and the results of a questionnaire regarding mucocutaneous involvement.

OBJECTIVE: The diagnosis and treatment of the mucocutaneous (MC), neuropsychiatric (NP), and renal (RN) manifestations of systemic lupus erythematosus (SLE) remain unsolved issues. To shed light on these issues, a questionnaire was prepared and sent to 153 lupus centres around the world, in order to determine the level of agreement between experts in their approach to these complex aspects of the disease. METHODS: The first section of the questionnaire was designed to collect information on the characteristics of the responding lupus centres. The second section was dedicated to MC manifestations, with questions focusing on: (i) the frequency of MC manifestations as a whole and of the single clinical MC entities; (ii) clinical features, outcome and therapy of subacute cutaneous lupus erythematosus (SCLE); (iii) the utility of the lupus band test (LBT); and (iv) the use of various therapeutic protocols to treat MC manifestations. RESULTS: Sixty-one questionnaires from 19 countries were analysed. Out of these, 37 were completed by Departments of Rheumatology, 21 by Departments of Internal Medicine or Clinical Immunology, and 3 by Departments of Nephrology. About 66% of these centres stated that they were currently following more than 100 lupus cases, 95% had an in-patient ward and 82% had their own laboratory. The American College of Rheumatology classification criteria and various scales for disease activity assessment were regularly used by 87% and 57% of centres respectively. The overall prevalence of MC manifestations was judged to be over 30% by 82% of the respondents (Rs), and over 60% by 36% of the Rs. Among the different MC manifestations, malar rash was reported to be the most frequent (40%), followed by alopecia (24.1%) and oral ulcers (18.6%). In reporting the prevalence of each MC manifestation, the Rs showed a low level of agreement, the coefficient of variation (CV) being > 0.75 for all of the manifestations listed with the exception of malar rash (CV = 0.54). Poor agreement among centers was also found for the reported association of various MC manifestations with SCLE (15 different answers), and on the prognostic factors for SCLE (17 different answers). There was agreement on the best procedure (up to 70% of the Rs preferred a non-UV exposed skin area) and on the utility of the LBT (83% using it only for diagnostic purpose). Hydroxychloroquine was the most popular therapeutic protocol, being used by 85% of the Rs for a wide variety of MC manifestations. Among other therapies, azathioprine was used by 59%, dapsone by 41%, and thalidomide by 35% of the Rs, all to treat a wide spectrum of MC manifestations. Pulse steroid, cyclosporin A and pulse cyclophosphamide were less commonly employed (by 27%, 22% and 13% of the Rs, respectively), and were reserved for the most severe MC manifestations, particularly vasculitis. CONCLUSION: The present survey indicates that, although most of the participating centres had extensive experience in the management of SLE, their approach to the MC manifestations was not homogeneous, and collaborative studies are clearly needed, particularly to optimise the therapeutic protocols.

Alopecia↗

PubMedDate not supplied

Clinical, autoimmune and demographic profile in systemic lupus erythematosus (SLE) patients from southern Brazil.

With the purpose of determining the association of clinical, autoimmune and demographic features, a group of 90 SLE patients from Southern Brazil were investigated. At diagnosis, 24% of them were under 20 years, 63% were between 20 and 40 years and 13% were older than 40 years. According to the ethnic background, there were 66% Brazilian-white patients, 21% Caucasians and 13% Mullatos/Blacks. Antinuclear antibodies (ANA) were present in 98%, anti-ds-DNA in 56% and anti-Sm in 31% of the patients. Anti-ds-DNA were more prevalent in the Caucasians (79%), while anti-Sm were increased in the Mullatos/Blacks (58%, p < 0.02) as compared to the white patients (Brazilian-whites = 22% and Caucasians = 42%). Neurologic involvement had lower prevalence in the group of Mullato/Black patients (8%) than in the Brazilian-whites (32%) and Caucasians (31%). Serositis was present in 51% of the Brazilian-whites, in 21% of the Caucasians and in 41% of the Mullatos/Blacks. On the other hand, the Mullato/ Black group had an increased prevalence of vasculitis (50%) and none of them presented with Raynaud's phenomenon. Younger patients at diagnosis presented higher frequency of renal involvement (p < 0.05), anti-ds-DNA positivity (p < 0.02) and more severe disease (p < 0.07), and in those patients diagnosed after age 40, 33% presented with Raynaud's phenomenon (p < 0.05). Regarding the anti-ds-DNA positivity, 78% of the patients had renal involvement (p < 0.01 RR 2.2) and 66% severe disease (p < 0.05). These results might be important in assessing clinical subsets and may aid individualized management of Brazilian SLE patients. Also, they may corroborate the need for special attention to racial composition in clinical and immunogenetic studies.

Adolescent↗

PubMed1997

[A case report of typical scleroderma accompanied with serum abnormalities characteristic of SLE during the course].

A 40-year-old woman had complained of cyanosis induced by cold exposure from the age of 26. When she was 32 years old, Raynaud's phenomenon occurred. She developed diffuse cutaneous sclerosis affecting the upper limbs, face and trunk, digital pitting scar, flexion contractures of hands, dilatation of lower esophagus and pulmonary fibrosis, and she was diagnosed as scleroderma. Laboratory findings revealed positive anti-topoisomerase I antibody and hypergammaglobulinemia (IgG 2,782, IgA 632, IgM 146 mg/dl). However, serum complement levels were normal and anti-DNA antibodies measured by radioimmunoassay (RIA) were negative. Initial dose of oral prednisolone was 30 mg/day and afterwards 5 mg/day of prednisolone was maintained. At the age of 36, scleroderma and contraction of hands were progressed, and telangiectasias appeared on her chest at the age of 36. Laboratory tests revealed hypocomplementemia (C3 27, C4 9 mg/dl, CH50 16 U/ml) and high titers, more than 100 U/ml, of anti-DNA antibodies measured by RIA. Clinical evidence suggestive of SLE could not be found. Reexamination of previous sera by enzyme immunoassay, in which anti-DNA antibody could not be detected by RIA, clarified the presence of IgG anti-dsDNA antibodies. It was considered that there existed low avidity/affinity of anti-dsDNA antibodies at first, and afterwards high avidity/affinity of anti-dsDNA antibodies appeared. Increasing of oral prednisolone up to 30 mg/day normalized serum complements and decreased titers of anti-DNA antibodies. She had not developed any clinical evidence that suspected SLE throughout the course.

Adult↗

PubMed1979

Purification of antilymphocyte antibody (ALA) from patients with systemic lupus erythematosus (SLE)-immunoabsorption and elution.

Purification of antilymphocyte antibody (ALA) from patients with systemic lupus erythematosus (SLE) was achieved by immunoabsorption and elution. Human tonsil cells or thymocytes were used as absorbents. Complement dependent microcytotoxicity tests showed that, in comparison to the parent sera, the eluate from tonsil cells was eight times, and that from thymocytes four times, more active. Antinuclear activity was eliminated by elution. The ALA was almost entirely IgM, IgG being involved in only a few cases. IgA lacked cytotoxic activity. ALA was directed at both T- and B-cell surface determinants, which suggests that, in SLE, it has a heterogeneous biological composition.

Adult↗

PubMed1997

ANA-negative SLE presenting with nephritis and oculomotor palsy--a case report.

Most large series of patients with systemic lupus erythematosus (SLE) have revealed a very small group of patients who are persistently antinuclear antibody (ANA) negative with prominent dermatologic involvement but infrequent neurological and renal involvement. We present a very unusual case report of a young female with ANA-negative SLE presenting with lupus nephritis and oculomotor palsy without any dermatologic manifestations.

Adolescent↗

PubMedDate not supplied

HIV infection and SLE: their pathogenic relationship.

Retroviruses have repeatedly been suggested as a possible trigger mechanism for systemic lupus erythematosus (SLE). We review the role of human immunodeficiency virus (HIV)-1 envelope glycoprotein (gp120) in the induction of immune dysregulation including autoimmunity in HIV-1 infection, and discuss the possible relationship between HIV and SLE in the retroviral etiology.

Acquired Immunodeficiency Syndrome↗

PubMed1998

Renal pathology and long-term outcome in childhood SLE.

Renal histology is increasingly used as a guide for therapy and prognosis in SLE but data in children are few and/or short-term. We assessed renal histological features in 19 children with SLE to determine whether these features are useful in predicting long-term outcome. Mean age at biopsy was 10 +/- 1.7 years old, male to female ratio was 1:2.8. Fourteen patients (73%) had diffuse proliferative lupus nephritis. Renal histology was evaluated using an activity index (AI) and chronicity index (CI). Clinical assessment of renal function at biopsy and outcome were graded according to urinalysis and serum creatinine. Renal function at biopsy correlated well with AI (p < 0.001) but not CI. At short-term follow-up (30 months), 3 patients had died from sepsis and another 2 reached end-stage renal disease. CI predicted poor clinical outcome, i.e. death or renal failure (p < 0.005) but AI did not. At long-term follow-up (mean 92.1 +/- 26.8 months) only one more patient reached end-stage renal disease. In others renal function assessment showed improvement or were stable. Neither CI nor AI correlated with clinical outcome. We conclude that although AI correlates well with renal function at biopsy and CI with short-term prognosis, neither can predict long-term outcome. Treatment may have altered the natural course of disease in these patients.

Biopsy↗

PubMed1998

[SLE with interstitial pneumonia during cyclophosphamide pulse therapy].

A 49 year-old man was admitted for edema and renal impairment due to SLE. Since he did not improve with predonisolone and methylprednisolone pulse therapy, cyclophosphamide pulse therapy (300 mg div.) was administered. The patient subsequently developed a fever, dyspnea and cough, and interstitial regions of the lungs exhibited shadows on X-ray and CT. The patient also suffered hypoxemia and poor lung function. Since several culture tests and viral antibody tests were negative for infection, antibiotics were not effective, and TBLB indicated interstitial pneumonia, which we speculated was induced by cyclophosphamide. However, this was such a severe case of interstitial pneumonia that it could not be cured merely by discontinuing the cyclophosphamide, but it did improve immediately after starting methylprednisolone pulse therapy. The incidence of cyclophosphamide-induced interstitial pneumonia is very low, but the mortality rate is high. Since cyclophosphamide pulse therapy is often used to treat SLE, attention should be focused on the incidence of interstitial pneumonia.

Cyclophosphamide↗

PubMed2026

SLE-Associated rs2295613(A) Allele Strengthens a Predicted c-MYC Motif and Enhances SLAMF1 Promoter Reporter Activity in B Cells.

SLAMF1 encodes CD150, an immunoregulatory receptor involved in lymphocyte activation, T-B-cell interactions, and humoral immune responses. The SLAMF1 promoter polymorphism rs2295613(G>A) was previously associated with systemic lupus erythematosus (SLE) susceptibility in a Chinese case-control cohort. Here, we investigated the regulatory activity of rs2295613 in the transformed B-cell lines Raji and MP1 and in primary human CD19+ B cells. The rs2295613(A)-containing reporter showed higher promoter activity than the rs2295613(G)-containing reporter in all three cellular systems. Bioinformatic analysis predicted that the G to A substitution strengthens a pre-existing MYC-compatible motif. Substitutions disrupting the motif-containing region attenuated the rs2295613(A)-associated increase in reporter activity and reduced enrichment of the promoter fragment in anti-c-MYC DNA pull-down assays. Partial siRNA-mediated reduction in MYC mRNA also decreased the activity of the rs2295613(A)-containing reporter in Raji cells. Together, these findings identify rs2295613 as a functional SLAMF1 promoter variant in B-cell reporter systems and support a contribution of c-MYC-associated regulation to the enhanced activity of the rs2295613(A)-containing promoter.

Humans↗