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Systemic lupus erythematosus after renal transplantation: patient and graft survival and disease activity. The Dutch Working Party on Systemic Lupus Erythematosus.

OBJECTIVE: To determine the outcome of renal transplantation in patients with systemic lupus erythematosus and end-stage renal failure and to compare disease activity after transplantation with disease activity before transplantation. DESIGN: Retrospective case finding using data for an 8-year period from the central registry for renal replacement therapy in The Netherlands. SETTING: Tertiary care hospitals with facilities for renal transplantation in the Netherlands. PATIENTS: Twenty-eight patients who fulfilled at least four of the American Rheumatology Association's criteria for the classification of systemic lupus erythematosus and who received a renal transplant. MEASUREMENTS: Actuarial survival rates for grafts and patients after transplantation, maximal nonrenal scores on the Systemic Lupus Erythematosus Disease Activity Index, and time-adjusted disease exacerbation rates in all patients before and after transplantation. RESULTS: The actuarial graft survival rate at 1 year and 5 years was 68% (95% CI, 47% to 82%) and 54% (CI, 25% to 77%), respectively, whereas the actuarial patient survival rate was 87% (CI, 69% to 96%) at 1 and 5 years. High disease activity was not found to affect graft survival adversely before the start of renal replacement therapy or during dialysis. After transplantation, disease activity per patient and the overall incidence of disease exacerbations decreased. One case of recurrent lupus nephritis was seen. CONCLUSIONS: Patients with systemic lupus erythematosus and end-stage renal failure are excellent candidates for renal transplantation; disease activity after transplantation is sporadic and low, and the recurrence of lupus nephritis is rare.

Actuarial Analysis↗

[Two cases of lupus cystitis complicated by lupus nephritis treated successfully with steroid therapy].

In patients with systemic lupus erythematosus(SLE), interstitial cystitis(lupus cystitis) is an uncommon, but important manifestation. We report two Japanese patients with lupus cystitis. Case 1 was a 49-year-old woman diagnosed as having rheumatoid arthritis and membranous nephropathy. She was treated with prednisolone(5 mg daily). Case 2 was a 41-year-old woman also diagnosed as having rheumatoid arthritis previously and treated with a non-steroidal anti-inflammatory drug. Both cases presented abdominal pain, vomiting, dysuria and frequency of micturition. We diagnosed these cases as SLE on the basis of arthritis, renal disorder(proteinuria and hematuria), and positive antinuclear and anti-dsDNA antibodies. In addition, bilateral hydronephrosis was found in both cases. Thus, they were also diagnosed as probable lupus cystitis. The patients were treated with one cycle of methylprednisolone pulse therapy. Thereafter they were treated with 60 mg/day of prednisolone and their symptoms resolved promptly. Furthermore, no abnormal finding was found by abdominal ultrasonography and/or the intravenous pyelogram after therapy. Renal biopsies were performed and both cases showed lupus glomerulopathy (case 1: WHO class Vb, case II: WHO class IVb). Abdominal pain and/or dysuria, which is common in SLE patients, requires further examinations to evaluate the lupus cystitis.

Adult↗

Defining response in systemic lupus erythematosus: a study by the Systemic Lupus International Collaborating Clinics group.

OBJECTIVE: In a preliminary attempt to develop a drug responder index for patients with systemic lupus erythematosus (SLE), 2 validated disease activity instruments were studied for their responsiveness and compared to a physician visual analog scale (VAS) assessment of disease activity. We attempted to determine whether these validated instruments were useful components in characterizing response in the setting of a clinical trial. METHODS: Eighty paper patients were assessed using the British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Disease Activity Index (SLEDAI) and by physician's assessment of global activity. The cases were arranged in random order and divided into groups of 20 patients and each group was assessed by 20 lupus experts; change in disease activity was recorded at 3 and 6 months compared to baseline using a physician VAS. RESULTS: Four different lupus experts assessed disease activity in all 80 patients at baseline and 3 and 6 months after initiation of therapy using the BILAG and SLEDAI instruments. BILAG and SLEDAI scores correlated well over time; however, in a regression analysis where average physician VAS were chosen as the outcome variable, a significant amount of variation in the average physician VAS not related to the SLEDAI and BILAG scores was noted. CONCLUSION: The physician VAS may be too blunt to assess response in SLE, because even among experienced lupus assessors, there were considerable differences in what influenced scoring decisions.

Adult↗

[Chronic lupus erythematosus in lupus disease. Retrospective study of 136 patients].

OBJECTIVES: The aim of this retrospective study of 136 patients was to specify the natural history and the systemic prognosis of chronic cutaneous lupus erythematosus. It has been stated that in most of the cases, the disease only affects the skin. METHODS: From 10 October 1980 to 31 December 1990, 136 patients with the following criteria were included in this retrospective study: clinical signs suggestive of chronic cutaneous lupus erythematosus, characteristic histology, insufficient evidence for the diagnosis of systemic lupus erythematosus. RESULTS: The prevalence of systemic clinical involvement in the population under study was nearly the same as in the general population. The following biologic or immunologic abnormalities were quite common: leukopenia, lymphopenia, thrombopenia (significantly more frequent among patients having widespread chronic cutaneous lupus erythematosus), low titers of complement levels, positive antinuclear antibodies (usually at a low titer). Eleven out of the 136 patients developed systemic lupus erythematosus, most often over 5 years after the onset of the cutaneous lesions. Four cases out of these 11 had poor prognosis: renal and/or neurologic involvement. CONCLUSION: This data suggests that patients with chronic cutaneous lupus erythematosus could benefit from long-term follow up, since the course to systemic disease occurs in only a few. Usually, antimalarials used singly or in combination with topical steroids may lead to the clearing of the lesions. Thalidomide will occasionally be useful whenever the disease is unresponsive to the preceding measures.

Adult↗

Autoantibody specificity in drug-induced lupus and neutrophil-mediated metabolism of lupus-inducing drugs.

A long-term side effect of therapy with a variety of drugs is a syndrome resembling the idiopathic autoimmune disease, systemic lupus erythematosus. Essentially all patients with drug-induced lupus display autoantibodies to nuclear histone components whose specificity appears to be related to the higher order structure of histones existing in chromatin. IgG antibodies to H1 and the (H2A-H2B)-DNA complex were observed in most patients with lupus induced by procainamide, hydralazine, and quinidine, whereas the H3-H4 tetramer, comprising half the mass of the nucleosome core particle, was largely nonantigenic. IgM antibodies to (H2A-H2B)-containing chromatin subunits were common also. IgM reactivity was observed with the DNA-free H3-H4 tetramer and with H1, especially in hydralazine-induced lupus. These results suggest that IgM antihistone antibodies may result from autoimmunization with a nonnative form of chromatin, whereas IgG antibodies may be selected for reactivity with H1 and a native form of the (H2A-H2B)-DNA subunit of the nucleosome. The chemical basis for induction of autoimmunity by drugs is unclear because lupus-inducing drugs do not have a common structural feature or biological activity nor are they capable of specific reactions with histones, the principal target antigen. However, in the presence of activated neutrophils, procainamide is transformed metabolically to the cytotoxic procainamide-hydroxylamine. Mixing experiments and cell-free studies demonstrated that procainamide was cooxidized with H2O2 by myeloperoxidase released when neutrophils undergo the respiratory burst and degranulation reactions. Preliminary results indicate other lupus-inducing drugs are also biotransformed by this mechanism suggesting that a common denominator linking these drugs may be the capacity to be oxidized to reactive metabolites by the action of activated phagocytic cells.

Antibody Specificity↗

The atherogenic effect of lupus sera: systemic lupus erythematosus-derived immune complexes stimulate the accumulation of cholesterol in cultured smooth muscle cells from human aorta.

The influence of systemic lupus erythematosus (SLE) patients' sera on lipid accumulation in the cultured smooth muscle cells (SMC) from unaffected human aortic intima was examined. It was demonstrated that the cholesterol uptake in the SMC cultured in the presence of SLE sera is 1.5- to 6-fold higher than in the cells cultured with normal human sera (NHS) obtained from healthy donors. Incubation of the SMC with circulating immune complexes (CIC) isolated from lupus sera by precipitation with 2.5% polyethylene glycol 6000 (PEG) caused a 3- to 4-fold rise in the intracellular cholesterol level. The atherogenic effect of lupus sera, as well as isolated CIC, strongly correlated (r = 0.98) with the low density lipoprotein (LDL) content in the PEG-precipitated CIC. The cholesterol level in cultured SMC also increased 2- to 3-fold when growth medium was supplemented with LDL, DNA, and anti-DNA autoantibodies (IgG) affinity isolated from lupus sera. Using immunofluorescent staining, it was shown that the addition of a DNA-anti-DNA IgG mixture to the growth medium, together with NHS, stimulated LDL incorporation in the SMC. The results of double-label staining suggest the formation of LDL-DNA-IgG complexes which seem to be entrapped in cells more actively than free LDL. The composition of PEG-precipitated CIC was studied by electrophoresis and immunoblotting. Significant amounts of apolipoprotein B, as well as low molecular weight DNA and immunoglobulins, were found in SLE-derived CIC. The data obtained suggest that the atherogenic effect of human lupus sera in vitro is generally due to the appearance of LDL-containing immune complexes. Different mechanisms possibly involved in the lupus atherogenesis are discussed.

Adult↗

Susceptibility of lupus-prone NZM mouse strains to lead exacerbation of systemic lupus erythematosus symptoms.

It has been repeatedly shown that the heavy metal mercury can induce or exacerbate lupus like autoimmunity in susceptible strains of rats and mice. A hallmark of such autoimmune induction is the accompaniment of an immune shift, in which there is usually an initial skewing toward a Th2-like immune environment. Another heavy metal, lead (Pb), has also been found to induce a Th2 shift in mice. However, exposure of normal mouse strains to Pb does not appear to induce autoimmunity. In order to investigate whether mice genetically predisposed to murine systemic lupus erythematosus (SLE) are susceptible to a Pb-induced exacerbation of lupus, males and females of four New Zealand mixed (NZM) mouse strains, along with BALB/c and C57Bl/6 controls, were administered three 100-microliter intraperitoneal injections of either 1.31 mM lead or sodium acetate per week for 3 wk. The four NZM strains chosen, NZM391, NZM2328, NZM88, and NZM2758, have differential genetic penetrance for SLE with variances in certain manifestations of the disease, but all of these strains naturally develop glomerulonephritis and produce high titers of anti-nuclear autoantibodies. The mice were prebled for baseline values and were bled directly after the injection period (d 1) and monthly thereafter for 5 mo. Sera were assessed for anti-double-stranded DNA titers, urea nitrogen levels, and creatine kinase activity, as well as four total immunoglobulin (Ig) G2a and IgG1 levels. Mortality and morbidity of the mice were also recorded. All NZM strains showed an acute, non-gender-based, susceptibility to Pb at d 1, but the control strains were unaffected. Over time, it became apparent that the strains diverged: The NZM391 strain showed gender-independent susceptibility to Pb enhancement of lupus manifestations and mortality; the NZM2328 strain exhibited gender-independent Pb susceptibility to manifestations, although only females had increased mortality; the NZM2758 strain exhibited non-gender-based elevations in urea nitrogen and creatine kinase activity levels; and the NZM88 strain displayed male susceptibility to anti-DNA and life span. Surprisingly, Pb increased the longevity of NZM88 and NZM2758 females. These results indicate that Pb indeed can exacerbate SLE in lupus-prone mice; however, even among lupus-prone strains, genetic differences determine the degree of exacerbation. Using the known phenotype and genetic differences, one can identify and characterize possible traits and loci associated with Pb susceptibility.

Analysis of Variance↗

Familial chilblain lupus, a monogenic form of cutaneous lupus erythematosus, maps to chromosome 3p.

Systemic lupus erythematosus is a prototypic autoimmune disease. Apart from rare monogenic deficiencies of complement factors, where lupuslike disease may occur in association with other autoimmune diseases or high susceptibility to bacterial infections, its etiology is multifactorial in nature. Cutaneous findings are a hallmark of the disease and manifest either alone or in association with internal-organ disease. We describe a novel genodermatosis characterized by painful bluish-red inflammatory papular or nodular lesions in acral locations such as fingers, toes, nose, cheeks, and ears. The lesions sometimes appear plaquelike and tend to ulcerate. Manifestation usually begins in early childhood and is precipitated by cold and wet exposure. Apart from arthralgias, there is no evidence for internal-organ disease or an increased susceptibility to infection. Histological findings include a deep inflammatory infiltrate with perivascular distribution and granular deposits of immunoglobulins and complement along the basement membrane. Some affected individuals show antinuclear antibodies or immune complex formation, whereas cryoglobulins or cold agglutinins are absent. Thus, the findings are consistent with chilblain lupus, a rare form of cutaneous lupus erythematosus. Investigation of a large German kindred with 18 affected members suggests a highly penetrant trait with autosomal dominant inheritance. By single-nucleotide-polymorphism-based genomewide linkage analysis, the locus was mapped to chromosome 3p. Haplotype analysis defined the locus to a 13.8-cM interval with a LOD score of 5.04. This is the first description of a monogenic form of cutaneous lupus erythematosus. Identification of the gene responsible for familial chilblain lupus may shed light on the pathogenesis of common forms of connective-tissue disease such as systemic lupus erythematosus.

Adolescent↗

The evolution of lupus activity among patients with end-stage renal disease secondary to lupus nephritis.

The aim of this study was to evaluate the evolution of lupus activity in end-stage renal disease (ESRD) patients due to lupus nephritis and to determine the long-term prognosis. We reviewed the clinical courses of 45 patients with ESRD due to systemic lupus erythematosus (SLE). We analyzed the course of SLE following the onset of ESRD, with special attention to the clinical and serological manifestations, survival time on dialysis, and renal transplantation outcome. Disease activity was measured using the SLE Disease Activity Index (SLEDAI). Of the 45 patients, 21 patients were being treated with hemodialysis (HD), 11 were undergoing peritoneal dialysis (PD), and 13 underwent transplantation. Duration of follow- up was 53 +/- 29 months. The SLEDAI score on commencement of renal replacement therapy was not significantly different among the 3 groups (HD: 4.2 +/- 4.2, PD: 4.3 +/- 2.3, Transplant: 3.2 +/- 1.9). However, disease activity scored by follow-up maximal SLEDAI during dialysis or transplantation showed a significant increase after peritoneal dialysis (HD: 5.0 +/- 3.6, PD: 7.4 +/- 3.7, Transplant: 2.2 +/- 1.7, p < 0.05). When the individual changes in the maximal SLEDAI score were considered, a significant increase was apparent after peritoneal dialysis (p < 0.05), but not after either hemodialysis or renal transplantation. There was no significant difference in cumulative survival rate, and also in technique or graft survival rates of the 3 groups. Among the variables tested, follow-up maximal SLEDAI score was the only significant factor associated with patient survival (odds ratio: 1.15, p < 0.05). The incidence (36% versus 19%) of high disease activity was greater, but not significantly, in the peritoneal dialysis group, as compared to the hemodialysis group. Clinical activity of SLE was apparent in 65% of patients in the first year of dialysis, but none showed any activity after the third year of dialysis. We found that although lupus disease activity declined after patients progressed to ESRD, lupus disease activity still affected patients' survival. An incremental increase in postdialysis lupus activity was not uncommon, especially during the first one year of dialysis. During the follow-up period, maximal SLEDAI score increased significantly after peritoneal dialysis. However, the long-term prognosis was not significantly different according to the treatment modality.

Adult↗

[A case report of systemic lupus erythematosus patient with severe lupus retinopathy and antiphospholipid antibody].

An eleven-year-old boy with systemic lupus erythematosus (SLE) developed severe bilateral lupus retinopathy when he was in active stage of SLE. The patient, who had suffered from SLE for 3 years, was admitted to our hospital because of high grade fever, systemic lymphadenopathy, leukopenia, elevation of erythrocyte sedimentation rate and hypocomplementemia. The dose of prednisolone was increased considering he was exacerbated of SLE, however, the convulsion as CNS lupus occurred to him. After the event he noted loss of vision in his bilateral eyes. The ophthalmologic examination revealed the lesions of cotton-wool spots, retinal vessel dilatations and diffuse occlusions of the retinal arterioles and venules which were compatible with lupus retinopathy. Although the coagulation time was normal, antiphospholipid antibodies were positive at the time of ocular involvement. Careful attention should be paid to the occurrence of lupus retinopathy when the patients with SLE developed in the active stage or CNS lupus, especially they have antiphospholipid antibodies.

Antibodies, Antiphospholipid↗

Determinants of patient survival in systemic lupus erythematosus--focusing on lupus nephritis.

INTRODUCTION: Systemic lupus erythematosus is more prevalent among Asians, African Americans, and Hispanics than in Caucasians. Progress in its management over the past few decades has resulted in improved patient survival, although a considerable proportion of patients are still afflicted with long-term illnesses such as chronic renal failure secondary to lupus nephritis. METHODS AND RESULTS: This is a review of the factors that affect the survival of patients with systemic lupus erythematosus, including discussion of the measures that can further improve the clinical outcomes and prevent long-term complications. Recent advances in the treatment of severe lupus nephritis are emphasized, especially with regard to the minimization of treatment-related adverse events. CONCLUSIONS: While the prognosis of patients with severe lupus manifestations continues to improve, prevention of relapse remains a clinical challenge. Concomitant attention to health economics and sociopolitical issues is required to ensure that all patients can benefit from scientific progress in the understanding and treatment of severe lupus erythematosus.

Adrenal Cortex Hormones↗

Fetal outcome of lupus pregnancy: a retrospective case-control study of the Hopkins Lupus Cohort.

OBJECTIVE: To determine the frequency of pregnancy loss, preterm birth, and therapeutic abortion in patients with systemic lupus erythematosus (SLE) versus controls. METHOD: The outcome of lupus pregnancy was determined in the largest retrospective case-control study to date, involving 203 patients with SLE with 481 pregnancies, 166 "best friends" with 356 pregnancies and 177 relatives with 566 pregnancies. RESULTS: Pregnancy loss (spontaneous abortion, miscarriage, or stillbirth) was more common in lupus pregnancies (21%) than in pregnancies in friends (14%, p = 0.01) or in relatives (8%, p < or = 0.0001) and was more common after the diagnosis of SLE than before (27 19%, p = 0.04). Preterm birth (< or = 36 weeks) was more common in lupus pregnancies (12%) than in pregnancies in friends (4%, p < or = 0.0001) or in relatives (4%, p < or = 0.0001) and was more common after the diagnosis of SLE than before (24 vs 6%, p < or = 0.0001). Preterm birth in lupus pregnancies after the diagnosis of SLE was associated with nonhigh school graduates (p = 0.001), Raynaud's phenomenon (p = 0.007), and hypertension requiring treatment (p = 0.01). CONCLUSION: We conclude that both pregnancy loss and preterm birth are significantly increased in lupus pregnancy versus control pregnancy and that these pregnancy outcomes are more common after the diagnosis of SLE.

Abortion, Spontaneous↗

[A case of male who was taken systemic lupus erythematosus with chilblain lupus].

A 38-year-old male was admitted to our hospital because of high grade fever, erythema of face and extremities and oral ulcer. The laboratory examination showed leukopenia, high titer (320 dil.) of antinuclear antibody, a positive reaction of anti-Sm antibody. Especially histopathology of hand erythema showed hydrophilic degeneration consist with chilblain lupus. Because his symptoms were consistent with the criteria of American College of Rheumatology (1982), he was diagnosed as systemic lupus erythematosus (SLE). After administration of 60 mg prednisolone daily, the symptoms gradually improved. Any recrudescence of serological abnormalities or clinical symptoms was not observed until now, although chilblain lupus still persisted. Originally SLE is frequent in young female and often attended with renal disease, central nerve disease and serositis. We reported a case of middle-aged male who developed SLE with chilblain lupus which was rare skin lesion of SLE without severe organic lesion and discussed the relation among chilblain lupus, discoid lupus erythematosus (DLE) and SLE.

Adult↗

Monozygotic twins concordant for idiopathic thrombocytopenic purpura and discordant for systemic lupus erythematosus and lupus nephritis.

We report on identical twin sisters with systemic lupus erythematosus and lupus nephritis after initial presentation as idiopathic thrombocytopenic purpura. The patients had diverse clinical signs, symptoms and pathological findings of lupus nephritis. The changes in antinuclear antibodies and anti-double-stranded DNA antibodies were quite different. We conclude that even with an identical genetic background and the same environment, the expression of systemic lupus erythematosus and subsequent progression to lupus nephritis in twins was distinct, and antinuclear antibodies and anti-double-stranded DNA antibodies are helpful for clinical monitoring.

Adolescent↗

Role of gamma globulins in pathogenesis of renal lesions in systemic lupus erythematosus and chronic membranous glomerulonephritis, with an observation on the lupus erythematosus cell reaction.

Utilizing the fluorescent antibody method for the histologic demonstration of localized gamma-globulins, we have made the following observations (in contradistinction to the lack of such findings in a variety of normal and pathologic, control kidneys). In systemic lupus erythematosus (a) gamma-globulins were localized in the thickened capillary walls, the "wire-loop" lesions, and the so called "hyaline thrombi" in glomeruli; (b) these sites of localization of gamma-globulins were correlated to a considerable degree with the pattern of accentuated eosinophilia of the glomeruli, as seen in hematoxylin-eosin sections, or with the pattern of PAS-positive areas in the glomeruli in sections stained with the periodic acid-Schiff reaction; (c) and gamma-globulins were localized rarely in large cytoplasmic granules in tubular epithelium and occasionally in glomerular capsular crescents, tubular protein casts, and inflammatory cells, particularly in the cytoplasm of cells identified as immature and mature plasma cells. In nephrotic glomerulonephritis (a) gamma-globulins were localized in the glomerular basement membrane and appertaining structures in chronic membranous glomerulonephritis; (b) gamma-globulins were apparently localized in the altered mesangium in chronic lobular glomerulonephritis; and (c) in the tubular protein casts, presumably representing abnormal glomerular filtrates, gamma-globulins were present in a lesser concentration and other serum proteins in a greater concentration than found in the glomeruli. In positive lupus erythematosus preparations the nuclei of leukocytes, while undergoing transformation and subsequent phagocytosis to form lupus erythematosus cells, were the sites of localization of gamma-globulin (presumably the lupus erythematosus factor) whereas in control preparations no nuclear localization of gamma-globulin occurred. These observations are discussed in relation to the pathogenesis of renal lesions in systemic lupus erythematosus, chronic membranous glomerulonephritis, and amyloidosis.

Amyloidosis↗

Protein array autoantibody profiles for insights into systemic lupus erythematosus and incomplete lupus syndromes.

The objective of this study was to investigate the prevalence and clinical significance of a spectrum of autoantibodies in systemic lupus erythematosus and incomplete lupus syndromes using a proteome microarray bearing 70 autoantigens. Microarrays containing candidate autoantigens or control proteins were printed on 16-section slides. These arrays were used to profile 93 serum samples from patients with systemic lupus erythematosus (SLE (n = 33), incomplete LE (ILE; n = 23), first-degree relatives (FDRs) of SLE patients (n = 20) and non-autoimmune controls (NC; n = 17). Data were analysed using the significance analysis of microarray (SAM) and clustering algorithms. Correlations with disease features were determined. Serum from ILE and SLE patients contained high levels of IgG autoantibodies to 50 autoantigens and IgM autoantibodies to 12 autoantigens. Elevated levels of at least one IgG autoantibody were detected in 26% of SLE and 19% of ILE samples; elevated IgM autoantibodies were present in 13% of SLE and 17% of ILE samples. IgG autoantibodies segregated into seven clusters including two specific for DNA and RNA autoantigens that were correlated with the number of lupus criteria. Three IgG autoantibody clusters specific for collagens, DNA and histones, were correlated with renal involvement. Of the four IgM autoantibody clusters, two were correlated negatively with the number of lupus criteria; none were correlated with renal disease. The IgG : IgM autoantibody ratios generally showed a stepwise increase in the groups following disease burden from NC to SLE. Insights derived from the expanded autoantibody profiling made possible with the antigen array suggest differences in autoreactivity in ILE and SLE. Determining whether the IgM aurotreactivity that predominates in ILE represents an early stage prior to IgG switching or is persistent and relatively protective will require further longitudinal studies.

Adult↗

Lupus anticoagulant: clinical significance in anticardiolipin positive patients with systemic lupus erythematosus.

The significance of anticardiolipin antibodies and the lupus anticoagulant was studied in 58 consecutive patients with systemic lupus erythematosus. On 85 occasions serum IgG and IgM anticardiolipin antibodies were measured by an enzyme linked immunosorbent assay (ELISA), and simultaneous plasma samples tested for lupus anticoagulant activity. The most significant association with clinical events (previous thrombosis or thrombocytopenia occurring in 11/58 patients) was with prolonged tissue thromboplastin inhibition time (TTIT) followed by prolonged kaolin cephalin clotting time (KCCT) then raised IgG anticardiolipin antibody concentrations and dilute Russell's viper venom time. Although IgG anticardiolipin antibodies or KCCT were the most sensitive tests in identifying this group, the TTIT was the most specific (98%). Nine patients were IgG anticardiolipin antibody positive and lupus anticoagulant negative, of whom one had thrombocytopenia but none had thrombosis. The presence of a lupus anticoagulant in anticardiolipin antibody positive patients increases specificity for certain adverse clinical events.

Antibodies↗

Anti-chromatin antibodies in systemic lupus erythematosus: a useful marker for lupus nephropathy.

BACKGROUND: Anti-chromatin antibodies have recently been described in patients with systemic lupus erythematosus (SLE) and it has been suggested that their presence is associated with lupus nephritis. OBJECTIVE: To assess the prevalence and clinical associations of these antibodies in SLE. METHODS: The presence of anti-chromatin antibodies in 100 patients with SLE was investigated by an enzyme linked immunosorbent assay (ELISA). To determine the specificity of these antibodies, 100 patients with primary Sjögren's syndrome, 30 with primary antiphospholipid syndrome (APS), 10 with systemic sclerosis, and 100 normal controls were also tested. RESULTS: Positive levels were detected in 69/100 (69%) patients with SLE. In contrast, they were found in only 8/100 (8%) of those with primary Sjögren's syndrome, in 1/10 (10%) with systemic sclerosis, in 2/30 (7%) with primary APS, and in none of the 100 healthy controls. Patients with anti-chromatin antibodies had a twofold higher prevalence of lupus nephropathy than those without these antibodies (58% v 29%, p<0.01). A significant correlation was found between the levels of anti-chromatin antibodies and disease activity score as measured by the European Consensus Lupus Activity Measurement (ECLAM; p=0.011). CONCLUSIONS: The measurement of anti-chromatin antibodies appears to be a useful addition to the laboratory tests that can help in the diagnosis and treatment of SLE. These antibodies are both sensitive and specific for SLE, and are a useful marker for an increased risk of lupus nephritis.

Adolescent↗