Relationships among organ damage, social support, and depression in African American women with systemic lupus erythematosus.

OBJECTIVES: Systemic lupus erythematosus (SLE) disproportionately strikes African American women. Social support can potentially reduce disease impact. The purpose of this study is to understand the relationship between organ damage and depression in African American women and how social support influences this relationship. METHODS: We used a mixed methods design, analyzing self-reported data on lupus-related organ damage, depression, and social support in 437 African American women with SLE recruited in the Georgians Organized Against Lupus (GOAL) cohort. Moreover, we conducted interviews among 15 GOAL participants to gather patients' perspectives about the role of social support in people who live with lupus. RESULTS: We found a significant association between organ damage and depression ( r = 0.163, p = 0.001), as well as between depression and social support ( F = 17.574, p < 0.001). The quantitative analysis did not render social support as a significant moderator in the organ damage-depression relationship. Interviews, however, revealed that African American women with the most severe organ damage have the greatest need for support. CONCLUSIONS: Social support is a key resource for lupus patients with high disease burden. Overall, these findings highlight the importance of monitoring depressive symptoms in this population and developing interventions aimed to increase social support available to lupus patients.

Perceived stress and reported cognitive symptoms among Georgia patients with systemic lupus erythematosus.

Objective To examine associations of perceived stress with cognitive symptoms among adults with systemic lupus erythematosus (SLE). Methods Among 777 adult (≥18 years) SLE patients, the association of Perceived Stress Scale (PSS) scores with two self-reported cognitive symptoms was examined: forgetfulness (severe/moderate vs. mild/none; from the Systemic Lupus Activity Questionnaire) and difficulty concentrating (all/most vs. some/little/none of the time; from the Lupus Impact Tracker). The study used multivariable logistic regression to estimate the odds ratios (ORs) per minimal important difference (MID = 0.5*SD) of PSS score and cognitive symptoms. Results Forgetfulness and difficulty concentrating were reported by 41.7% and 29.5%, respectively. Women and those with less education and high disease activity had higher PSS scores and were more likely to report cognitive symptoms than their counterparts. With adjustment for age, race, sex, education, and disease activity, each MID increase in PSS score was associated with higher prevalence of forgetfulness (OR = 1.43, 95% CI 1.29-1.47) and difficulty concentrating (OR = 2.19, 95% CI 1.90-2.52). No substantial differences in this association by age, race, sex, or disease activity were noted. Conclusions SLE patients, particularly those with high disease activity, report a high burden of cognitive symptoms, for which stress may be a modifiable risk factor.

Early discoid lupus erythematosus protects against renal disease in patients with systemic lupus erythematosus: longitudinal data from a large Latin American cohort.

OBJECTIVES: The objective of this study was to examine whether early discoid lupus erythematosus (DLE) would be a protective factor for further lupus nephritis in patients with systemic lupus erythematosus (SLE). METHODS: We studied SLE patients from GLADEL, an inception longitudinal cohort from nine Latin American countries. The main predictor was DLE onset, which was defined as physician-documented DLE at SLE diagnosis. The outcome was time from the diagnosis of SLE to new lupus nephritis. Univariate and multivariate survival analyses were conducted to examine the association of DLE onset with time to lupus nephritis. RESULTS: Among 845 GLADEL patients, 204 (24.1%) developed lupus nephritis after SLE diagnosis. Of them, 10 (4.9%) had DLE onset, compared to 83 (12.9%) in the group of 641 patients that remained free of lupus nephritis (hazard ratio 0.39; P = 0.0033). The cumulative proportion of lupus nephritis at 1 and 5 years since SLE diagnosis was 6% and 14%, respectively, in the DLE onset group, compared to 14% and 29% in those without DLE (P = 0.0023). DLE onset was independently associated with a lower risk of lupus nephritis, after controlling for sociodemographic factors and disease severity at diagnosis (hazard ratio 0.38; 95% confidence interval 0.20-0.71). CONCLUSIONS: Our data indicate that DLE onset reduces the risk of further lupus nephritis in patients with SLE, independently of other factors such as age, ethnicity, disease activity, and organ damage. These findings have relevant prognosis implications for SLE patients and their clinicians. Further studies are warranted to unravel the biological and environmental pathways associated with the protective role of DLE against renal disease in patients with SLE.

Validity of a self-administered version of the brief index of lupus damage in a predominantly African American systemic lupus erythematosus cohort.

OBJECTIVE: To assess the reliability and criterion and construct validity of the self-administered Brief Index of Lupus Damage (SA-BILD), a patient-reported measure of organ damage in systemic lupus erythematosus (SLE). METHODS: The validity of the SA-BILD was assessed using data from the Georgians Organized Against Lupus (GOAL) survey. GOAL is a longitudinal cohort of SLE patients predominantly derived from the Georgia Lupus Registry, a population-based registry established in Atlanta, Georgia. In total, 711 participants with documented SLE completed the SA-BILD. To test reliability, the SA-BILD was readministered to 32 patients. Criterion validity was examined in 150 respondents for whom the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) was also completed. Construct validity was assessed among 711 GOAL participants by dividing the SA-BILD scores into quartiles and examining the association with demographics, health status, and health care utilization. RESULTS: The test-retest correlation score was 0.93 (P < 0.0001), the item-by-item agreement with the SDI was >80% for most SA-BILD items, and the Spearman's rho correlation coefficient for the SDI and SA-BILD was moderately high (ρ = 0.59, P < 0.0001). SA-BILD scores showed significant associations in the expected directions with age, disease duration, disease activity, overall health, comorbidity index, and physician visits. CONCLUSION: The SA-BILD was reliable and had very good or good criterion validity compared with the SDI when tested in a predominantly African American cohort of US SLE patients. Associations of SA-BILD scores with sociodemographics and health status were consistent with previous studies. These findings support the use of the SA-BILD as a valid measure of patient-reported damage in SLE.

Benefits of a self-management program in low-income African-American women with systemic lupus erythematosus: results of a pilot test.

Minorities with systemic lupus erythematosus (SLE) are at high risk of poor disease outcomes and may face challenges in effectively self-managing multiple health problems. The Chronic Disease Self-Management Program (CDSMP) is an evidence-based intervention that improves the health of people with chronic illnesses. Although the CDSMP is offered by organizations throughout the United States and many countries around the world, it has not been tested among SLE patients. We pilot tested the benefits of the CDSMP in low-income African American patients with SLE. CDSMP workshops were delivered to 49 African American women with SLE who received medical care at a public lupus clinic in Atlanta, Georgia, US. We compared pre-post CDSMP changes (from baseline to 4 months after the start of the intervention) in health status, self-efficacy and self-management behaviors using self-reported measures. Additionally, we assessed health care utilization changes using electronic administrative records in the 6-month periods before and after the intervention. We observed significant improvements post-intervention in the SF-36 physical health component summary (mean change = 2.4, p = 0.032); self-efficacy (mean change = 0.5, p = 0.035); and several self-management behaviors: cognitive symptoms management (mean change = 0.3, p = 0.036); communication with physicians (mean change = 0.4, p = 0.01); and treatment adherence (mean change = 0.4, p = 0.01). The median number of outpatient visits decreased from 3 to 1 (p < .0001). The CDSMP is a promising intervention for low-income African Americans with SLE. It is an inexpensive program with growing availability around the world that should be further evaluated as a resource to improve patient-centered outcomes and decrease health service utilization among SLE patients.

A rare opportunistic infection in a woman with systemic lupus erythematosus and multiple skin lesions.

Cutaneous lesions in patients with systemic lupus erythematosus (SLE) represent diagnostic challenges. Opportunistic infections should be considered when lupus patients are on immunosuppressive therapy and other causes, such as disease activity, are less likely to explain the skin lesions. Within the spectrum of skin opportunistic infections that might occur in SLE patients, Blastomyces dermatitidis should be suspected when acid-fast positive material with no bacilliform organisms is seen on Ziehl-Nielsen skin biopsy preparations. In this study, we describe one patient with SLE on immunosuppressive therapy, who developed cutaneous blastomycosis despite living in a non-endemic area. Because of lack of awareness about this association and misinterpretation of the skin biopsy results, the diagnosis of atypical mycobacterial infection was initially considered. Subsequent proper tissue staining and interpretation revealed the correct diagnosis of disseminated cutaneous blastomycosis. This description represents the first report of this rare opportunistic skin infection in SLE, illustrating the importance of performing correct preparation and elucidation of the skin biopsy to avoid misdiagnosis and treatment delay.

Obstructive uropathy as the only manifestation of flare in a patient with systemic lupus erythematosus and anti-phospholipid syndrome.

Chronic interstitial cystitis and ureteral stenosis has occasionally been reported in systemic lupus erythematosus, mostly associated with gastrointestinal symptoms. We report a case of obstructive uropathy associated to chronic interstitial cystitis as the only manifestation of lupus flare in a patient with SLE and anti-phospholipid syndrome (APS) who had been in remission for many years. The development of chronic interstitial cystitis in patients with SLE and APS has not been previously reported. Histopathological study of her urinary bladder and ureteral meatus showed chronic inflammatory infiltrate in the subepithelium. Lack of significant lower urinary tract symptoms and gastrointestinal involvement were some of the factors that could have prevented an earlier diagnosis. Obstructive uropathy and renal insufficiency initially improved with immunosuppressive treatment and endoureteral protheses, but poor compliance to the therapy led to ominous ending.

Hiperhomocisteinemia como factor de riesgo trombótico en pacientes con lupus eritematoso sistémico y síndrome antifosfolípido / Hyperhomocysteine like thrombocytic risk factor in patient with systemic lupus erythematous with antiphospholipid syndrome

Objetivos: Determinar la prevalencia de hiperhomocisteinemia (hiperhcy) en pacientes con lupus eritematoso sistémico (LES) con y sin síndrome antifosfolípido (SAF); comparar los niveles de homocisteína (Hcy) entre pacientes con LES (con y sin SAF asociado) y un grupo de controles sanos y determinar la correlación entre hiperhcy y la presencia de anticuerpos antifosfolípidos. Pacientes y métodos: Se estudiaron 44 ptes con LES, portadores o no de SAF. Se los dividió en 2 grupos: 17 con LES y SAF y 27 con LES sin SAF y se compararon con 24 controles sanos. A todos se les realizó interrogatorio, examen físico y pruebas de laboratorio: anticuerpo s anticardiolipinas (aCL), anticoagulante lúpico y Hcy. Se consideró hiperhcy a valores superiores a 9. A los ptes con hiperhcy se los trató con ácido fólico + B6 + B 12 durante un mes. Análisis estadístico: variables cualitativas: Chi cuadrado o Exacta de Fischer y cuantitativas: test T de Student o MannWhitney test. Resultados y conclusiones: Hubo 35 manifestaciones trombóticas en los 44 pacientes. Se encontró Hiperhcy en 27 ptes con LES (61.4%), de los cuales 12 tenían SAF. La diferencia entre los valores de Hcy de los pacientes con o sin SAF no fue significativa (p=0,42). Comparando las concentraciones de Hcy entre pacientes y controles, la diferencia fue muy significativa (p=O,002).También tuvo significación estadística la diferencia entre las concentraciones de Hcy de los pacientes con LES sin SAF vs. controles (p=0,015) y LES con SAF vs. controles (p=0,003). A 33 ptes se les dosó aCL: 20 (60,6%) fueron (+). De estos, 15 (75%) tenían hiperhcy. De los 27 pacientes con LES que tenían hiperhcy, sólo 18 cumplieron con el mes de tratamiento con a.fólico+ B6+ B 12. 16 de 18 (88,8%) normalizaron o disminuyeron la Hcy.

Objectives: to detect the prevalence of hyperhcy in SLE patients with and without antiphospholipid syndrome; to compare the Hcy levels between those patients and healthy controls and to determine the correlation between hyperhcy and antiphospholipid antibodies. Patients and methods: we studied 44 SLE patients: 17 had antiphospholipid syndrome and 27 didn't have it, and we compared them to 24 healthy controls. All of them where checked clinically and with laboratory tests like anticardiolypin antibodies, lupus anticoagulant and Hcy. Hcy > 9 was considered abnormal. Patient who had hyperhcy were treated with folic acid+vitB6+vitBI2 a month along. Statistical analysis: qualitative variables: chi square or Fischer's; quantitative variables: Student's T test or Mann Whitney’s test. Results and conc1utions: there were 35 trombotic manifestations in 44 patients. Hyperhcy was present in 27 SLE patients (61,4%), 12 of them had antiphospholipid syndrome. Hcy concentrations patients vs. controls was statistically different (p=0,002). There was also statistically different the hcy concentration from SLE patients with SAF vs controls (p=O,003) and without SAF vs. controls (p= 0,015). From 33 SLE patients, 20 (33%) were aCL( +). 15(75%) of them had hiperhcy.

Hiperhomocisteinemia como factor de riesgo trombótico en pacientes con lupus eritematoso sistémico y síndrome antifosfolípido / Hyperhomocysteine like thrombocytic risk factor in patient with systemic lupus erythematous with antiphospholipid syndrome

Objetivos: Determinar la prevalencia de hiperhomocisteinemia (hiperhcy) en pacientes con lupus eritematoso sistémico (LES) con y sin síndrome antifosfolípido (SAF); comparar los niveles de homocisteína (Hcy) entre pacientes con LES (con y sin SAF asociado) y un grupo de controles sanos y determinar la correlación entre hiperhcy y la presencia de anticuerpos antifosfolípidos. Pacientes y métodos: Se estudiaron 44 ptes con LES, portadores o no de SAF. Se los dividió en 2 grupos: 17 con LES y SAF y 27 con LES sin SAF y se compararon con 24 controles sanos. A todos se les realizó interrogatorio, examen físico y pruebas de laboratorio: anticuerpo s anticardiolipinas (aCL), anticoagulante lúpico y Hcy. Se consideró hiperhcy a valores superiores a 9. A los ptes con hiperhcy se los trató con ácido fólico + B6 + B 12 durante un mes. Análisis estadístico: variables cualitativas: Chi cuadrado o Exacta de Fischer y cuantitativas: test T de Student o MannWhitney test. Resultados y conclusiones: Hubo 35 manifestaciones trombóticas en los 44 pacientes. Se encontró Hiperhcy en 27 ptes con LES (61.4%), de los cuales 12 tenían SAF. La diferencia entre los valores de Hcy de los pacientes con o sin SAF no fue significativa (p=0,42). Comparando las concentraciones de Hcy entre pacientes y controles, la diferencia fue muy significativa (p=O,002).También tuvo significación estadística la diferencia entre las concentraciones de Hcy de los pacientes con LES sin SAF vs. controles (p=0,015) y LES con SAF vs. controles (p=0,003). A 33 ptes se les dosó aCL: 20 (60,6%) fueron (+). De estos, 15 (75%) tenían hiperhcy. De los 27 pacientes con LES que tenían hiperhcy, sólo 18 cumplieron con el mes de tratamiento con a.fólico+ B6+ B 12. 16 de 18 (88,8%) normalizaron o disminuyeron la Hcy.(AU)

Objectives: to detect the prevalence of hyperhcy in SLE patients with and without antiphospholipid syndrome; to compare the Hcy levels between those patients and healthy controls and to determine the correlation between hyperhcy and antiphospholipid antibodies. Patients and methods: we studied 44 SLE patients: 17 had antiphospholipid syndrome and 27 didnt have it, and we compared them to 24 healthy controls. All of them where checked clinically and with laboratory tests like anticardiolypin antibodies, lupus anticoagulant and Hcy. Hcy > 9 was considered abnormal. Patient who had hyperhcy were treated with folic acid+vitB6+vitBI2 a month along. Statistical analysis: qualitative variables: chi square or Fischers; quantitative variables: Students T test or Mann Whitneyãs test. Results and conc1utions: there were 35 trombotic manifestations in 44 patients. Hyperhcy was present in 27 SLE patients (61,4%), 12 of them had antiphospholipid syndrome. Hcy concentrations patients vs. controls was statistically different (p=0,002). There was also statistically different the hcy concentration from SLE patients with SAF vs controls (p=O,003) and without SAF vs. controls (p= 0,015). From 33 SLE patients, 20 (33%) were aCL( +). 15(75%) of them had hiperhcy.(AU)

Serum anti-beta2-glycoprotein-I and anticardiolipin antibodies during thrombosis in systemic lupus erythematosus patients.

PURPOSE: Antibodies to beta2-glycoprotein-I are more strongly associated with clinical antiphospholipid syndrome than are anticardiolipin antibodies. We previously found a decrease in anticardiolipin antibodies at the time of thrombosis in 6 patients with systemic lupus erythematosus (SLE). We therefore sought to determine the prevalence and levels of antibodies to beta2-glycoprotein-I and to cardiolipin before, during, and after thrombosis in patients with SLE, and to compare them with patients who did not have thrombosis. METHODS: We studied 24 patients with SLE who had at least one episode of thrombosis and 102 patients with SLE without thrombosis. Serum anticardiolipin antibodies were measured by conventional enzyme-linked immunosorbent assay (ELISA) using newborn calf serum as the blocking agent. Serum anti-beta2-glycoprotein-I antibodies were measured by ELISA on nonirradiated plates, using purified human beta2-glycoprotein-I without phospholipid. RESULTS: All patients with thrombosis had anti-beta2-glycoprotein-I antibodies, compared with only 17% of controls (P <0.0001). We observed a significant decrease in serum anti-beta2-glycoprotein-I levels at the time of thrombosis, as compared with previous and subsequent samples. The prevalence and levels of IgG and IgM anticardiolipin antibodies were similar in patients with and without thrombosis. A decrease in IgG or IgM anticardiolipin titers occurred during thrombosis in 6 patients. Anticoagulant, corticosteroid, and immunosuppressive treatments did not appear to affect anti-beta2-glycoprotein-I levels at the time of thrombosis. CONCLUSION: Anti-beta2-glycoprotein-I antibodies are strongly associated with thrombosis in patients with SLE. The decrease of anti-beta2-glycoprotein-I levels at the time of thrombosis may indicate a pathogenic role. This antibody may also be a marker of predisposition for thrombosis in these patients.

Vasculitis in systemic lupus erythematosus.

We studied the frequency, location, clinical and histopathological features, associated manifestations, and prognosis of vasculitides in a cohort of 667 SLE patients. Exclusion of patients with previous vasculitis or insufficient information left 540 patients, 194 of whom has vasculitis (incidence density: 0.053 new cases/person/year, cumulative incidence of 0.051 at one year, 0.232 at 5 years and 0.411 at 10 years). Vasculitis was confirmed by biopsy in 46 cases, by arteriography in five, and by both in three. A single episode of vasculitis occurred in 119 and two or more in 75 patients. Vasculitis was cutaneous in 160, visceral in 24, both in 10. In the first episode of cutaneous vasculitides, 111 had punctuate lesions, 32 palpable purpura, 6 urticaria, 6 ulcers, 8 papules, 5 erythematous plaques or macules confirmed with biopsy, 2 erythema with necrosis, and 1 panniculitis (plus small vessel vasculitis). Of 29 with visceral vasculitis in the first episode, 19 had mononeuritis multiplex, 5 digital necrosis, 3 large artery vasculitis of limbs, one mesenteric, and one coronary, more than one type could appear simultaneously or in subsequent episodes. Patients with vasculitis had longer disease duration and followup, younger age of onset of SLE, and were more frequently males than those without. Lupus manifestations associated with vasculitis in univariate logistic regression included myocarditis, psychosis, Raynaud's phenomenon, serositis, leukopenia, lymphopenia and pleuritis. Vasculitis also associated with the antiphospholipid syndrome. The strength of this association increased when patients with vasculitis confirmed by biopsy and/or arteriography were considered separately. Visceral vasculitis associated with increased mortality when controlled for age of onset and nephropathy.

Prevalence of 13 autoantibodies and of the 16/6 and related pathogenic idiotypes in 465 patients with systemic lupus erythematosus and their relationship with disease activity.

With a cross sectional study of 465 consecutive systemic lupus erythematosus (SLE) patients tested for 13 autoantibodies (Aab) and two idiotypes we determined the prevalence of Aab according to disease activity, both general and at particular organ systems. Seventy seven percent of SLE sera had at least one Aab and 56% had it at high titres. Pathogenic idiotypes had a prevalence of less than 10% and 166 sera had Aab to 5 or more antigens and 9 sera had Aab against all 13 antigens tested. Patients with active disease had increased prevalence of Aab to DNP, ssDNA, ENA, mitochondria and histones when considered at 5 s.d. above the mean of normal controls. The higher positivity of Aab in patients with active disease was confirmed in logistic regression analysis adjusted by age, disease duration, and intensity of treatment. A trend was observed of increased prevalence and titres of Aab from inactive disease without treatment, to inactive disease but still being treated, to active disease. Only 22% of patients with active disease had no Aab and the higher the number of Aab the higher the frequency of active disease. Patients with active arthritis, and to a lesser degree those with active mucocutaneous involvement, had higher prevalence and titres of most autoantibodies than patients with disease activity at other organ systems. Active renal disease associated only with anti-dsDNA, whereas active CNS disease associated with anti-mitochondrial Aab. Our findings support the vision of SLE as an immune dysregulation leading to polyclonal B cell activation with resulting production of multiple Aab. Their profiles seem influenced by genetical, hormonal and environmental factors and, in turn, they contribute to the clinical picture in each patient. Disease activity influences the presence of some, but not all, Aab and some of them may remain present in some patients, even in remission.

Relationship of anticardiolipin antibodies and antiphospholipid syndrome to HLA-DR7 in Mexican patients with systemic lupus erythematosus (SLE).

We studied IgG and IgM anticardiolipin antibodies (aCL) by an ELISA method in 80 Mexican systemic lupus erythematosus (SLE) patients and 378 of their first degree relatives. Sixty five percent of SLE patients and 16% of their relatives were positive for aCL. We also determined allele and haplotype frequencies of Major Histocompatibility Complex (MHC) genes (classes I, II and III) in both patients and relatives. MHC allele and haplotype frequencies of aCL positive and negative individuals were compared to those of normal ethnically matched controls. SLE patients with aCL had statistically significant increased corrected frequencies of HLA-DR3 (pC = 0.04, RR = 2.78); DR7 (pC = 0.005), RR = 3.42) and DQ2 (pC = 0.003, RR = 2.58) antigens. Their first degree relatives positive for aCL also had increased frequency of HLA-DR7 but it did not remain significant after correcting the P value. On the other hand, SLE patients negative for aCL had a moderate increased frequency of DR3 and DQ2 but not of DR7. These results suggest that DR7 associates with the presence of aCL. The distribution of MHC alleles in SLE patients positive for aCL resembles that found in their aCL positive first degree relatives. Since the presence of the antibody is not sufficient to predict a clinical outcome, we studied those patients with reliable clinical data regarding the presence of the antiphospholipid syndrome (aPLS). SLE patients with aPLS had significantly increased frequency of DR7 (pC = 0.004), as did those with probable aPLS (pC = 0.05), while the frequency of DR7 in SLE patients in the doubtful or negative aPLS categories was no different from normal controls. These data support a possible role of DR7 in the development of aCL in SLE patients and their relatives and suggest a contribution of this class II MHC antigen to the development of aPLS within SLE.

Systemic lupus erythematosus in males. A study of 107 Latin American patients.

Clinical and laboratory features were analyzed in 107 Latin American male patients with systemic lupus erythematosus (SLE) who were compared with a group of 1,209 Latin American female patients with SLE to determine the presence of gender-associated differences. Males had an increased prevalence of renal disease, vascular thrombosis, and the presence of anti-dsDNA antibodies, as well as the use of moderate to high doses of corticosteroids, compared with female SLE patients. Although there was no difference in mortality from all causes, SLE-related mortality was higher in the male group. All these findings are consistent with a more severe disease in Latin American males than in female patients from the same region.

Remission of systematic lupus erythematosus.

The occurrence and characteristics of remissions in patients with systematic lupus erythematosus (SLE) have not been determined. We therefore studied this in a cohort of 667 patients and found that 156 patients had achieved at least 1 period of 1 year or more of treatment-free clinical remission. This represents an incidence density of 0.028 new cases/person/year. Remission occurred within the first 2 years of disease in 62 patients. The mean duration of first remission was 4.6 years (range, 1-21 yr), and 81 patients were still in the initial remission up until cutoff time. Half of the remaining 75 patients who flared after achieving remission have not entered again in remission. Twenty-six of the 38 patients who did remained in remission, and the remaining 12 had subsequent flares and remissions. Treatment-free remission accounted for a mean of 5.8 years, corresponding to half the time of follow-up. Remission was not limited to patients with mild disease: at least 41 patients achieved remission despite renal involvement, 19 had had neuropsychiatric lupus, 15 had had thrombocytopenia, and 8 had had hemolytic anemia. We also found that the longer the time lapse between the initial manifestation and the diagnosis of SLE, the less likely it was for a patient to enter into remission. There was a continuous increase in likelihood of achieving a first remission from the beginning of disease up to 30 years of disease duration, when it reached 70%. Patients who achieved remission had increased survival, independently of the effect of other disease manifestations that cause increased mortality. We conclude that a significant proportion of patients with SLE, including those with severe organ involvement, may become symptom-free and in need of no more medication, perhaps indefinitely. Our findings support the notion that, in general, SLE is a more benign disease than previously considered.

Influence of the antiphospholipid syndrome in the survival of patients with systemic lupus erythematosus.

OBJECTIVE: To determine prognostic factors for mortality in a cohort of 667 patients with systemic lupus erythematosus (SLE) including those variables associated with the presence of antiphospholipid antibodies (aPL) as well as antiphospholipid syndrome (APS) itself. METHODS: Analysis of the cohort under a nested case control design by means of Cox proportional hazards regression with and without stepwise method. RESULTS: During the 2039 person-years of followup, there were 49 deaths (cases). Thrombocytopenia, arterial occlusions, and hemolytic anemia were the aPL related manifestations that were associated with decreased survival in univariate analyses. The first 2 were also selected among risk factors for mortality in stepwise Cox multivariate analysis. The syndrome itself was also associated with increased mortality rates, independently of other variables. CONCLUSION: APS is among the variables that confer decreased survival on patients with SLE. This decreased survival is due to some (e.g., thrombocytopenia or arterial occlusions), but not all, of the manifestations of APS.

Presence of serum antibodies to coagulation protein C in patients with systemic lupus erythematosus is not associated with antigenic or functional protein C deficiencies.

Antibodies directed to immunopurified coagulation protein C (PC) were investigated in serum samples from 108 patients with systemic lupus erythematosus (SLE) and found in 12 of them. However, their presence was not associated with antigenic or functional deficiencies of PC, which were documented in 6 and 17 patients, respectively.