Differences in clinical manifestations and increased severity of systemic lupus erythematosus between two groups of Hispanics: European Caucasians versus Latin American mestizos (data from the RELESSER registry).

BACKGROUND: Systemic lupus erythematosus (SLE) is regarded as a prototype autoimmune disease because it can serve as a means for studying differences between ethnic minorities and sex. Traditionally, all Hispanics have been bracketed within the same ethnic group, but there are differences between Hispanics from Spain and those from Latin America, not to mention other Spanish-speaking populations. OBJECTIVES: This study aimed to determine the demographic and clinical characteristics, severity, activity, damage, mortality and co-morbidity of SLE in Hispanics belonging to the two ethnic groups resident in Spain, and to identify any differences. METHODS: This was an observational, multi-centre, retrospective study. The demographic and clinical variables of patients with SLE from 45 rheumatology units were collected. The study was conducted in accordance with Good Clinical Practice guidelines. Hispanic patients from the registry were divided into two groups: Spaniards or European Caucasians (EC) and Latin American mestizos (LAM). Comparative univariate and multivariate statistical analyses were carried out. RESULTS: A total of 3490 SLE patients were included, 90% of whom were female; 3305 (92%) EC and 185 (5%) LAM. LAM patients experienced their first lupus symptoms four years earlier than EC patients and were diagnosed and included in the registry younger, and their SLE was of a shorter duration. The time in months from the first SLE symptoms to diagnosis was longer in EC patients, as were the follow-up periods. LAM patients exhibited higher prevalence rates of myositis, haemolytic anaemia and nephritis, but there were no differences in histological type or serositis. Anti-Sm, anti-Ro and anti-RNP antibodies were more frequently found in LAM patients. LAM patients also had higher levels of disease activity, severity and hospital admissions. However, there were no differences in damage index, mortality or co-morbidity index. In the multivariate analysis, after adjusting for confounders, in several models the odds ratio (95% confidence interval) for a Katz severity index >3 in LAM patients was 1.45 (1.038-2.026; p = 0.02). This difference did not extend to activity levels (i.e. SLEDAI >3; 0.98 (0.30-1.66)). CONCLUSION: SLE in Hispanic EC patients showed clinical differences compared to Hispanic LAM patients. The latter more frequently suffered nephritis and higher severity indices. This study shows that where lupus is concerned, not all Hispanics are equal.

Systemic lupus erythematosus in Spanish males: a study of the Spanish Rheumatology Society Lupus Registry (RELESSER) cohort.

Objective The objective of this study was to describe the demographic, clinical, and immunological manifestations of systemic lupus erythematosus (SLE) in male patients. Methods A cross-sectional, multicenter study was carried out of 3651 patients (353 men, 9.7%, and 3298 women, 90.2%) diagnosed with SLE, included in the Spanish Rheumatology Society SLE Registry (RELESSER). Results Mean ages (18-92 years) of symptom onset were 37 (SD 17) years (men) and 32 (SD 14) years (women). Male/female ratio was 1/9. Age of onset of symptoms and age at diagnosis were higher in men than in women ( p < 0.001). Males were diagnosed earlier than females (p = 0.04) and had more cardiovascular comorbidities ( p < 0.001). Two hundred and thirty-six males (68%) with SLE required hospitalization in comparison with 1713 females (53%) ( p < 0.001). During follow-up, 208 patients died: 30 men (9.3%) and 178 women (5.9%) ( p = 0.02). As regards clinical manifestations, loss of weight ( p = 0.01), lymphadenopathies ( p = 0.02), and splenomegaly ( p = 0.02) were more common in male patients. Female patients were more likely to have inflammatory rash, alopecia, and arthritis ( p < 0.05). As for lung involvement, men with SLE had more pleural fibrosis ( p < 0.001) and pulmonary embolism ( p = 0.01). However, Raynaud's phenomenon was more common in women (35%) than in men (23.7%) ( p < 0.001); lupus nephritis was more common in men, being present in 155 (44.8%) of males versus 933 (29%) of females ( p < 0.001). Multivariate analysis showed that SLE patients with a high Charlson index (more than 3 points) and age > 50 years had a higher mortality (odds ratios 3.6 and 2.1, respectively). Furthermore, SLE patients who developed pulmonary hemorrhage, pulmonary hypertension, psychiatric involvement, complement deficiency, and hemophagocytic syndrome also had higher mortality, regardless of gender. Conclusion Patients with SLE over the age of 50 years have an increased risk of mortality. In Caucasians, age at diagnosis and symptom onset is higher in men than in women. The diagnostic delay is shorter in men. Male SLE patients present more cardiovascular comorbidities, and also more serositis, adenopathies, splenomegaly, renal involvement, convulsion, thrombosis, and lupus anticoagulant positivity than women.

Gender-associated comorbidities in rheumatoid arthritis and their impact on outcome: data from GENIRA.

GENIRA [Gender in Rheumatoid Arthritis (RA)] is a comprehensive project aimed at studying gender differences in RA patients and how these differences impact on these patient outcomes. We are now reporting such data. Seventy RA patients of each gender were cross-sectionally evaluated following a preestablished protocol. Univariate and multivariate analyses focused in the different gender-associated comorbidity profiles and how they impact in the quality of life and disability of RA patients as assessed by the SF-36 and the Modified Health Assessment Questionnaire (M-HAQ), respectively. Both groups were comparable regarding their main demographic and clinical features. Different comorbidity profiles were found in both genders, with higher frequencies of diabetes mellitus, peptic ulcer, ischemic heart disease, smoking and chronic obstructive pulmonary disease among men and of depression and osteoporosis among women. The M-HAQ was lower in women than in men (0.89 ± 2.6 vs 0.22 ± 0.9, p = 0.04) as there were some sub-scales of the SF-36 [mental health (63.7 ± 22.0 vs 71.8 ± 21.1; p = 0.02), general health (41.3 ± 21.7 vs 50.0 ± 24.3; p = 0.02), physical functioning (PF) (57.7 ± 22.1 vs 67.3 ± 22.7; p = 0.01) and the physical summary component (PSC) (39.3 ± 8.9 vs 42.4 ± 9.3, p = 0.04)]. Multivariate analysis indicated the independent association between depression and osteoporosis rather than gender with the M-HAQ, PSC and PF and of only depression with the MH and GH. Women with RA present significantly worse disability and QOL outcomes than men; these differences can be explained by female gender-associated comorbidities such as depression and osteoporosis rather than gender per se.

Analysis of disease activity and response to treatment in a large Spanish cohort of patients with systemic lupus erythematosus.

OBJECTIVES: The objectives of this paper are to study the impact of disease activity in a large cohort of patients with systemic lupus erythematosus (SLE) and estimate the rate of response to therapies. METHODS: We conducted a nationwide, retrospective, multicenter, cross-sectional cohort study of 3658 SLE patients. Data on demographics, disease characteristics: activity (SELENA-SLEDAI), damage, severity, hospitalizations and therapies were collected. Factors associated with refractory disease were identified by logistic regression. RESULTS: A total of 3658 patients (90% female; median SLE duration (interquartile range): 10.4 years (5.3-17.1)) were included. At the time of their last evaluation, 14.7% of the patients had moderate-severe SLE (SELENA-SLEDAI score ≥6). There were 1954 (53.4%) patients who were hospitalized for activity at least once over the course of the disease. At some stage, 84.6% and 78.8% of the patients received glucocorticoids and antimalarials, respectively, and 51.3% of the patients received at least one immunosuppressant. Owing to either toxicity or ineffectiveness, cyclophosphamide was withdrawn in 21.5% of the cases, mycophenolate mofetil in 24.9%, azathioprine in 40.2% and methotrexate in 46.8%. At some stage, 7.3% of the patients received at least one biologic. A total of 898 (24.5%) patients had refractory SLE at some stage. Renal, neuropsychiatric, vasculitic, hematological and musculoskeletal involvement, a younger age at diagnosis and male gender were associated with refractory disease. CONCLUSIONS: A significant percentage of patients have moderately-to-severely active SLE at some stage. Disease activity has a big impact in terms of need for treatment and cause of hospitalization. The effectiveness of the standard therapies for reducing disease activity is clearly insufficient. Some clinical features are associated with refractory SLE.

Circulating cytokines in active polymyalgia rheumatica.

OBJECTIVE: To characterise the circulating cytokine profile and the cellular source of circulating cytokines in polymyalgia rheumatica (PMR). METHODS: The study included 34 patients with active untreated PMR and 17 age-matched healthy controls (HC). Circulating cytokines were measured by cytometric bead array and ELISA. Intracellular cytokines were assessed in CD3+ and CD14+ cells by flow cytometry. Cytokines in cell culture supernatants were also determined after polyclonal stimulation of patients' peripheral blood mononuclear cells. RESULTS: Circulating levels of interleukin-6 (IL6) were significantly higher in subjects with active PMR than in HC. Corticosteroid (CS) treatment was followed by a decrease in the level of IL6. Intracellular cytokine staining showed that circulating monocytes did not produce higher amounts of proinflammatory cytokines in patients with PMR than in HC. There was a discordance between serum levels and cytokine-producing monocyte and T cells, and it was not possible to demonstrate a Th1 bias in the peripheral compartment. CONCLUSIONS: Active PMR is characterised by increased serum levels of IL6, but not of other proinflammatory cytokines, that are rapidly suppressed by CS treatment. As circulating monocytes do not show increased production of proinflammatory cytokines, IL6 may be mainly produced in the inflamed tissue. A study of the circulating cytokine profile and its cellular source may provide a clue to new therapeutic options.

Validation of the Spanish, Portuguese and French versions of the Lupus Damage Index questionnaire: data from North and South America, Spain and Portugal.

We have previously developed and validated a self-administered questionnaire, modelled after the Systemic Lupus International Collaborating Clinics Damage Index (SDI), the Lupus Damage Index Questionnaire (LDIQ), which may allow the ascertainment of this construct in systemic lupus erythematosus (SLE) patients followed in the community and thus expand observations made about damage. We have now translated, back-translated and adapted the LDIQ to Spanish, Portuguese and French and applied it to patients followed at academic and non-academic centres in North and South America, Portugal and Spain while their physicians scored the SDI. A total of 887 patients (659 Spanish-speaking, 140 Portuguese-speaking and 80 French-speaking patients) and 40 physicians participated. Overall, patients scored all LDIQ versions higher than their physicians (total score and all domains). Infrequent manifestations had less optimal clinimetric properties but overall agreement was more than 95% for the majority of items. Higher correlations were observed among the Spanish-speaking patients than the Portuguese-speaking and French-speaking patients; further adjustments may be needed before the Portuguese and French versions of the LDIQ are applied in community-based studies. The relationship between the LDIQ and other outcome parameters is currently being investigated in a different patient sample.

Relationship between damage and mortality in juvenile-onset systemic lupus erythematosus: Cluster analyses in a large cohort from the Spanish Society of Rheumatology Lupus Registry (RELESSER).

OBJECTIVES: To identify patterns (clusters) of damage manifestation within a large cohort of juvenile SLE (jSLE) patients and evaluate their possible association with mortality. METHODS: This is a multicentre, descriptive, cross-sectional study of a cohort of 345 jSLE patients from the Spanish Society of Rheumatology Lupus Registry. Organ damage was ascertained using the Systemic Lupus International Collaborating Clinics Damage Index. Using cluster analysis, groups of patients with similar patterns of damage manifestation were identified and compared. RESULTS: Mean age (years) ±â€¯S.D. at diagnosis was 14.2 ±â€¯2.89; 88.7% were female and 93.4% were Caucasian. Mean SLICC/ACR DI ±â€¯S.D. was 1.27 ±â€¯1.63. A total of 12 (3.5%) patients died. Three damage clusters were identified: Cluster 1 (72.7% of patients) presented a lower number of individuals with damage (22.3% vs. 100% in Clusters 2 and 3, P < 0.001); Cluster 2 (14.5% of patients) was characterized by renal damage in 60% of patients, significantly more than Clusters 1 and 3 (P < 0.001), in addition to increased more ocular, cardiovascular and gonadal damage; Cluster 3 (12.7%) was the only group with musculoskeletal damage (100%), significantly higher than in Clusters 1 and 2 (P < 0.001). The overall mortality rate in Cluster 2 was 2.2 times higher than that in Cluster 3 and 5 times higher than that in Cluster 1 (P < 0.017 for both comparisons). CONCLUSIONS: In a large cohort of jSLE patients, renal and musculoskeletal damage manifestations were the two dominant forms of damage by which patients were sorted into clinically meaningful clusters. We found two clusters of jSLE with important clinical damage that were associated with higher rates of mortality, especially for the cluster of patients with predominant renal damage. Physicians should be particularly vigilant to the early prevention of damage in this subset of jSLE patients with kidney involvement.

Acute bacterial arthritis caused by group C streptococci.

OBJECTIVES: To summarize the characteristics of and clinical experience with joint infection by group C streptococcus. METHODS: Two new cases of acute bacterial arthritis due to group C streptococci are reported, and a MEDLINE search was performed, which located 22 additional cases. RESULTS: Mean age (+/- standard deviation) of all 24 patients was 46 +/- 25 years, and most patients were men (75%). The species most frequently identified was Streptococcus equisimilis (12 cases). Eight patients (33%) had a general risk factor for infection, and the same percentage had some type of arthropathy. Only 3 patients had previous contact with animals. The infection was polyarticular in a third of cases, and the joint most frequently involved was the knee. The majority of patients showed a good response to the treatment with intravenous penicillin. However, 3 patients had functional sequelae, 2 more had residual radiological lesions, and 3 died. CONCLUSIONS: Acute bacterial arthritis due to group C streptococci is a serious but uncommon entity that can affect patients without risk factors. Rapid diagnosis and treatment may improve the outcome. Semin Arthritis Rheum 31:43-51.

Functional outcome and subset identification in RA patients from meridional Europe: analysis of a Spanish cohort.

The aim of this study was to study the short-term functional and anatomical prognosis of rheumatoid arthritis (RA) in a series of Spanish patients and to identify different subsets of patients as well as possible baseline factors associated with specific outcomes. All patients seen in our division who met the ACR criteria for RA and with disease duration between 2 and 7 years were eligible for the study. Available patients were further evaluated at the clinic for disease activity using biological tests and joint indices as joint counts and Thompson's index, functional capacity using the ACR functional classification (ACR-FC) and the modified Health Assessment Questionnaire (M-HAQ) and radiologic damage by the Sharp's radiologic scoring method. Cluster analysis was used to identify different clinical subsets of patients. One hundred and sixty-three patients were eligible for the study, 13 could not be located or refused to participate and 12 had died. Mean (+/-SD) age at disease onset and mean disease duration were, respectively, 56(+/-14) years and (55+/-20) months. Median (interquartile range) of M-HAQ was 0.4 (0.1-1.1) and 41% of patients were in III or IV ACR-FC. The majority of patients (93%) showed radiologic lesions and 65% had erosions. Cluster analysis identified three subsets: cluster I (70% of patients) was characterised by a good prognosis, cluster II (13%) by a high level of disease activity, and cluster III (17%) by a greater anatomic damage and longer disease duration. No baseline predictive markers were found for these different outcomes. We concluded that RA portends an overall poor short-term prognosis in a relative large percentage of our patients with significant anatomic and functional sequelae. Aggressive management is specially indicated in this subgroup of patients, although definitive prognostic markers for its early identification are still lacking.

Synovial Biopsy of the Knee Joint under Direct Visualization by Needle Arthroscopy.

During a 30-month period, 51 needle-arthroscopic procedures using a small diameter (1.9 mm) flexible fiberoptic arthroscope were performed to obtain synovial tissue from knee joints. This procedure was performed in an outpatient setting using only a local anesthetic. Twenty-eight procedures were conducted involving 23 rheumatoid arthritis patients and 1 osteoarthritis patient as part of research protocols. In addition, arthroscopy and synovial biopsy were performed on 23 patients to obtain tissue for diagnostic purposes. Large samples of synovial tissue (over 10 g) were easily obtained in most patients. No complications were observed.The easy accessibility to large quantities of synovial tissue provided by needle arthroscopy should enable investigators to better define the pathogenic mechanisms operative in early rheumatoid arthritis. In addition, this technique will afford opportunities for determining the influence of current treatments as well as experimental treatments on molecular events in the synovium of patients. We conclude that outpatient needle arthroscopy using a small fiberoptic arthroscope can be used safely by rheumatologists trained in arthroscopy to obtain synovial biopsies for research and diagnostic purposes.

Predictors of renal involvement in patients with recent onset systemic lupus erythematosus.

We have examined the demographic, clinical, and laboratory predictors of the concomitant or subsequent occurrence of lupus nephritis among a cohort of patients with disease manifestations < 1 year diagnosed as systemic lupus erythematosus (SLE) either at entry (baseline) into the cohort or during the subsequent 5 years. Patients studied were from a larger cohort with either early defined or undifferentiated connective tissue diseases; of the possible 74 patients, data were adequate in 58, 44 with SLE at entry and 14 at followup. Demographic, clinical, and laboratory data were examined first by univariate and then by multivariate analyses for their ability to predict lupus nephritis.Thirty of the 54 patients developed lupus nephritis with proteinuria as its most common manifestation. In 25 of the 30, nephritis occurred within the first 2 years of disease. Among those with a renal biopsy, the most common finding was World Health Organization class IV. By univariate analysis, the following variables were found more frequently among those patients who developed lupus nephritis versus those who did not: younger age, hypertension, anti-double-stranded deoxyribonucleic acid and baseline diagnosis of SLE. In the multivariate analysis, only SLE at baseline and hypertension were identified as predictors of nephritis, whereas the presence of nonvasculitic skin rashes were negatively associated.Lupus nephritis is an early event in the course of SLE. Antinuclear antibody negative patients and those with incomplete SLE are less likely to develop lupus nephritis. Concomitant hypertension is associated with an increased risk of nephritis.

[Biopsy of the temporal artery. Experience with the use of a protocol at an ENT department]. / Biopsia de la arteria temporal. Experiencia de su protocolización en un Servicio de ORL.

INTRODUCTION: Temporal artery (TA) biopsy is required for the evaluation of several clinical scenarios because of the therapeutic implications of a positive result. We describe the experience of our department, which is the center for this technique in our hospital. PATIENTS AND METHODS: A retrospective study was made of all TA biopsies performed in the Department of Otolaryngology, Hospital Sierrallana, from January 1995 to April 1997. RESULTS: Thirty-four biopsies of the TA artery were performed in 33 patients for possible temporal arteritis. Mean age was 76 years (range: 61-87 years). Twelve were male and 21 female. Twenty-two patients (66.6%) had recent onset cephalalgia, 11 (33.3%) polymyalgia rheumatica, 5 (15.1%) mandibular claudication, and 8 (24.2%) ocular symptoms. In 75.8% ESR was elevated. An abnormal TA exam was observed in 9 cases (27.3%), 6 of which had a positive biopsy. Only 1 of the 15 patients without local findings at the physical examination had a positive TA biopsy. No information on the physical exam was recorded for the other 2 patients with a positive biopsy. CONCLUSIONS: TA biopsy is a relatively easy surgical procedure with a very low rate of complications. An abnormal physical TA examination correlates with a positive histopathological study.

European consensus statement on the terminology used in the management of lupus glomerulonephritis.

Systemic lupus erythematosus (SLE) is a complex, multisystem autoimmune disorder, which often involves referral to multiple medical specialists. Lupus nephritis (LN) occurs in ~35% of adults with SLE and predicts poor survival. There is currently no consensus on how to manage patients with SLE or LN across specialties and across different European countries. The Lupus Nephritis Terminology Advisory Group was formed to address this issue as it impacts upon LN treatment. It has developed consensus statements based on opinions from expert panel meetings with nephrologists, nephropathologists, rheumatologists, clinical immunologists and internal medicine specialists from many European countries, after reviewing current guidelines from the European League Against Rheumatism, the American College of Rheumatology and the participants' experience. In this article, we report consensus statements that were developed in six important areas: classification of patients with LN, how classification affects the selection of treatment options and definitions of induction, response, flare and maintenance. We have also proposed a consensus for the terminology involved in the management of LN that is consistent with clinical opinion gathered from multidisciplinary expert meetings and with existing guidelines. We believe this consensus approach provides agreed expert opinion to clinicians and will form the basis for optimising LN treatment.

Systemic lupus erythematosus in a multiethnic U.S. cohort (LUMINA) XXXI: factors associated with patients being lost to follow-up.

The objective of this study was to determine the frequency of loss to follow-up and the factors predictive of its occurrence in a systemic lupus erythematosus (SLE) multiethnic cohort. We studied SLE patients from the LUMINA cohort (Hispanics from Texas and from the Island of Puerto Rico, African-Americans and Caucasians). Loss to follow-up was defined as subjects who failed to attend two or more of the latest consecutive yearly study visits. The relationship between baseline features and loss to follow-up was examined by univariable and multivariable Cox regression analyses with loss to follow-up as the dependent variable. The retention rate in the cohort was estimated by the Kaplan-Meier method. Five-hundred and fifty-four patients with a mean (SD) follow-up of 3.4 (2.9) years were studied. One-hundred and fifty-eight (29%) met the definition of lost to follow-up. The cumulative loss to follow-up rate at five years was 36%. The cumulative loss to follow-up rate at five years was higher for the African-Americans. Patients lost to follow-up tended to be younger and more likely to have poor social support and higher levels of helplessness. They also tended to have more renal involvement and more active disease as per the Systemic Lupus Activity Measure-Revised. Disease activity (hazard ratio = 1.04, 95% confidence interval 1.01-1.07, P = 0.02) was the only variable independently contributing to loss to follow-up. Our data suggest that in longitudinal SLE studies, loss to follow-up does not occur at random and it differs between ethnic groups and is also particularly higher among patients with more active disease. Pro-active measures may need to be applied to decrease the probability of patients 'at risk' of becoming lost to follow-up and to preserve the integrity of the cohort.

Systemic lupus erythematosus in a multiethnic cohort: LUMINA XXXV. Predictive factors of high disease activity over time.

AIM: To ascertain the predictive factors of high levels of disease activity in systemic lupus erythematosus (SLE). PATIENTS AND METHODS: Patients with SLE (American College of Radiology criteria), aged >or=16 years, with disease duration 10). A basic multivariable model (including age, sex, ethnicity, health insurance, social support, abnormal illness-related behaviours, helplessness and prior disease activity) was first examined. Additional models were built by including other variables. RESULTS: 554 patients (100 Hispanics from Texas, 94 Hispanics from Puerto Rico, 199 African Americans, 161 Caucasians) and 2366 visits were analysed; 47% of the patients and 29% of the visits met the definition of high disease activity (more common among African Americans (72.0%) and Hispanics from Texas (71.3%) than among Caucasians (43.9%) and Hispanics from Puerto Rico (31.9%)). Variables found to predict high levels of disease activity were Hispanic (from Texas) and African American ethnicities, lack of health insurance, helplessness, abnormal illness-related behaviours and poor social support; age was negatively associated with high levels of disease activity. African admixture and anti-double-stranded DNA antibodies also predicted high levels of disease activity, as did prior disease activity. None of the human leucocyte antigen variables were retained in the models. CONCLUSIONS: Socioeconomic-demographic (age, ethnicity, health insurance), behavioural and psychological variables are important mediators of high levels of disease activity in SLE during its course. Interventions aimed at modifiable factors may improve the outcomes of SLE.

Systemic lupus erythematosus in a multiethnic US cohort (LUMINA): XXIV. Cytotoxic treatment is an additional risk factor for the development of symptomatic osteonecrosis in lupus patients: results of a nested matched case-control study.

BACKGROUND: Osteonecrosis is common in systemic lupus erythematosus (SLE) and often disabling. The role of glucocorticoids in its development is well known. OBJECTIVE: To explore other possible risk factors for osteonecrosis in SLE. METHODS: A nested matched case-control study undertaken in the context of a large, longitudinal, multiethnic lupus cohort (LUMINA), currently formed of 571 SLE patients meeting American College of Rheumatology criteria. All those developing symptomatic osteonecrosis after the diagnosis of SLE were considered cases. Two controls matched for age, disease duration, ethnicity, and centre were selected for each case. Cases and controls were compared by univariable analyses using selected variables. Variables with p<0.10 and those thought clinically relevant were entered into conditional logistic regression models including either the average dose or the highest dose of glucocorticoids, with osteonecrosis as the dependent variable. RESULTS: 32 cases were identified and 59 matched controls selected (in five cases only one control could be found). By univariable analyses, both groups were largely comparable for socioeconomic-demographic, clinical, and laboratory variables. Cases were less exposed to hydroxychloroquine (as assessed by the percentage of exposure time) (p = 0.026), used higher doses of glucocorticoids (average and highest doses) (p = 0.011 and 0.001, respectively), and received cytotoxic drugs more often (p = 0.015). In the multivariable analyses only cytotoxic drug use (both models) and the highest dose of glucocorticoids remained associated with the occurrence of osteonecrosis. CONCLUSIONS: Cytotoxic drug use is a risk factor for the development of symptomatic osteonecrosis in SLE patients, along with glucocorticoids. No definite protective factors were identified.

Lack of recording of systemic lupus erythematosus in the death certificates of lupus patients.

OBJECTIVE: To determine to what extent the diagnosis of systemic lupus erythematosus (SLE) in deceased lupus patients is under-reported in death certificates, and the patient characteristics associated with such an occurrence. METHODS: The death certificates of 76 of the 81 deceased SLE patients from two US lupus cohorts (LUMINA for Lupus in Minorities: Nature vs Nurture and CLU for Carolina Lupus Study), including 570 and 265 patients, respectively, were obtained from the Offices of Vital Statistics of the states where the patients died (Alabama, Georgia, North Carolina, South Carolina, Tennessee and Texas). Both cohorts included patients with SLE as per the American College of Rheumatology criteria, aged > or =16 yr, and disease duration at enrolment of < or =5 yr. The median duration of follow-up in each cohort at the time of these analyses ranged from 38.1 to 53.0 months. Standard univariable analyses were performed comparing patients with SLE recorded anywhere in the death certificate and those without it. A multivariable logistic regression model was performed to identify the variables independently associated with not recording SLE in death certificates. RESULTS: In 30 (40%) death certificates, SLE was not recorded anywhere in the death certificate. In univariable analyses, older age was associated with lack of recording of SLE in death certificates [mean age (standard deviation) 50.9 (15.6) years and 39.1 (18.6) yr among those for whom SLE was omitted and included on the death certificates, respectively, P = 0.005]. Patients without health insurance, those dying of a cardiovascular event and those of Caucasian ethnicity were also more likely to be in the non-recorded group. In the multivariable analysis, variables independently associated with not recording SLE as cause of death were older age [odds ratio = (95% confidence interval) 1.043 (1.005-1.083 per yr increase); P = 0.023] and lack of health insurance [4.649 (1.152-18.768); P = 0.031]. CONCLUSIONS: A high proportion of SLE diagnoses are not recorded in death certificates. Older patients and those without health insurance are more prone to have SLE not recorded. These findings do have implications for the assessment of the impact of this disease in epidemiological studies conducted using vital statistics records.