Challenges to optimal rheumatology care: a patient-centered focus group study.

OBJECTIVE: To assess challenges to optimal rheumatology care from the perspective of patients served by our institution's rheumatology division. DESIGN SETTING AND PARTICIPANTS: Focus group study of adult rheumatic disease patients who attend clinics at a university teaching hospital in Montreal, Canada. INTERVENTIONS: Individuals participated in 1-h focus group discussions concerning their experiences and beliefs regarding rheumatology care. Sessions were recorded and transcripts generated. A thematic analysis approach was used by two individual analyzers. MAIN OUTCOME MEASURES AND RESULTS: Eighteen patients participated in three focus groups (group one = 8 patients; group two = 5; group three = 5). Eleven patients had systemic lupus erythematosus, 6 had rheumatoid arthritis, and 1 patient had psoriatic arthritis. The average age (standard deviation) was 51.2 (14.0) years, disease duration 23.5 (14.5) years, and in the majority had at least a high school education. All participants were female and 72.2% were Caucasian. Three main themes emerged: theme 1 identified patients' needs for information and support, at diagnosis and throughout the disease trajectory; theme 2 identified barriers to accessing health care: theme 3 identified patients' beliefs regarding improvements needed to optimize their experiences throughout the disease course. CONCLUSIONS: Our focus group study not only clarified the needs of rheumatology patients with chronic inflammatory disease, and identified barriers to optimal rheumatology care, but also was a source of recommendations that might improve patient experiences in seeking health care in a rheumatology setting. Limitations include the fact that our participants were all female, and mostly were middle aged, Caucasian and well educated. Regardless, the findings can help inform efforts to improve rheumatology care. Key Points • Our focus group study clarified the needs of chronic inflammatory rheumatic disease, and identified barriers to optimal rheumatology care. • Despite some potential limitations, our work provides recommendations that could improve patient experiences when seeking health care in a rheumatology setting.

What investigations are needed to optimally monitor for malignancies in SLE?

OBJECTIVE: The overall cancer incidence risk in systemic lupus erythematosus (SLE) is approximately 15%-20% more than in the general population. Nevertheless, to date, the optimal malignancy screening measures in SLE remain undefined. Our objective is to determine what investigations are needed to optimally monitor for malignancies in SLE in order to inform upcoming Canadian Rheumatology Association recommendations. METHODS: We conducted a systematic search looking at three scientific sources, Embase, Medline and Cochrane, in an attempt to identify cancer screening recommendations for patients with SLE. We used a filter for observational studies and included articles published in 2000 and onward. RESULTS: The initial search strategy led to 986 records. After removal of duplicates and articles unrelated to SLE, we were left with 497 titles. From those, 79 research articles on cancer incidence in SLE were isolated and reviewed. Of the 79 original research papers, 25 offered screening recommendations, 14 suggested additional cancer screening whereas 11 studies simply promoted adherence to general population screening measures. The suggestions for more rigorous screening included recommending human papilloma virus testing in addition to routine cervical screening, and/or that cervical screening should be performed annually and/or suggested urine cancer screening in SLE patients with a history of cyclophosphamide exposure. CONCLUSIONS: We found no original research studies directly comparing cancer screening strategies in SLE. Generally, authors recommend adherence to general population screening measures, particularly cervical screening. This, possibly with adding targeted screening in special cases (e.g. annual urine cytology in patients with prior cyclophosphamide exposure, and considering existing lung cancer screening guidelines for past heavy smokers), may be a reasonable approach for cancer screening in SLE.

Neurodevelopmental disorders in children born to mothers with systemic lupus erythematosus.

Children born to women with systemic lupus erythematosus seem to have a potentially increased risk of neurodevelopmental disorders compared to children born to healthy women. Recent experimental data suggest in utero exposure to maternal antibodies and cytokines as important risk factors for neurodevelopmental disorders. Interestingly, women with systemic lupus erythematosus display high levels of autoantibodies and cytokines, which have been shown, in animal models, to alter fetal brain development and induce behavioral anomalies in offspring. Furthermore, subjects with systemic lupus erythematosus and neurodevelopmental disorders share a common genetic predisposition, which could impair the fetal immune response to in utero immunologic insults. Moreover, systemic lupus erythematosus pregnancies are at increased risk of adverse obstetrical outcomes and medication exposures, which have been implicated as potential risk factors for neurodevelopmental disorders. In this article, we review the current state of knowledge on neurodevelopmental disorders and their potential determinants in systemic lupus erythematosus offspring.

The needs of persons with lupus and health care providers: a qualitative study aimed toward the development of the Lupus Interactive Navigator™.

OBJECTIVE: Systemic lupus erythematosus is an inflammatory autoimmune disease associated with high morbidity and unacceptable mortality. A major challenge for persons with lupus is coping with their illness and complex care. Our objective was to identify the informational and resource needs of persons with lupus, rheumatologists, and allied health professionals treating lupus. Our findings will be applied toward the development of an innovative web-based technology, the Lupus Interactive Navigator (LIN™), to facilitate and support engagement and self-management for persons with lupus. METHODS: Eight focus groups were conducted: four groups of persons with lupus (n=29), three groups of rheumatologists (n=20), and one group of allied health professionals (n=8). The groups were held in British Columbia, Ontario, and Quebec. All sessions were audio-recorded and transcribed verbatim. Qualitative analysis was performed using grounded theory. The transcripts were reviewed independently and coded by the moderator and co-moderator using 1) qualitative data analysis software developed by Provalis Research, Montreal, Canada, and 2) manual coding. RESULTS: Four main themes emerged: 1) specific information and resource needs; 2) barriers to engagement in health care; 3) facilitators of engagement in health care; and 4) tools identified as helpful for the self-management of lupus. CONCLUSION: These findings will help guide the scope of LIN™ with relevant information topics and specific tools that will be most helpful to the diverse needs of persons with lupus and their health care providers.

Features associated with cardiac abnormalities in systemic lupus erythematosus.

OBJECTIVES: To determine the prevalence of echocardiographic abnormalities and identify associated clinical and laboratory features in a large systemic lupus erythematosus (SLE) cohort. METHODS: Patients fulfilling ACR criteria for SLE underwent a transthoracic echocardiogram (TTE) between January 2005 and June 2006. Variables used as potential correlates included age, sex, ethnicity, lupus duration, lupus disease activity (SLEDAI), cumulative damage (SLICC/ACR damage index (DI)), arterial hypertension, diabetes, current smoking, medication use and laboratory data. Multivariate logistic regression was used to examine the association between TTE abnormalities and potential determinants. RESULTS: For the 217 subjects with a TTE performed during the study, the main abnormalities were of the mitral valve (37.3%) and included thickening (25.4%) and insufficiency (25.8%). Other findings included pulmonary artery pressure (PAP) ≥ 30( )mm( )Hg (10.1%), pericardial effusion (4.6%), hypokinesis (2.8%), and aortic insufficiency (3.7%). In multivariate analysis, mitral insufficiency was associated with the use of corticosteroids (OR 2.90; 95%CI 1.42-5.94) and hypokinesis with angiotensin-converting enzyme inhibitors (12.89; 1.06-157.18). Elevated PAP was associated with age (1.04; 1.01-1.07) and with DI (1.20; 1.01-1.42). CONCLUSION: Valvular abnormalities are frequent in patients with SLE, with mitral valve lesions occurring in over one third. TTE screening may be indicated in patients with SLE, especially for those with identified risk factors such as corticosteroid use.

Association of the Charlson comorbidity index with mortality in systemic lupus erythematosus.

OBJECTIVE: To investigate whether comorbidity as assessed by the Charlson Comorbidity Index (CCI) is associated with mortality in a long-term followup of systemic lupus erythematosus (SLE) patients. METHODS: Data were collected from 499 SLE patients attending the Lupus Clinic at the McGill University Health Center, Montreal, Quebec, Canada, and 170 SLE patients from the Department of Rheumatology at Lund University Hospital, Lund, Sweden. This included data on comorbidity, demographics, disease activity, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), and antiphospholipid antibody syndrome (APS). Variables were entered into a Cox proportional hazards survival model. RESULTS: Mortality risk in the Montreal cohort was associated with the CCI (hazard ratio [HR] 1.57 per unit increase in the CCI, 95% confidence interval [95% CI] 1.18-2.09) and age (HR 1.04 per year increase in age, 95% CI 1.00-1.09). The CCI and age at diagnosis were also associated with mortality in the Lund cohort (CCI: HR 1.35, 95% CI 1.13-1.60; age: HR 1.09, 95% CI 1.05-1.12). Furthermore, the SDI was associated with mortality in the Lund cohort (HR 1.40, 95% CI 1.19-1.64), while a wide CI for the estimate in the Montreal cohort prevented a definitive conclusion (HR 1.20, 95% CI 0.97-1.48). We did not find a strong association between mortality and sex, race/ethnicity, disease activity, or APS in either cohort. CONCLUSION: In this study, comorbidity as measured by the CCI was associated with decreased survival independent of age, lupus disease activity, and damage. This suggests that the CCI may be useful in capturing comorbidity for clinical research in SLE.

Acute lupus myocarditis: Clinical features and outcome.

BACKGROUND: Cardiomyopathy in systemic lupus erythematosus (SLE) may be secondary to myocardial inflammation (i.e. myocarditis) or to systemic complications such as hypertension. Symptomatic left ventricular dysfunction is the most common clinical presentation of cardiomyopathy and is potentially life threatening. Identifying the cause is critical as it dictates therapy. METHODS: We present three cases of left ventricular failure suggestive of myocarditis in SLE patients followed in the Lupus Clinic of the Montreal General Hospital over a 5-year period. RESULTS: The most frequent presentation is acute onset of a marked reduction of the left ventricular ejection fraction (LVEF). All patients were treated with cardiac support, prednisone, and additional immunosuppressive medications. Improvement of symptoms and LVEF was observed in two of three patients. CONCLUSION: Myocarditis is a rare, but life-threatening, manifestation of SLE. With immunosuppressive medications and cardiovascular support, the long-term outcome is usually favorable.

Surveillance of systemic autoimmune rheumatic diseases using administrative data.

There is growing interest in developing tools and methods for the surveillance of chronic rheumatic diseases, using existing resources such as administrative health databases. To illustrate how this might work, we used population-based administrative data to estimate and compare the prevalence of systemic autoimmune rheumatic diseases (SARDs) across three Canadian provinces, assessing for regional differences and the effects of demographic factors. Cases of SARDs (systemic lupus erythematosus, scleroderma, primary Sjogren's, polymyositis/dermatomyositis) were ascertained from provincial physician billing and hospitalization data. We combined information from three case definitions, using hierarchical Bayesian latent class regression models that account for the imperfect nature of each case definition. Using methods that account for the imperfect nature of both billing and hospitalization databases, we estimated the over-all prevalence of SARDs to be approximately 2-3 cases per 1,000 residents. Stratified prevalence estimates suggested similar demographic trends across provinces (i.e. greater prevalence in females-versus-males, and in persons of older age). The prevalence in older females approached or exceeded 1 in 100, which may reflect the high burden of primary Sjogren's syndrome in this group. Adjusting for demographics, there was a greater prevalence in urban-versus-rural settings. In our work, prevalence estimates had good face validity and provided useful information about potential regional and demographic variations. Our results suggest that surveillance of some rheumatic diseases using administrative data may indeed be feasible. Our work highlights the usefulness of using multiple data sources, adjusting for the error in each.

Polymyalgia rheumatica prevalence in a population-based sample.

OBJECTIVE: To determine polymyalgia rheumatica (PMR) prevalence using population-based administrative data, and to estimate the error associated with case ascertainment approaches when using these databases. METHODS: Cases were ascertained using physician billing and hospitalization data from the province of Manitoba (population 1.1 million). Focusing on the population age >/=45 years, we compared 3 different case definition algorithms and also used statistical methods that accounted for imperfect case ascertainment to estimate the prevalence and the properties of the ascertainment algorithms. A hierarchical Bayesian latent class regression model was developed that also allowed us to assess differences across patient demographics (sex and region of residence). RESULTS: Using methods that account for the imperfect nature of both billing and hospitalization databases, we estimated the prevalence of PMR in women age >/=45 years to be lower in urban areas (754.5 cases/100,000; 95% credible interval [95% CrI] 674.1-850.3) compared with rural areas (1,004 cases/100,000; 95% CrI 886.3-1,143). This regional trend was also seen in men age >/=45 years, where the prevalence was estimated at 273.6 cases/100,000 (95% CrI 219.8-347.6) in urban areas and 380.7 cases/100,000 (95% CrI 311.3-468.1) in rural areas. Billing data appeared more sensitive in ascertaining cases than hospitalization data, and a large proportion of diagnoses was made by physicians other than rheumatologists. CONCLUSION: These data suggest a higher prevalence of PMR in rural versus urban regions. Our approach demonstrates the usefulness of methods that adjust for the imperfect nature of multiple information sources, which also allow for estimation of the sensitivity of different case ascertainment approaches.

Application of a nomogram for exercise capacity in women with systemic lupus erythematosus.

The aim of this study is to examine the exercise capacity in women with systemic lupus erythematosus (SLE). Women with SLE underwent exercise testing; their performance was compared to nomogram predictions. We assessed the potential effects of disease activity and cumulative damage on exercise capacity. We evaluated 52 female SLE patients aged >35 years. The mean workload achieved was somewhat higher than the nomogram predictions. However, over one fifth of the women performed at a very poor level, which in the general population is associated with a twofold increased risk of cardiovascular disease. Compared to other subjects, participants who did poorly tended toward higher disease activity, higher body mass index, and greater smoking prevalence, although the results were not definitive. Exercise testing may be used to identify a subpopulation of lupus patients with a low level of fitness. Extrapolating from general population data, these individuals are likely at particular risk for cardiovascular disease and may, therefore, benefit the most from aggressive cardiovascular risk factor reduction.

Scleroderma prevalence: demographic variations in a population-based sample.

OBJECTIVE: To estimate the prevalence of systemic sclerosis (SSc) using population-based administrative data, and to assess the sensitivity of case ascertainment approaches. METHODS: We ascertained SSc cases from Quebec physician billing and hospitalization databases (covering approximately 7.5 million individuals). Three case definition algorithms were compared, and statistical methods accounting for imperfect case ascertainment were used to estimate SSc prevalence and case ascertainment sensitivity. A hierarchical Bayesian latent class regression model that accounted for possible between-test dependence conditional on disease status estimated the effect of patient characteristics on SSc prevalence and the sensitivity of the 3 ascertainment algorithms. RESULTS: Accounting for error inherent in both the billing and the hospitalization data, we estimated SSc prevalence in 2003 at 74.4 cases per 100,000 women (95% credible interval [95% CrI] 69.3-79.7) and 13.3 cases per 100,000 men (95% CrI 11.1-16.1). Prevalence was higher for older individuals, particularly in urban women (161.2 cases per 100,000, 95% CrI 148.6-175.0). Prevalence was lowest in young men (in rural areas, as low as 2.8 cases per 100,000, 95% CrI 1.4-4.8). In general, no single algorithm was very sensitive, with point estimates for sensitivity ranging from 20-73%. CONCLUSION: We found marked differences in SSc prevalence according to age, sex, and region. In general, no single case ascertainment approach was very sensitive for SSc. Therefore, using data from multiple sources, with adjustment for the imperfect nature of each, is an important strategy in population-based studies of SSc and similar conditions.

Estimating the prevalence of polymyositis and dermatomyositis from administrative data: age, sex and regional differences.

OBJECTIVE: To estimate the prevalence of polymyositis and dermatomyositis using population-based administrative data, the sensitivity of case ascertainment approaches and patient demographics and these parameters. METHODS: Cases were ascertained from Quebec physician billing and hospitalisation databases (approximately 7.5 million beneficiaries). Three different case definition algorithms were compared, and statistical methods were also used that account for imperfect case ascertainment, to generate estimates of disease prevalence and case ascertainment sensitivity. A hierarchical Bayesian latent class regression model was developed to assess patient characteristics with respect to these parameter estimates. RESULTS: Using methods that account for the imperfect nature of both billing and hospitalisation databases, the 2003 prevalence of polymyositis and dermatomyositis was estimated to be 21.5/100,000 (95% credible interval (CrI) 19.4 to 23.9). Prevalence was higher for women and for older individuals, with a tendency for higher prevalence in urban areas. Prevalence estimates were lowest in young rural men (2.7/100,000, 95% CrI 1.6 to 4.1) and highest in older urban women (70/100,000, 95% CrI 61.3 to 79.3). Sensitivity of case ascertainment tended to be lower for older versus younger individuals, particularly for rheumatology billing data. Billing data appeared more sensitive in ascertaining cases in urban (vs rural) regions, whereas hospitalisation data seemed most useful in rural areas. CONCLUSIONS: Marked variations were found in the prevalence of polymyositis and dermatomyositis according to age, sex and region. These methods allow adjustment for the imperfect nature of multiple data sources and estimation of the sensitivity of different case ascertainment approaches.

Fertility preservation treatment for young women with autoimmune diseases facing treatment with gonadotoxic agents.

OBJECTIVE: To describe a case series of seven women with SLE and other systemic autoimmune rheumatic diseases (SARDs) who required cyclophosphamide therapy and underwent fertility preservation treatments. METHODS: Of the seven patients reported here, five women had SLE with nephritis, the sixth had immune thrombocytopenia purpura (ITP) and the seventh had microscopic polyangiitis (MPA) with renal involvement. All women were nulliparous and younger than 35 yrs. RESULTS: Patients with SLE underwent in vitro maturation (IVM) of immature oocytes aspirated during a natural menstrual cycle followed by vitrification of the matured oocytes if a male partner was not available, or vitrification of embryos if one was available. The patient with ITP and the patient with MPA underwent gonadotropin ovarian stimulation followed by oocyte or embryo vitrification. All women completed fertility preservation treatment successfully and mature oocytes or embryos (36 and 13, respectively) were vitrified. No complications were associated with this treatment and cytotoxic therapy was initiated as scheduled in all cases. CONCLUSIONS: Oocyte or embryo cryopreservation should be considered for fertility preservation in young women with SARDs who face imminent gonadotoxic treatment. In patients, where gonadotropin ovarian stimulation is deemed unsafe, IVM of immature oocytes, aspirated during a natural menstrual cycle, followed by vitrification or fertilization of the mature oocytes, seems to be safe and feasible. For patients in whom hormonal ovarian stimulation is not contraindicated, this method may be considered depending on the urgency to start cytotoxic therapy.

A population-based assessment of systemic lupus erythematosus incidence and prevalence--results and implications of using administrative data for epidemiological studies.

OBJECTIVES: To estimate (i) systemic lupus erythematosus (SLE) incidence and prevalence using multiple sources of population-based administrative data; (ii) the sensitivity and specificity of case ascertainment methods; and (iii) variation in performance of each ascertainment approach, according to patient and physician characteristics. METHODS: We examined the physician billing and hospitalization databases of the province of Quebec (1994-2003) covering all health care beneficiaries (approximately 7.5 million). We compared various approaches to ascertain SLE cases, using information from each database separately or combining sources; we then estimated the sensitivity and specificity of these alternative approaches. We used regression models to determine if sensitivity was independently influenced by patient or physician characteristics. RESULTS: Using billing data, we calculated SLE incidence at 3.0/100,000 person-years [95% confidence interval (CI) 2.6-3.4]; prevalence was 32.8/100,000 persons, in 2003. Results were similar using hospitalization data. However, only a proportion of prevalent cases were identified as having SLE by both methods. Combining cases from billing and hospitalization data, we found a prevalence of 51/100,000 in 2003. Our latent class regression model estimated a prevalence of 44.7/100,000 (95% CI 37.4-54.7). We found high specificity for SLE diagnoses across all strategies and data sources; sensitivity ranged from 42.1% to 67.6%, and was independently influenced by both patient and physician characteristics. CONCLUSIONS: In observational studies, particularly with administrative databases, SLE incidence and prevalence estimates differ considerably, according to the approach for case ascertainment. In the absence of gold standards, statistical modelling can provide sensitivity and specificity estimates for different approaches.

Headache, Raynaud's syndrome and serotonin receptor agonists in systemic lupus erythematosus.

There are potential concerns regarding serotonin receptor agonists in SLE patients with migraine, particularly patients with concomitant Raynaud's syndrome. We estimated the prevalence of lupus-related headache and Raynaud's syndrome in the Montreal General Hospital SLE clinic cohort and evaluated the relationship between these two variables in multivariable logistic regression models, controlling for age, sex, race, SLE duration and the presence of lupus anticoagulant and antibodies to cardiolipin and beta2 glycoprotein I. We also assessed, through chart review in those individuals with both Raynaud's syndrome and migraine, a history of serotonin receptor agonist use, and any associated worsening vasospasm. Based on Systemic Lupus Activity Measure (SLAM) scores, the cumulative incidence of lupus-related headache in our sample (n = 391) was 46.1%; the prevalence of Raynaud's syndrome was 49.4%. The adjusted odds ratio (OR) for lupus-related headache and Raynaud's syndrome was 1.7 (95% CI 1.1, 2.5). In addition, there was a strong independent relationship between headache and anti-beta2 glycoprotein I antibodies (adjusted OR 5.6 [95% CI 1.8, 17.0]). The data from our chart review suggest that careful use of serotonin receptor agonists in patients with both Raynaud's syndrome and migraines may be undertaken, although caution would necessitate that these agents not be used in individuals with very severe Raynaud's (eg, digital ulcerations, and so on).

A comparison of damage accrual across different calendar periods in systemic lupus erythematosus patients.

Therapeutic approaches in systemic lupus erythematosus (SLE) have evolved over the last few decades, but their impact on prevention of organ damage is unknown. The objective of this study was to compare new cumulative damage in SLE patients across different calendar periods. Patients from a large SLE cohort were divided into two subcohorts; the first diagnosed and followed between 1978 and 1988 (cohort #1, n=100) and the second between 1989 and 1999 (cohort #2, n=51). Initial Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) scores, and changes in scores over the observation intervals, were compared for the two groups. Logistic regression estimated adjusted odds ratios (OR) comparing damage accrual between the two cohorts. Medication exposures were noted. Baseline characteristics were similar between the two groups. At first assessment, the adjusted OR for a SLICC/ACR DI score > or =1 was 1.79 (95% CI 0.82, 3.88) for cohort #1 versus cohort #2. At the end of the observation interval, the adjusted OR for a SLICC/ACR DI score > or =1 was 1.22 (0.58, 2.55) for cohort #1 versus cohort #2. The adjusted OR for accruing damage over the observation interval in cohort #1 versus cohort #2 was 0.94 (0.39, 2.44). Increased medication exposure was evident for cohort #2 compared to cohort #1. Despite increased therapeutic measures used for patients in more recent periods, our data do not establish a clear difference in damage accrual. This emphasizes the need for strategies to effectively treat lupus-specific manifestations, while minimizing side effects and comorbidities.

Osteoporosis in systemic lupus erythematosus: factors associated with referral for bone mineral density studies, prevalence of osteoporosis and factors associated with reduced bone density.

The purpose of this study was to evaluate the clinical characteristics of women with systemic lupus erythematosus (SLE) sent for a dual energy X-ray absorptiometry (DEXA) study, and to analyse the factors associated with a lower bone mineral density in these patients. Women with SLE who had a DEXA done between 1 January 1995 and 31 December 2000 were compared with those who did not have DEXA scans performed. SLE patients with osteoporosis (OP) were compared with those with a normal bone density. Of 516 women with SLE, 205 had a DEXA done. These patients had more traditional risk factors for osteoporosis, higher lupus disease activity, renal involvement, increased damage, higher mean steroid dose, increased use of immunosuppressants and occurrence of avascular necrosis. Of the 205 patients with DEXA, 18% had osteoporosis, 48.8% had osteopenia and 33.2% had normal bone mineral density. The two statistically significant predictors of a low bone density were a higher age at time of DEXA (P = 0.0003) and a higher SDI score (P = 0.0019). Osteoporosis is a significant comorbidity in SLE. Lupus patients referred for a DEXA have more traditional risk factors and use more corticosteroids. The main factors associated with a low bone density were however found to be age and increased damage. Interestingly, disease activity and corticosteroid use were not associated with osteoporosis in this study which may suggest other potential causes such as decreased physical activity associated with damage.