Lupus nephritis and socioeconomic status: Findings from the Southern California lupus registry.

OBJECTIVE: Lupus nephritis (LN) is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). Despite multiple studies addressing healthcare disparities, disparate outcomes in LN persist. We investigate herein the association between socioeconomic status (SES) and LN as well as the association between SES, SLE disease activity index (SLEDAI), and treatment response. METHODS: Patients were selected from the Southern California Lupus Registry (SCOLR), a registry enrolling all-comers with SLE. Analysis was completed on individuals with public vs. private insurance. Insurance and ethnicity were used as surrogate variables for SES, and we tested differences in means. RESULTS: After adjusting for age and sex, public insurance was independently associated with the prevalence of LN. Analysis of 35 patients revealed greater proteinuria and mean SLEDAI in patients with public insurance at baseline and 6 months. Baseline, 6-, and 12-month SLEDAI means were significantly lower in Asian/Pacific Islanders (PI) compared to others. While non-Hispanic Whites demonstrated mean SLEDAI improvement over 6 months, Asians/PI, Blacks, and Hispanics demonstrated worsened disease activity on average. CONCLUSION: Low SES, when defined by insurance, is associated with greater adverse outcomes in SLE. This is the first regional study that compares differences in treatment response in LN patients with low SES as well as association of SES with long-term outcomes in SLE and LN in southern California.

The Southern California Lupus Registry: I. Baseline characteristics of lupus patients in uncharted territory.

OBJECTIVE: This study aimed to determine the baseline characteristics of a multi-ethnic systemic lupus erythematosus (SLE) cohort in Southern California established with the intent of addressing regional health inequity. METHODS: Patients ≥18 years of age with SLE per the Systemic Lupus International Collaborating Clinics (SLICC) criteria were recruited into the Southern California Lupus Registry (SCOLR) if they resided in San Bernardino and Riverside counties in California. Individuals were categorized according to their stated ethnicity as non-Hispanic White, Hispanic, Black, or Asian. Descriptive statistics were utilized for analysis. Predictors of renal disease were assessed by binomial regression. RESULTS: The SCOLR cohort comprised 162 patients: 57 non-Hispanic White, 58 Hispanic, 17 Asian, and 30 Black. A difference in the rate of renal involvement and SLE duration was found among the four ethnic groups. Renal involvement was significantly higher in Hispanics compared with non-Hispanic Whites. CONCLUSION: In line with other cohorts, this study shows greater renal involvement in Hispanics than non-Hispanic Whites, demonstrating a need for more aggressive screening and early intervention to improve long-term outcomes. As a multi-ethnic SLE cohort, the SCOLR serves as a foundation for longitudinal studies addressing health inequity in this region.

Cardiovascular disease and bone health in aging female rheumatic disease populations: A review.

Rheumatic diseases cover a wide spectrum of conditions, including primary and secondary degenerative joint diseases and autoimmune inflammatory rheumatic diseases. The risks of cardiovascular disease and osteoporosis and resultant fractures in aging female rheumatic disease populations, especially those with autoimmune rheumatic diseases, are increased. Changes in the immune system in aging populations need to be considered especially among patients with autoimmune rheumatic diseases. Immunosenescence is closely aligned to reduced adaptive immunity and increased non-specific innate immunity leading to chronic inflammation of inflammaging. The effective use of disease-modifying antirheumatic drugs to control autoimmune rheumatic diseases may also mitigate factors leading to cardiovascular disease and osteoporosis. Rheumatic diseases, which largely manifest as arthritis, predispose patients to premature joint degeneration and poor bone health and therefore have a higher risk of developing end-stage arthritis requiring joint arthroplasties sooner or more often than other patients without rheumatic disease.

Chemosis as an Initial Presentation of Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) can present in a multitude of ways, which can be confounding and misleading for a clinician. Chemosis as an initial presentation is rare and has only been documented on a few case reports. However, when present, simultaneous involvement of other organs is likely. We present a previously healthy 29-year-old male who presented with severe bilateral chemosis and was subsequently diagnosed with SLE and antiphospholipid syndrome. Complications included multiple acute cerebral infarcts, lupus psychosis, lupus pleuritis, and lupus nephritis. The patient recovered well with appropriate treatment and chemosis ultimately resolved. Recognizing chemosis as an initial presentation of SLE is vital for appropriate evaluation and timely treatment to prevent disease progression.

Barriers to Enrollment in Rheumatology Research: Who, What, Where, When, and Why?

Despite the evidence that complicated rheumatic diseases are more prevalent and severe in ethnic minorities, rheumatology research is afflicted with inadequate patient representation. It is lacking in ethnic and socioeconomic diversity. The objective of this study is to identify barriers to enrollment in rheumatology research and propose possible solutions to overcome these barriers. In this study, 184 patients from two rheumatology clinics (Safety Net clinic, a university-based clinic) were surveyed for concerns regarding participation in clinical research. Patients were asked to rank their top five of eight concerns. Data were then stratified by self-reported ethnicity and clinic site to determine ranking differences in both groups. Fear of risks associated with clinical research was ranked as the primary barrier in all ethnicities. More non-Hispanic Whites (NHW) (24.4%) ranked work responsibilities as a primary barrier compared to Hispanics (10%). Fear of discovering a serious illness as a primary barrier was more frequent at the Safety Net clinic (25%) compared to the university-based clinic (6.3%) and ranked more frequently in the top five in Hispanics compared to NHW. Fears associated with research risks, work responsibilities, and fear of discovering a serious illness were the top-ranked barriers to enrollment in research among patients. However, differences in rankings between ethnicities and clinic sites were identified. This sheds light on the importance of health literacy and the responsibility of researchers in addressing gaps in communication while acknowledging potential cultural components that warrant further investigation.

Vitamin D and Lupus: Are we doing enough?

The aim of this study is to identify rheumatology practice care gaps in evaluating for vitamin D deficiency in systemic lupus erythematosus (SLE), as well as adherence to vitamin D replacement in SLE patients currently on corticosteroid therapy. Data for this study were collected from the Southern California Lupus Registry in addition to data extraction from medical health records. Evaluation of serum vitamin D level within 6 months of patient encounter, current or prior use of systemic corticosteroids, and vitamin D replacement in patients receiving corticosteroid therapy were noted. Vitamin D deficiency was defined as serum 25(OH)D3 less than 30 ng/ml. Of 182 patients in the cohort, data were available for 176. Evaluation of vitamin D deficiency was noted in 49 patients (28%), 27 (55%) of whom had abnormal values. Current corticosteroid use was noted in 56 (32%) patients and prior use in 73 (41%). Vitamin D replacement was prescribed to 30 (54%) patients with current corticosteroid use. In an academic rheumatology clinic, we have identified underevaluation for vitamin D deficiency in SLE patients despite increasing awareness of its contribution to disease activity. Further, routine supplementation of vitamin D is particularly lacking in individuals receiving systemic corticosteroids. This presents a practical opportunity for improvement in SLE clinical care.

Underserved Communities: Enhancing Care with Graduate Medical Education.

The shortage of health care professionals is projected to worsen in the coming years. This is particularly concerning in underserved areas that are fraught with disparities in disease outcomes and life expectancy, quality of life, and health care access. The onus is on medical education institutions to train students to serve vulnerable communities to improve both health care access and the quality of medical school education. When health disparities are formally included in medical education curricula and the culture of medical education shifts to a community-based learning approach, patients and health care providers alike will reap the benefits.

Five-Year Follow-Up of Coronary Microvascular Dysfunction and Coronary Artery Disease in Systemic Lupus Erythematosus: Results From a Community-Based Lupus Cohort.

OBJECTIVE: The present study was undertaken to investigate prospective change in the prevalence of coronary microvascular dysfunction (CMD) and obstructive coronary artery disease (CAD) in a cohort of subjects with systemic lupus erythematosus (SLE) initially evaluated for anginal chest pain (CP). Prior work documented a relatively high prevalence of CMD in the absence of obstructive CAD in subjects with SLE. METHODS: Twenty female SLE subjects with CP who underwent stress cardiac magnetic resonance imaging (CMRI) and coronary computed tomography angiography at baseline were reevaluated at 5 years. RESULTS: Seventeen subjects (85%) were available and reenrolled, of which 11 (65%) had persistent CP at follow-up. Fourteen subjects had complete follow-up CMRI, of which 36% (n = 5) demonstrated CMD at follow-up. Further, 25% (1 of 4) of the originally abnormal myocardial perfusion reserve index (MPRI) findings at baseline were lower at follow-up, while 2 additional abnormal MPRI findings at follow-up were noted in previously normal MPRI results. The prevalence of CMD and nonobstructive/obstructive CAD both was unchanged between baseline and follow-up, respectively (both P values not significant). During follow-up, 33% of subjects (5 of 15) had adverse cardiac outcomes, including pericarditis, unstable angina, or intracranial aneurysm clipping procedure. CONCLUSION: At the 5-year follow-up of SLE subjects with CP who were evaluated at baseline and follow-up, a majority had persistent CP, and nearly one-half had similar or worse myocardial perfusion consistent with CMD without obstructive CAD. These findings propose an alternative explanation for CP in SLE subjects compared to the more common SLE-related accelerated obstructive CAD accounting for CP and adverse outcomes. These findings support further studies of CMD as an etiology for cardiac morbidity and mortality in SLE.

The impact of obesity on SLE disease activity: findings from the Southern California Lupus Registry (SCOLR).

The role of obesity in systemic lupus erythematosus (SLE) remains controversial. Studies have linked adiposity with a heightened risk of clinical complications including neurocognitive decline, renal impairment, dampened physical activity, and depressed quality of life-but not disease activity. We aimed to reexamine whether obesity in SLE patients independently associates with higher disease activity. Adult patients with SLE were recruited from the longitudinal, multi-ethnic Southern California Lupus Registry (SCOLR). Disease status was ascertained by calculating SLE Disease Activity Index (SLEDAI), which was then statistically analyzed for association with increased body mass index (BMI) by univariable and multivariable regression analyses. One hundred and thirty-seven patients were included in the study; 37% were obese (BMI ≥ 30 kg/m2). Obesity was significantly associated with SLEDAI (P = 0.026) and current steroid use (P = 0.029). Multivariable regression analysis demonstrated that obesity remained independently associated with lupus activity (OR 2.335, P = 0.026). In a representative sample of patients with SLE, obesity independently associated with worse SLE disease activity. Obesity may therefore be an important target for improving SLE outcomes.

Atypical manifestations of sarcoidosis in a Hispanic male.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can present with nonspecific features, often resulting in delayed diagnosis. The diagnosis requires the demonstration of non-caseating granulomas on biopsy. While the prevalence of sarcoidosis in the USA is rare, the disease is rarer yet in Hispanics. It is for this reason that we report herein the case of a Hispanic gentleman with a unique clinical manifestations of sarcoidosis. With what began as a two-month history of joint pain and skin rash, this 55-year-old man was hospitalized with multiple joint pain, weight loss, fatigue and a pruritic rash with leonine facies in the setting of anemia, leukopenia, hypercalcemia, elevated serum creatinine, and urine Bence-Jones proteinuria. CT imaging of the chest was nonspecific, but skin biopsy revealed non-caseating granulomatous disease. After completing an infectious and malignancy evaluation, the patient was diagnosed with sarcoidosis, which was treated successfully with low-dose steroid therapy.

A case report of heart transplant for ischaemic cardiomyopathy from lupus coronary vasculitis.

BACKGROUND: Coronary vasculitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE). CASE SUMMARY: A 23-year-old woman with SLE presented with typical angina and worsening dyspnoea on exertion. Coronary angiography revealed severe triple vessel disease with a 'string of beads' appearance classic for coronary vasculitis. Transthoracic echocardiogram revealed ejection fraction of 25-30% with a severely hypokinetic distal septum and distal anterior wall and an akinetic apical wall. Despite vasculitis treatment with cyclophosphamide and pulse-dose steroids, her coronary vasculitis did not improve. She was refractory to anti-anginal and guideline-directed medical therapy for heart failure and successfully underwent orthotopic heart transplant (OHT). DISCUSSION: This is the first reported case of OHT in the case of SLE coronary vasculitis. Chronic SLE coronary vasculitis is caused by lymphocyic infiltration leading to inflammation and fibrosis of the major epicardial coronary arteries but can be successfully managed with OHT when refractory to medical SLE and heart failure therapies. It can affect patients of all ages with SLE, emphasizing the importance of thorough history taking and clinical evaluation in young patients presenting with cardiac symptoms to establish an appropriate diagnosis and treatment plan.

Dilemma in management: a patient with active systemic lupus erythematosus presenting with pulmonary cavitary lesion.

Pulmonary manifestations of systemic lupus erythematosus (SLE) include, but are not limited to, pneumonia, interstitial pneumonitis, atelectasis and pleural effusion. Cavitary lung lesions are rarely associated with SLE. We present herein the case of a female patient with SLE and lupus nephritis who presented to the hospital with respiratory failure, rash and arthralgias. She was found to have a cavitary lung lesion most concerning for infection. However, despite an extensive inpatient antibiotic course, her symptoms persisted. After a collaborative effort between the primary team, pulmonology, infectious disease and rheumatology, she was placed on systemic glucocorticoid therapy, which resolved not only her respiratory failure, but also her cavitary lung lesion on subsequent follow-up with imaging. The dilemma of management in such cases will be discussed in addition to a review of previously reported cases.

Sarcoidosis and Systemic Sclerosis: Strange Bedfellows.

Coexistence of systemic sclerosis and sarcoidosis is rare. Both have predominant lung manifestations, each with distinctive features on computed tomography (CT) of the chest. We present herein a 52-year-old male with limited systemic sclerosis manifested primarily by sclerodactyly and subsequently by shortness of breath. A series of CT scans of the chest were reviewed. Initial CT chest one year prior to sclerodactyly onset revealed bilateral hilar and right paratracheal, prevascular, and subcarinal adenopathy. Five-year follow-up demonstrated thin-walled cysts, mediastinal lymphadenopathy, and nonspecific nodules. Due to progression of dyspnea, follow-up CT chest after one year again demonstrated multiple cysts with peripheral nodularity and subpleural nodules, but no longer with hilar or mediastinal adenopathy. Diagnostic open lung biopsy was significant for noncaseating granulomas suggestive of sarcoidosis. This is the first known case of a patient with systemic sclerosis diagnosed with sarcoidosis through lung biopsy without radiographic evidence of hilar or mediastinal lymphadenopathy at the time of biopsy. A review of cases of concomitant sarcoidosis and systemic sclerosis is discussed, including the pathophysiology of each disease with shared pathways leading to the development of both conditions in one patient.

Chest pain in lupus patients: the emergency department experience.

Heart disease, a major cause of morbidity and mortality in SLE patients, often manifests as chest pain (CP). Our goal was to understand the prevalence and outcome of CP presentations for SLE patients in the emergency department (ED). Billing records of patients who presented to Cedars-Sinai Medical Center ED with ICD-9 codes for SLE and secondary ICD-9 codes for CP (786.50-786.59) between March 2009 and October 2013 were reviewed. Two study groups were formed: discharge from ED versus hospital admission. Visits were evaluated for basic cardiac work-up with an electrocardiogram (EKG) and cardiac enzymes; hospital admissions were evaluated for CP etiology and discharge diagnoses. Of 2675 ED visits with ICD-9 codes for SLE, 397 visits had secondary codes for CP (15%); 173 were discharged and 224 became hospital admissions. While 92% of admissions had basic cardiac work-up, over 50% had chest pain attributed to non-cardiac causes. Only 7.2% had a discharge diagnosis related to cardiovascular disease. Fifteen percent of all SLE coded patients had complaints of CP, a figure higher than the national average for non-SLE CP (10%). There is a majority of non-cardiac diagnoses given to SLE patients at discharge. CP is likely to be a window of opportunity to address the known cardiac morbidity and mortality in SLE patients perhaps at an early stage of development of this complication. Our study strengthens the need for more investigations to assess the etiology of CP in this population.