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Purposes: To describe a cohort of patients who presented with interstitial lung disease (ILD) of unknown cause, features of primary Sjögren syndrome (pSS), and a positive minor salivary gland biopsy (MSGB).Methods: Thirty-eight patients with ILD evaluated at our center underwent an MSGB to confirm a diagnosis of pSS. All of the samples were reviewed by pathologists experienced in the evaluation of salivary gland histology. We defined a positive MSGB finding as a lymphocyte focus score of >1.Results: At presentation, all patients had ILD, and symptoms of cough and dyspnea. None had a definable connective tissue disease (CTD) or known cause for their ILD. Thirteen patients (34%) had positive MSGB findings. Of these, the median age was 61 years (age range, 33 to 75 years); 7 patients (54%) were women; 8 patients (62%) had a smoking history; and 10 patients (77%) had sicca symptoms. In all patients, a thoracic high-resolution CT scan evaluation demonstrated bibasilar, peripheral-predominant, ground-glass, and reticular opacities. Four patients (31%) were negative for both antinuclear autoantibody (ANA) and rheumatoid factor (RF) autoantibody, and three patients (23%) were negative for ANA, RF, Sjögren syndrome (SS)-A, and SS-B autoantibodies. No patients experienced any complications from the MSGB. The identification of underlying pSS did not affect the management of ILD in these patients.Conclusions: Confirming a diagnosis of pSS-related ILD by performing MSGB allows for a more precise CTD classification. This study provides evidence that CTD may exist subclinically, and longitudinal studies are needed to determine whether identifying occult CTD impacts on management, longitudinal changes in lung function, or survival. [ABSTRACT FROM AUTHOR]

Purposes: To evaluate the pathologic patterns, clinical features, and survival among subjects with scleroderma (ie, systemic sclerosis [SSc]) and clinically significant interstitial lung disease (ILD) evaluated at an ILD center.Methods: Retrospective cohort study of all SSc patients who had been referred for further evaluation of ILD and had undergone surgical lung biopsy. Clinical data were abstracted by review of the medical record, and lung biopsy specimens were reviewed and classified according to current pathologic criteria.Results: All patients presented with significant respiratory symptoms. Twenty-two of 27 subjects had surgical lung biopsy-proven ILD, and 5 subjects had miscellaneous non-ILD patterns. Of those subjects with ILD, 64% (14 of 22 subjects) had a nonspecific interstitial pneumonia (NSIP) pathologic pattern (fibrotic NSIP, 13 subjects; cellular NSIP, 1 subject), and 36% (8 of 22 subjects) had the usual interstitial pneumonia (UIP) pattern. Subjects with NSIP were younger (median age, 42 vs 58 years, respectively; p = 0.003), but no differences were noted in pulmonary physiology (FVC: NSIP group, 52% predicted; UIP group, 65% predicted; p = 0.22; diffusing capacity of the lung for carbon monoxide: NSIP group, 40% predicted; UIP group, 42% predicted; p = 1.0). All patients had limited skin involvement. The Scl-70 antibody was absent among those assessed (NSIP group, 0 of 10 subjects; UIP group, 0 of 7 subjects). All patients were treated with cytotoxic therapy. The median survival time for those with NSIP was 15.3 years (5,596 days) compared with 3 years (1,084 days) for those with UIP (p = 0.07 [log-rank test]).Conclusions: In SSc patients with limited cutaneous disease and clinically significant ILD, fibrotic NSIP and UIP are the predominant pathologic patterns. Those with the UIP pattern of disease had a trend toward shorter survival time. [ABSTRACT FROM AUTHOR]

Females are more susceptible than males to many autoimmune diseases. The processes causing this phenomenon are incompletely understood. Here, we demonstrate that aged female mice acquire a previously uncharacterized population of B cells that we call age-associated B cells (ABCs) and that these cells express integrin α(X) chain (CD11c). This unexpected population also appears in young lupus-prone mice. On stimulation, CD11c(+) B cells, both from autoimmune-prone and healthy strains of mice, secrete autoantibodies, and depletion of these cells in vivo leads to reduction of autoreactive antibodies, suggesting that the cells might have a direct role in the development of autoimmunity. We have explored factors that contribute to appearance of ABCs and demonstrated that signaling through Toll-like receptor 7 is crucial for development of this B cell population. We were able to detect a similar population of B cells in the peripheral blood of some elderly women with autoimmune disease, suggesting that there may be parallels between the creation of ABC-like cells between mice and humans.

Objective: Disease activity and medication use can complicate pregnancies in patients with systemic lupus erythematosus (SLE). We therefore examined contraceptive counseling and use among women in the University of California, San Francisco Lupus Outcomes Study., Methods: In 2008, we queried participants regarding their pregnancy intentions, contraceptive use, and receipt of contraceptive counseling. Premenopausal women age <45 years who were sexually active with men were considered at risk of pregnancy. We compared self-reported rates of contraceptive counseling and use stratified by treatment with teratogenic medications and by history of thrombosis or antiphospholipid antibodies (aPL), using chi-square tests. We used logistic regression models to examine predictors of contraceptive counseling and use., Results: Among 206 women, 86 were at risk for unplanned pregnancy. Most (59%) had not received contraceptive counseling in the last year, 22% reported inconsistent contraceptive use, and 53% depended solely on barrier methods. Intrauterine device contraceptives (IUDs) were used by 13%. Women using potentially teratogenic medications were no more likely to have received contraceptive counseling, to have used contraception consistently, or to have used more effective contraceptives. A history of thrombosis or aPL did not account for low rates of hormonal methods. Four women with a history of thrombosis or aPL were using estrogen-containing contraceptives., Conclusion: Most women at risk for unplanned pregnancy reported no contraceptive counseling in the past year, despite common use of potentially teratogenic medications. Many relied upon contraceptive methods with high failure rates; few used IUDs. Some were inappropriately using estrogen-containing contraceptives. These findings suggest the need to improve the provision of contraceptive services to women with SLE., (Copyright © 2011 by the American College of Rheumatology.)

Objective: Systemic lupus erythematosus (SLE) primarily affects women of reproductive age. Here we summarize the scientific evidence supporting recently developed quality indicators (QIs) pertaining to reproductive health., Methods: We used a modification of the RAND/UCLA Appropriateness Method to develop QIs for SLE. We performed systematic reviews of the literature pertaining to each proposed indicator. Three indicators focusing on reproductive health were included in the final set. Relevant literature was presented to an expert panel, who rated the validity and feasibility of the indicators., Results: Three QIs were rated as valid and feasible. These indicators specifically address laboratory testing during pregnancy in SLE, the treatment of antiphospholipid antibody syndrome, and counseling for drugs with teratogenic potential., Conclusion: We used a rigorous method to develop reproductive health QIs for SLE. In the future, these indicators can be used in the assessment and delivery of care to patients with SLE., (Copyright © 2011 by the American College of Rheumatology.)

Objective: To describe characteristics of systemic lupus erythematosus (SLE) patients who are frequent users of the emergency department and to identify predictors of frequent emergency department use., Methods: Data for this study were derived from the University of California, San Francisco Lupus Outcomes Study, a large cohort of persons with SLE who undergo annual structured interviews. Participants were categorized into 1 of 3 levels of emergency department utilization: nonusers (no visits in the preceding year), occasional users, (1-2 visits), and frequent users (> or =3 visits). We compared characteristics of the 3 groups and determined predictors of frequent emergency department use (> or =3 visits) using multivariate logistic regression, adjusting for a variety of potential confounding covariates., Results: Of 807 study participants, 499 (62%) had no emergency department visits; 230 (28%) had occasional emergency department visits (1-2 visits); and 78 (10%) had frequent (> or =3 visits) emergency department visits. Frequent users were younger, less likely to be employed, and less likely to have completed college. They also had greater disease activity, worse general health status, and more depressive symptoms. Frequent emergency department users were more likely to have Medicaid as their principal insurance. In multivariate logistic regression, older age predicted a lower likelihood of frequent emergency department visits, whereas greater disease activity and having Medicaid insurance predicted a higher likelihood of frequent emergency department visits., Conclusion: In persons with SLE, greater disease activity and Medicaid insurance are associated with more frequent emergency department use.

Introduction: Cancer and infections are leading causes of mortality in systemic lupus erythematosus (SLE) after diseases of the circulatory system, and therefore preventing these complications is important. In this study, we examined two categories of preventive services in SLE: cancer surveillance (cervical, breast, and colon) and immunizations (influenza and pneumococcal). We compared the receipt of these services in SLE to the general population, and identified subgroups of patients who were less likely to receive these services., Methods: We compared preventive services reported by insured women with SLE enrolled in the University of California, San Francisco Lupus Outcomes Study (n=685) to two representative samples derived from a statewide health interview survey, a general population sample (n=18,013) and a sample with non-rheumatic chronic conditions (n=4,515). In addition, using data from the cohort in both men and women (n=742), we applied multivariate regression analyses to determine whether characteristics of individuals (for example, sociodemographic and disease factors), health systems (for example, number of visits, involvement of generalists or rheumatologists in care, type of health insurance) or neighborhoods (neighborhood poverty) influenced the receipt of services., Results: The receipt of preventive care in SLE was similar to both comparison samples. For cancer surveillance, 70% of eligible respondents reported receipt of cervical cancer screening and mammography, and 62% reported colon cancer screening. For immunizations, 59% of eligible respondents reported influenza immunization, and 60% reported pneumococcal immunization. In multivariate regression analyses, several factors were associated with a lower likelihood of receiving preventive services, including younger age and lower educational attainment. We did not observe any effects by neighborhood poverty. A higher number of physician visits and involvement of generalist providers in care was associated with a higher likelihood of receiving most services., Conclusions: Although receipt of cancer screening procedures and immunizations in our cohort was comparable to the general population, we observed significant variability by sociodemographic factors such as age and educational attainment. Further research is needed to identify the physician, patient or health system factors contributing to this observed variation in order to develop effective quality improvement interventions.

Objectives: To describe the clinical features of patients presenting with "idiopathic" interstitial pneumonia that were diagnosed with anti-synthetase syndrome based on clinical features and positive anti-PL-7 or PL-12 antibodies., Methods: Over a 24-month period, we evaluated 37 patients who presented with clinical features of anti-synthetase (AS) syndrome, negative anti-Jo-1 antibodies, and who were assessed for other anti-tRNA synthetase (anti-tRS) antibodies. All data were abstracted from the medical record., Results: Nine (24%) were confirmed to have non-anti-Jo-1 positive AS syndrome based on clinical features and the presence of other anti-tRS antibodies (seven with anti-PL-7, two with anti-PL-12 antibodies). All presented with dyspnea as the initial symptom and with ILD as the first manifestation. Elevated CPK was identified in three patients but only two had muscle weakness. Pulmonary physiology revealed restriction (forced vital capacity 60% of predicted) and impaired gas transfer (diffusing capacity for carbon monoxide 40% of predicted). All had similar findings on thoracic HRCT scans, with basilar predominance of abnormalities and patterns suggestive of non-specific interstitial pneumonia and organizing pneumonia. Immunomodulatory therapies were used to treat the ILD-responses were variable, but some subjects clearly improved., Conclusion: Anti-PL-7 and PL-12 antibodies may be more common among patients presenting with "idiopathic" interstitial pneumonia than formerly considered and should be checked in patients with features of AS syndrome despite a negative screen for anti-nuclear or anti-Jo-1 antibodies. Further research is needed to advance understanding of anti-PL-7 or anti-PL-12-positive AS syndrome, including its prognosis and optimal approaches to therapy.

Objective: To systematically develop a quality indicator (QI) set for systemic lupus erythematosus (SLE)., Methods: We used a validated process that combined available scientific evidence and expert consensus to develop a QI set for SLE. We extracted 20 candidate indicators from a systematic literature review of clinical practice guidelines pertaining to SLE. An advisory panel revised and augmented these candidate indicators and, through 2 rounds of voting, arrived at 25 QIs. These QIs advanced to the next phase of the project, in which we employed a modification of the RAND/UCLA Appropriateness Method. A systematic review of the literature was performed for each QI, linking the proposed process of care to potential improved health outcomes. After reviewing this scientific evidence, a second interdisciplinary expert panel convened to discuss the evidence and provide final ratings on the validity and feasibility of each QI., Results: The final expert panel rated 20 QIs as both valid and feasible. Areas covered included diagnosis, general preventive strategies (e.g., vaccinations, sun avoidance counseling, and screening for cardiovascular disease), osteoporosis prevention and treatment, drug toxicity monitoring, renal disease, and reproductive health., Conclusion: We employed a rigorous multistep approach with systematic literature reviews and 2 expert panels to develop QIs for SLE. This new set of indicators provides an opportunity to assess health care quality in patients with SLE and represents an initial step toward the important goal of improving care in this patient population.

Objective: To evaluate the associations between Medicaid insurance and distance traveled by patients to treating physicians and health care utilization for patients with systemic lupus erythematosus (SLE)., Methods: A total of 982 adults with SLE were recruited between 2002 and 2004. We calculated the distance between patient homes and physicians using Mapquest, an Internet mapping program. We then assessed the association between Medicaid status and distance traveled to the primary SLE provider, presence of > or =1 physician visits, and the number of all physician visits, with and without adjustment for demographic and medical covariates., Results: On an unadjusted basis, Medicaid patients traveled longer distances to see their primary SLE provider. This effect was pronounced for patients under the care of a rheumatologist. Adjustment reduced, but did not eliminate, these differences. With adjustment for covariates, Medicaid patients were equally as likely to see a rheumatologist as non-Medicaid patients. However, Medicaid patients were more likely to be seen by a general practitioner or in the emergency room for their SLE, and reported more visits to general practitioners and the emergency room for SLE., Conclusion: Medicaid patients with SLE traveled longer distances to see an SLE physician, especially rheumatologists. They also reported a different pattern of health care utilization. These results suggest that Medicaid patients may face barriers in obtaining comprehensive medical services in proximity to their residences.

Objective: To examine the role of sociodemographic factors (age, race/ethnicity, and sex) and socioeconomic factors (income and education) in the utilization of rheumatology subspecialty care in a large cohort of subjects with systemic lupus erythematosus (SLE)., Methods: Data were derived from a cohort of 982 English-speaking subjects with SLE. Between 2002 and 2004, trained survey workers administered a telephone survey to subjects eliciting information regarding demographics, SLE disease status, medications, health care utilization, health insurance, and socioeconomic status. We identified predictors of utilization of rheumatology subspecialty care, defined as at least 1 visit to a rheumatologist in the previous year. In addition, we examined factors associated with identifying any specialist as primarily responsible for SLE care., Results: Older age, lower income, Medicare insurance, male sex, and less severe disease were associated with lack of rheumatology care. However, race/ethnicity and educational attainment were not significantly related to seeing a rheumatologist. After multivariate adjustment, only older age, lower income, and male sex remained associated with absence of rheumatology visits. Those least likely to identify a specialist as primarily responsible for their SLE care included older subjects and those reporting lower incomes., Conclusion: Although elderly subjects and those with lower incomes traditionally have access to health care through the Medicare and Medicaid programs, the presence of health insurance alone did not ensure equal utilization of care. This finding suggests that additional barriers to accessing rheumatology subspecialty care may exist in these patient populations.