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Objective: Quality of care (QoC) delivery in rheumatoid arthritis (RA) continues to suffer from various challenges (eg, delay in diagnosis and referral) that can lead to poor patient outcomes. This study aimed to identify good practice interventions that address these challenges in RA care in North America., Methods: The study was conducted in three steps: (1) literature review of existing publications and guidelines (April 2005 to April 2021) on QoC in RA; (2) in-person visits to >50 individual specialists and health care professionals across nine rheumatology centers in the United States and Canada to identify challenges in RA care and any corresponding good practice interventions; and (3) collation and organization of findings of the two previous methods by commonalities to identify key good practice interventions, followed by further review by RA experts to ensure key challenges and gaps in RA care were captured., Results: Several challenges and eight good practice interventions were identified in RA care. The interventions were prioritized based on the perceived positive impact on the challenges in care and ease of implementation. High-priority interventions included the use of technology to improve care, streamlining specialist treatment, and facilitating comorbidity assessment and care. Other interventions included enabling patient access to optimal medication regimens and improving patient self-management strategies., Conclusion: Learnings from the study can be implemented in other rheumatology centers throughout North America to improve RA care. Although the study was completed before the COVID-19 pandemic, the findings remain relevant., (© 2024 The Author(s). ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Monoclonal gammopathies result from neoplastic clones of the B-cell lineage and may cause kidney disease by various mechanisms. When the underlying clone does not meet criteria for a malignancy requiring treatment, the paraprotein is called a monoclonal gammopathy of renal significance (MGRS). One rarely reported kidney lesion associated with benign paraproteins is thrombotic microangiopathy (TMA), provisionally considered as a combination signifying MGRS. Such cases may lack systemic features of TMA, such as a microangiopathic hemolytic anemia, and the disease may be kidney limited. There is no direct deposition of the paraprotein in the kidney, and the presumed mechanism is disordered complement regulation. We report three cases of kidney limited TMA associated with benign paraproteins that had no other detectable cause for the TMA, representing cases of MGRS. Two of the cases are receiving clone directed therapy, and none are receiving eculizumab. We discuss in detail the pathophysiological basis for this possible association. Our approach to therapy involves first ruling out other causes of TMA as well as an underlying B-cell malignancy that would necessitate direct treatment. Otherwise, clone directed therapy should be considered. If refractory to such therapy or the disease is severe and multisystemic, C5 inhibition (eculizumab or ravulizumab) may be indicated as well., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Filippone, Newman, Li, Gulati and Farber.)

Objectives: To describe and evaluate the impact of primary and specialty care integration via asynchronous communication at a large integrated healthcare system., Study Design: In January 2014, Geisinger's primary care providers (PCPs) were given access to an asynchronous communication tool, Ask-a-Doc (AAD), that enabled direct communication with specialists in 14 medical specialties and 5 surgical specialties. Internal data were collected to assess PCPs' acceptance and use of the tool, as well as satisfaction. Insurance claims data were obtained to assess the impact on healthcare utilization and cost., Methods: A retrospective analysis of health plan claims data was conducted among those patients who had at least 1 specialist visit with 1 of the participating specialties between January 2014 and December 2016. A set of difference-in-differences multivariate linear regression models with patient fixed effects was estimated, in which those who were not exposed to AAD served as the comparison group., Results: Acceptance and use of AAD among PCPs gradually increased over time but varied by specialty. AAD was associated with an approximately 14% reduction in total cost of care during the first month of follow-up and a 20% reduction (P <.001) during the second month. These reductions in cost of care appeared to be driven by reductions in emergency department visits and physician office visits., Conclusions: Geisinger's AAD experience suggests that the integration of primary and specialty care via the use of a highly reliable and efficient asynchronous communication system can potentially lead to reductions in avoidable care and more efficient use of specialty care.

One significant driver of the disjointed healthcare often observed in the United States is the traditional fee-for-service payment model which financially incentivizes the volume of care delivered over the quality and coordination of care. This problem is compounded by the wide, often unwarranted variation in healthcare charges that purchasers of health services encounter for substantially similar episodes of care. The last 10 years have seen many stakeholder organizations begin to experiment with novel financial payment models that strive to obviate many of the challenges inherent in customary quantity-based cost paradigms. The Patient Protection and Affordable Care Act has allowed many care delivery systems to partner with Medicare in episode-based payment programs such as the Bundled Payments for Care Improvement (BPCI) initiative, and in patient-based models such as the Medicare Shared Savings Program. Several employer purchasers of healthcare services are experimenting with innovative payment models to include episode-based bundled rate destination centers of excellence programs and the direct purchasing of accountable care organization services. The Geisinger Health System has over 10 years of experience with episode-based payment bundling coupled with the care delivery reengineering which is integral to its ProvenCare® program. Recent experiences at Geisinger have included participation in BPCI and also partnership with employer-purchasers of healthcare through the Pacific Business Group on Health (representing Walmart, Lowe's, and JetBlue Airways). As the shift towards value-focused care delivery and patient experience progresses forward, bundled payment arrangements and direct purchasing of healthcare will be critical financial drivers in effecting change., (Copyright © 2017 by the Congress of Neurological Surgeons.)

Objective: To examine the potential value of a theory-based, interactive decision support tool in clinical practice for patients with rheumatoid arthritis who are candidates for biologic agents., Methods: We conducted an 8-week, 2-arm, parallel, single-blind pilot trial in which candidates for treatment escalation with a biologic agent were randomized to receive either a link to a web-based tool or usual care. Outcomes included changes in objective knowledge, subjective knowledge, values clarification, and satisfaction with risk communication as well as the proportion of subjects defined as making an informed choice to escalate care at 2 weeks., Results: A total of 125 subjects were randomized. Significant between-group differences at 2 weeks favoring the intervention group were seen for changes in objective knowledge, subjective knowledge, and values clarification. No significant between-group differences were found in subjects' satisfaction with risk communication. Among those deciding to escalate care, a greater percentage met the criteria for an informed choice at 2 weeks in the intervention group compared to the control group (32% versus 13%; P = 0.02). Improvements in subjective knowledge and values clarification persisted at 8 weeks. There were no between-group differences in objective knowledge at 8 weeks., Conclusion: In this study, use of a decision support tool at the time of decision-making resulted in improved objective and subjective knowledge, as well as values clarity, compared to usual care. Not all improvements were sustained, emphasizing the need to offer educational support should additional escalation of care be required over the course of the illness., (© 2015, American College of Rheumatology.)

Objective: Electronic health records (EHRs) are not optimized for chronic disease management. To improve the quality of care for patients with rheumatic disease, we developed electronic data capture, aggregation, display, and documentation software., Methods: The software integrated and reassembled information from the patient (via a touchscreen questionnaire), nurse, physician, and EHR into a series of actionable views. Core functions included trends over time, rheumatology-related demographics, and documentation for patient and provider. Quality measures collected included patient-reported outcomes, disease activity, and function. The software was tested and implemented in 3 rheumatology departments, and integrated into routine care delivery. Post-implementation evaluation measured adoption, efficiency, productivity, and patient perception., Results: Over 2 years, 6,725 patients completed 19,786 touchscreen questionnaires. The software was adopted for use by 86% of patients and rheumatologists. Chart review and documentation time trended downward, and productivity increased by 26%. Patient satisfaction, activation, and adherence remained unchanged, although pre-implementation values were high. A strong correlation was seen between use of the software and disease control (weighted Pearson's correlation coefficient 0.5927, P = 0.0095), and a relative increase in patients with low disease activity of 3% per quarter was noted., Conclusion: We describe innovative software that aggregates, stores, and displays information vital to improving the quality of care for patients with chronic rheumatic disease. The software was well-adopted by patients and providers. Post-implementation, significant improvements in quality of care, efficiency of care, and productivity were demonstrated., (Copyright © 2015 by the American College of Rheumatology.)

Summary: A programmatic outpatient high-risk osteoporosis clinic (outpatient HiROC) and inpatient fracture liaison service (inpatient HiROC) is described. Results document that this population is more effectively treated and followed up in this specialty pathway than with primary care follow-up., Introduction: We describe a programmatic approach to outpatient care of high-risk osteoporosis patients (outpatient HiROC). We similarly describe an inpatient fracture liaison service (inpatient HiROC), which integrates into the existing outpatient HiROC pathway., Methods: The development of outpatient HiROC and inpatient HiROC is described. Outpatient visits (July 29, 2008 to October 27, 2011) are included with a 200 patients random sample calculation. Inpatient consultation visits between November 18, 2008 and October 27, 2011 are included., Results: Between July 29, 2008 and December 31, 2011, 1917 outpatient consults were seen. Of the 200 patient samples, 87% were female, mean age of 69.8 years, previous fractures occurred in 34% patients, and glucocorticoid users constituted 10.6%. Eighty-six percent of this group was high risk, where drug therapy is indicated, and such treatment was started in 89%. A total of 1041 inpatient fracture consults were seen during the evaluable period; 14.7% of this population died before the 6-month follow-up. Females comprised 77.6%, mean age was 76.1 years, and 58.2% of fractures were hip fragility, 11.6% vertebral, and 1.7% midshaft and 1.6% subtrochanteric. Patients seen in our outpatient HiROC pathway were significantly more likely to be treated than those followed up by one of our primary care doctors (80.6 versus 32.2%, P<0.0001). Mean vitamin D levels at baseline (27.0 ng/mL) improved to 34.6 ng/mL at 6-month follow-up (P<0.0001)., Conclusions: Our outpatient and inpatient HiROC model is efficient and effective in risk stratifying and treating patients at high risk for fractures.

Dietary flavonoid intake, especially berry flavonoids, has been associated with reduced risks of cardiovascular disease (CVD) in large prospective cohorts. Few clinical studies have examined the effects of dietary berries on CVD risk factors. We examined the hypothesis that freeze-dried strawberries (FDS) improve lipid and lipoprotein profiles and lower biomarkers of inflammation and lipid oxidation in adults with abdominal adiposity and elevated serum lipids. In a randomized dose-response controlled trial, 60 volunteers [5 men and 55 women; aged 49 ± 10 y; BMI: 36 ± 5 kg/m(2) (means ± SDs)] were assigned to consume 1 of the following 4 beverages for 12 wk: 1) low-dose FDS (LD-FDS; 25 g/d); 2) low-dose control (LD-C); 3) high-dose FDS (HD-FDS; 50 g/d); and 4) high-dose control (HD-C). Control beverages were matched for calories and total fiber. Blood draws, anthropometrics, blood pressure, and dietary data were collected at screening (0 wk) and after 12-wk intervention. Dose-response analyses revealed significantly greater decreases in serum total and LDL cholesterol and nuclear magnetic resonance (NMR)-derived small LDL particle concentration in HD-FDS [33 ± 6 mg/dL, 28 ± 7 mg/dL, and 301 ± 78 nmol/L, respectively (means ± SEMs)] vs. LD-FDS (-3 ± 11 mg/dL, -3 ± 9 mg/dL, and -28 ± 124 nmol/L, respectively) over 12 wk (0-12 wk; all P < 0.05). Compared with controls, only the decreases in total and LDL cholesterol in HD-FDS remained significant vs. HD-C (0.7 ± 12 and 1.4 ± 9 mg/dL, respectively) over 12 wk (0-12 wk; all P < 0.05). Both doses of strawberries showed a similar decrease in serum malondialdehyde at 12 wk (LD-FDS: 1.3 ± 0.2 μmol/L; HD-FDS: 1.2 ± 0.1 μmol/L) vs. controls (LD-C: 2.1 ± 0.2 μmol/L; HD-C: 2.3 ± 0.2 μmol/L) (P < 0.05). In general, strawberry intervention did not affect any measures of adiposity, blood pressure, glycemia, and serum concentrations of HDL cholesterol and triglycerides, C-reactive protein, and adhesion molecules. Thus, HD-FDS exerted greater effects in lowering serum total and LDL cholesterol and NMR-derived small LDL particles vs. LD-FDS in the 12-wk study. These findings warrant additional investigation in larger trials. This trial was registered at clinicaltrials.gov as NCT01883401., (© 2014 American Society for Nutrition.)

Aims. To examine the antioxidant and anti-inflammatory effects of pomegranate polyphenols in obese patients with type 2 diabetes (T2DM) (n = 8) and in healthy nondiabetic controls (n = 9). Methods. Participants received 2 capsules of pomegranate polyphenols (POMx, 1 capsule = 753 mg polyphenols) daily for 4 weeks. Blood draws and anthropometrics were performed at baseline and at 4 weeks of the study. Results. Pomegranate polyphenols in healthy controls and in T2DM patients did not significantly affect body weight and blood pressure, glucose and lipids. Among clinical safety profiles, serum electrolytes, renal function tests, and hematological profiles were not significantly affected by POMx supplementation. However, aspartate aminotransferase (AST) showed a significant increase in healthy controls, while alanine aminotransferase (ALT) was significantly decreased in T2DM patients at 4 weeks (P < 0.05), though values remained within the normal ranges. Among the biomarkers of lipid oxidation and inflammation, oxidized LDL and serum C-reactive protein (CRP) did not differ at 4 weeks in either group, while pomegranate polyphenols significantly decreased malondialdehyde (MDA) and hydroxynonenal (HNE) only in the diabetic group versus baseline (P < 0.05). Conclusions. POMx reduces lipid peroxidation in patients with T2DM, but with no effects in healthy controls, and specifically modulates liver enzymes in diabetic and nondiabetic subjects. Larger clinical trials are merited.

Objective: While questionnaires have been developed to capture patient-reported outcomes (PROs) in rheumatology practice, these instruments are not widely used. We developed a touchscreen interface designed to provide reliable and efficient data collection. Using the touchscreen to obtain PROs, we compared 2 different workflow models implemented separately in 2 rheumatology clinics., Methods: The Plan-Do-Study-Act methodology was used in 2 cycles of workflow redesign. Cycle 1 relied on off-the-shelf questionnaire builder software, and cycle 2 relied on a custom programmed software solution., Results: During cycle 1, clinic 1 (private practice model, resource replete, simple flow) demonstrated a high completion rate at the start, averaging between 74% and 92% for the first 12 weeks. Clinic 2 (academic model, resource deficient, complex flow) did not achieve a consistent completion rate above 60%. The revised cycle 2 implementation protocol incorporated a 15-minute "nurse visit," an instant messaging system, and a streamlined authentication process, all of which contributed to substantial improvement in touchscreen questionnaire completion rates of ∼80% that were sustained without the need for any additional clinic staff support., Conclusion: Process redesign techniques and touchscreen technology were used to develop a highly successful, efficient, and effective process for the routine collection of PROs in a busy, complex, and resource-depleted academic practice and in typical private practice. The successful implementation required both a touchscreen questionnaire, human behavioral redesign, and other technical solutions., (Copyright © 2012 by the American College of Rheumatology.)

Calcineurin inhibitors (CNI) have been clearly associated with posttransplant thrombotic microangiopathy (PTTMA). We report a case of de novo PT-TMA involving predominantly small arteries and arterioles of a renal allograft in a patient receiving tacrolimus. Serial biopsies demonstrate the natural history of this lesion through the chronic nonspecific phase. The case is discussed in the context of a literature review of PT-TMA in general and CNI use in particular.

To determine the proportion of rheumatoid arthritis (RA) patients receiving preventive health care according to US Preventive Services Task Force recommendations compared with a community-based population sample, with emphasis on dyslipidemia testing, given the increased risk of cardiovascular disease (CVD) in RA patients. Patients with RA (ICD-9 code 714.0 at ≥2 office visits with a rheumatologist) and a primary care physician (PCP) at the Geisinger Health System (GHS) were identified through electronic health records. The records were searched back from 3/31/08 for the length of time required to satisfy each outcome measure. Percentages were compared with population testing rates using the Pearson Chi-square test. Eight hundred and thirty-one RA patients were compared to 169,476 subjects with a PCP at GHS, stratified by gender and age. Patients with RA were more likely to have had dyslipidemia and osteoporosis testing compared with the general population (86 vs. 75 and 75 vs. 55%, respectively, P < 0.0001 for both). The proportion of RA patients receiving breast and cervical cancer testing was similar to the general population. The majority (79%) of lipid testing was ordered by PCPs. Those RA patients with recommended lipid testing had more traditional CVD factors (hypertension, diabetes, coronary artery disease). RA patients are screened more than the general population for two RA-related co-morbidities, i.e. dyslipidemia and osteoporosis. The RA patients with traditional cardiovascular risk factors are more likely to be tested for dyslipidemia. Further work is warranted to improve testing for modifiable CVD risk factors in this group with multiple co-morbidities.

In its first decade, the goals of the Geisinger Health System Osteoporosis Program at its inception were to increase awareness, diagnosis, and treatment of osteoporosis and to monitor predefined outcomes. The program was innovative in that it crossed specialties and regions and used guidelines in an effective manner. In addition, success in reducing hip fracture and cost were demonstrated, and it remains one of the few programs today that has done so, as reported by Newman et al. (Osteoporos Int 14:146-151, 2003). The osteoporosis program has now moved from a provider and allied provider empowerment focus to reorganizing our thoughts about how to best manage osteoporosis care across our healthcare system by defining and acting on four major osteoporosis care gaps: (1) at-risk patients do not get tested, (2) tested patients are not accurately risk assessed, (3) high-risk patients do not get treated, and (4) treated patients are not adherent. Results of current internal programs and future steps are discussed.

Background/objectives: Several studies have associated hydroxychloroquine use with decreased risk of diabetes mellitus (diabetes) or improved glycemic control in rheumatoid arthritis patients, but the studies were small or used data from self-report. The present study sought to replicate this protective relationship in a health system using electronic health records with laboratory data and physician diagnoses., Methods: This study is a retrospective cohort of 1127 adults with newly diagnosed rheumatoid arthritis and no diabetes within the Geisinger Health System between January 1, 2003, and March 31, 2008. Patients were classified as ever users (n = 333) or never users (n = 794) of hydroxychloroquine. Incident diabetes cases were defined using 2010 American Diabetes Association criteria., Results: The median follow-up times for the ever and never hydroxychloroquine users were 26.0 and 23.0 months, respectively (P = 0.28). The median duration of hydroxychloroquine exposure was 14.0 months. Of the 48 cases developing diabetes during observation, 3 were exposed to hydroxychloroquine at time of development and 45 were nonexposed, yielding incidence rates of 6.2 and 22.0 per 1000 per year (P = 0.03), respectively. In time-varying Cox proportional hazards regression models adjusting for sex, age, body mass index, positive rheumatoid factor and anti-cyclic citrullinated peptide antibodies, erythrocyte sedimentation rate, and nonsteroidal anti-inflammatory drug, glucocorticoid, methotrexate, and tumor necrosis factor α inhibitor use, the hazard ratio for incident diabetes among hydroxychloroquine users was 0.29 (95% confidence interval, 0.09-0.95; P = 0.04) compared with nonusers., Conclusions: Our findings support the potential benefit of hydroxychloroquine in attenuating the risk of diabetes in rheumatoid arthritis patients. Further work is needed to determine its potential preventive role in other groups at high risk for diabetes.

Unlabelled: To determine whether a process redesign could improve detection and treatment of osteoporosis, at-risk women over the age of 65 were identified using an electronic medical record and proactively contacted by letter and phone call. This resulted in a significant increase in testing for osteoporosis by DXA scan. The high-risk patients were then offered a shared medical appointment, which resulted in improved treatment outcomes compared to usual care., Introduction: Our objective was to determine if redesigning care through proactive contact with women 65 at-risk of osteoporosis increased BMD testing and to determine if a shared medical appointment (SMA) improved treatment for high-risk women., Methods: Two primary care sites received the redesign intervention and two other sites served as the usual care controls. At the intervention sites, all women 65 who had not had a DXA scan performed in the prior 2 years were contacted by mail and phone calls. High-risk patients were invited to attend a SMA or follow-up visit with their primary physician., Results: A significantly higher proportion of women at the intervention sites had a DXA (39.6% vs. 13.2%, p < 0.0001). Patients who attended the SMA were more likely to have calcium and vitamin D recommended, a vitamin D level checked, and receive a prescription medicine than those patients who had follow-up with their primary care physician., Conclusions: The redesigned process was highly effective in improving BMD testing for women 65. The SMA was shown to be a more effective method to make calcium and vitamin D recommendations, to evaluate secondary causes of low bone density, and to prescribe prescription medications, compared to usual care with the PCP.

Redesigning the delivery-of-care processes for rheumatic diseases within rheumatology practices and health systems is critical to improving the outcomes and costs of care for the patients we serve. This work is best accomplished using Continuous Quality Improvement Methods, also known as Plan-Do-Study-Act (PDSA) cycles that are widely utilized in many other industries, but not often in health care or among physicians. This first of two companion articles provides background on health care redesign, understanding of PDSA methods, and examples of successful rheumatology practice process redesigns based on PDSA. It is offered as a starting point for rheumatologists preparing for this necessary work.

Changing delivery-of-care processes for rheumatic diseases to improve outcomes and costs will require redesign not only within rheumatology practices but also within health systems. Preventive services, acute care, management of chronic co-morbidities, and rheumatology care for rheumatic disease patients can only be accomplished through the close integration of multiple practices and other health system resources. Rheumatologists can play an important role in system-level process improvement without which our own patient care will be compromised. Continuous Quality Improvement methods, also known as Plan-Do-Study-Act (PDSA) cycles, are ideally suited for system-level process redesign. This second of two companion articles describes the properties of systems and explores the redesign of interdisciplinary rheumatic disease care.

Introduction: Patients who take chronic glucocorticoids (GC) are at increased risk of osteoporosis and fracture. Only a minority of patients who take chronic GC receive optimal osteoporosis prevention, diagnosis, and/or treatment., Methods: An organized program of care--GIOP (Glucocorticoid-Induced Osteoporosis Program)--was designed and implemented. The program goals were to identify patients at risk of fracture, provide education, redesign and implement new pathways of care, and monitor outcomes. Two hundred chronic GC users were seen at baseline, and follow-up visits scheduled at 6 months and 1 year., Results: Patient retention of knowledge, frequent exercise, and 25-OH Vitamin D levels all significantly improved at 1 year. A significant decrease in GC dose was seen. In terms of adherence, 91% of patients considered at high risk were taking a bisphosphonate or teriparatide at 1 year, and 96% of patients overall were adherent to their prescribed regimen of calcium, vitamin D, and prescription treatment (if indicated). Bone density at the spine and total hip increased significantly., Conclusions: GIOP is the first organized program of care for patients who take chronic GC that has demonstrated a clinically significant improvement in outcome. The program's design can be adapted and used by other health systems and organizations.

Currently, it is unusual to combine evaluation for vertebral fracture with measurement of bone mineral density in clinical practice. Using Quantitative Morphometric Vertebral Analysis (Instant Vertebral Assessment [IVA]) in our existing Mobile Dual-Energy X-Ray Absorptiometry (DXA) Program, we implemented a testing procedure that examined 5 different IVA protocols focusing on clinical utility and cost. Using small-scale tests of change (PDSA cycles), data from the preceding cycle drives the development of the next cycle. In this article, we describe the process and rationale for selecting patients for the IVA study. In addition, we review the literature on vertebral fracture assessment using DXA and emphasize the clinical utility of point of service testing by providing the needed knowledge for best patient care by simultaneous DXA and IVA testing. The application of this new technology increased identification of the high-risk patient by 11%, with a nominal additional cost per DXA study of $14. This study provides a useful framework for the integration of IVA into a clinical DXA program.

Osteoporosis diagnosis and monitoring is best accomplished with dual X-ray absorptiometry (DXA), but technology availability can hinder access to care. We designed a mobile DXA program incorporating a Hologic Delphi-C trade mark bone densitometer housed in a specially configured 30-ft Winnebago trade mark. The mobile DXA program provided osteoporosis testing and education at the convenience of the patient's primary care site within our rural health care system. DXA results were sent electronically to the patient's physician within 48-72 h. The mobile DXA patient group tended to be older and at high risk for future fracture. The service provided was rated as excellent by patients. Given the volume of patients studied, the program was financially self-sustaining. Other healthcare systems or groups should consider development of a similar program.

A 5 year observational study is reported which reviews the implementation of the Geisinger Health System Osteoporosis Disease Management Program. This program includes Osteoporosis Clinical Practice Guidelines, physician and allied health care provider education, community education, and a bone density testing program. All women over the age of 55 years enrolled in the Geisinger Health Plan (GHP) from 1996 to 2000 were included in this analysis. The guidelines led health care providers to increase the evaluation of osteoporosis via bone density measurements and to increase the prescription treatment of osteoporosis. Most importantly, the age-adjusted incidence of hip fractures fell significantly in the entire group as well as in several age strata (65-74, 75-84 and 85+ years). Compared with a predictive model of no intervention, there was an overall reduction in health care costs to GHP estimated to be US$7.8 million over a 5 year period. This report is the first to suggest that an organized Osteoporosis Disease Management Program delivered by a health care system can result in increased evaluation and treatment of osteoporosis with a resultant significant decrease in hip fractures while decreasing the total direct costs of care to a health plan.

Background: Children with juvenile dermatomyositis (JDMS) have variable initial presentations., Objective: Our purpose was to evaluate the epidemiology trends, presenting clinical features, laboratory data, and outcome of patients with JDMS., Methods: A total of 16 patients were identified at Geisinger Medical Center by a 30-year retrospective chart review., Results: Sex ratio, age at diagnosis, and outcome were similar to data published in previous studies. However, certain trends were noted. The most common initial physical examination findings were an extremity rash (94%) and periungual erythema (75%). New associations of JDMS that were uncovered included the findings of pruritus (38%) and a psoriasiform scalp dermatitis (25%). Nonspecific laboratory elevations were the most common initial laboratory changes (erythrocyte sedimentation rate, lactate dehydrogenase, and aspartate aminotransferase). Tubuloreticular inclusions as found on electron microscopy of muscle biopsy specimens were present in all 3 patients tested. One patient with tubuloreticular inclusions had otherwise normal muscle biopsy findings on hematoxylin-and-eosin staining. Two of the 16 patients had cutaneous findings of JDMS but did not exhibit muscle involvement after long-term follow-up at 4 and 5 years., Conclusion: Our study confirms that the initial physical and laboratory findings in patients with JDMS may be nonspecific. The heliotrope rash and Gottron papules classically associated with dermatomyositis appeared less commonly than an extremity rash and periungual erythema. Creatinine kinase and aldolase levels may not be elevated on initial presentation. Pruritus, a psoriasiform scalp dermatitis, and tubuloreticular inclusions found on muscle biopsy electron microscopy should be additional factors to consider. The long-term follow-up in 2 patients without muscle involvement lends support to the existence of amyopathic dermatomyositis.

We review our experience with needle muscle biopsy, including technique, results, complications, and outcome. We have collected data from 40 consecutive patients undergoing needle muscle biopsy of the quadriceps muscle. All biopsies were performed by the same operator and 98% were performed in the outpatient clinic. Specimens were sent to pathology for processing, staining, and interpretation. Follow-up clinical information was obtained by chart review. The ages of the patients ranged from 9 to 84 years, including three children. Of the 27 patients with a prebiopsy suspicion of idiopathic inflammatory myopathy (polymyositis, dermatomyositis, or inclusion body myositis) 13 had biopsies with consistent pathologic changes. Seven patients in this group had no pathologic diagnosis - none of these patients subsequently developed active myositis. Other conditions seen included mitochondrial myopathy, neuropathy, and type II fiber atrophy. Biopsies were very well tolerated, and no significant complications were seen. Therapeutic decisions were influenced most by needle muscle biopsy results obtained from patients suspected of having idiopathic inflammatory myopathy. Needle muscle biopsy charges were approximately 40% lower than those for open biopsies performed during the same interval. Needle muscle biopsy is a safe, care-effective, and cost-effective alternative to open muscle biopsy in the evaluation of a variety of myopathies.

We have performed a survey to determine the percentage of patients with primary biliary cirrhosis (PBC) and arthritis followed at Geisinger Clinic, a rural tertiary care center. We have assessed the clinical features of the arthritis, delineated any signs that suggest the diagnosis of asymptomatic PBC in patients with arthritis, and explored coexisting immune diseases.From January 1988 through November 1993, 36 patients with PBC were identified from a computer search of the Geisinger Gastroenterology Clinic database. These records were reviewed for clinical information of an associated arthritis, other autoimmune processes, and demographic information.Twenty-five percent of the patients with PBC had an inflammatory arthritis. Two patients had classic, seropositive rheumatoid arthritis with erosions and nodules. The remaining seven patients had a predominantly symmetrical, nonnodular inflammatory arthritis involving both large and small joints. Tenosynovitis was the most common presenting rheumatic feature. Sjögren's syndrome, Raynaud's phenomenon, and hypothyroidism were more common in the subgroup of PBC patients with arthritis.A diagnosis of PBC should be considered in any patient presenting with tenosynovitis or an unexplained inflammatory arthritis, especially in the setting of Raynaud's phenomenon and signs of Sjögren's syndrome.

The objective of this study was to evaluate the efficacy of low-dose oral methotrexate (MTX) in improving muscle strength and reducing the corticosteroid requirement of patients with polymyositis (PM) and dermatomyositis (DM). The method used was a retrospective chart review of our clinic's PM/DM cohort, Between September 1989 and May 1993, 12 of 53 patients with PM/DM received low-dose oral MTX (mean maximum dose 14.4 +/- 1.2 mg/wk, range 7.5-20 mg/wk). A significant increase in strength coupled with a fall in prednisone requirement (42.5 +/- 5.8 mg/d to 13.0 +/- 5.4 mg/d) was seen during the mean follow-up time of 24.0 +/- 4.3 months of MTX therapy. The number of flares per year fell from 1.00 +/- 0.35 before oral MTX institution to 0.07 +/- 0.05 during oral MTX therapy. Toxicity was minimal. Three patients were able to stop corticosteroids altogether. Low-dose oral MTX therapy should be considered early in the treatment course of patients with PM/DM to improve strength and lower the required dose of corticosteroids.

The myelodysplastic syndromes (MDS) are a group of therapeutically refractory anemias resulting from a clonal stem cell disorder often associated with cytogenetic abnormalities. Immunologic abnormalities and occasionally vasculitis have been reported although no series has characterized an associated arthritis. All cases of MDS diagnosed in 1990 by bone marrow biopsy and followed up at the authors' institution were reviewed. Of the 28 consecutive patients, 8 had acute seronegative inflammatory arthritis temporally related to the initial discovery of cytopenia. Five patients had a symmetric polyarthritis resolving only with use of steroids or upon evolution to leukemia, and 3 had episodes of oligoarthritis with systemic features including fever, pleuritis, pericarditis, and hemolytic anemia. Arthrocenteses in 2 cases did not show crystals or infection. Serological studies were nondiagnostic. The arthritis and systemic features responded to steroids in all 5 treated patients. Inflammatory arthritis appears to be common in MDS. Most compelling is the apparent bone marrow response to steroids in 2 cases, possibly identifying a treatable subgroup.

Between 1981 and 1989, 3 of 134 patients with rheumatoid arthritis (RA) treated with methotrexate (MTX) developed clinically significant hepatic dysfunction and showed histologic evidence of severe liver disease (fibrosis and cirrhosis). Factors identified in these patients that may have been linked to liver toxicity included diabetes, congestive heart failure and Felty's syndrome. In the patient group that received a post-MTX liver biopsy, pulmonary fibrosis and obesity were significantly associated with hepatic fibrosis/cirrhosis. Severe liver disease may occur in patients with RA treated with low dose MTX (less than 3%). Early liver biopsy is recommended in selected cases.

Objective: To explore alterations in energy utilization as a potential etiology for weakness in polymyositis and dermatomyositis (PM/DM)., Methods: P-31 magnetic resonance spectroscopy studies were performed in patients with acute and treated PM/DM and in normal controls, at rest and with exercise., Results: Patients with acute and treated PM/DM showed increased ratios of inorganic phosphate to phosphocreatine (PCr) during exercise, with loss of ATP disproportional to loss of PCr., Conclusion: This study demonstrates changes in energy utilization in PM/DM, thus supporting the notion of a metabolic etiology for the weakness associated with these diseases.

Sulfasalazine therapy has been shown effective in rheumatoid arthritis and ankylosing spondylitis. We treated 10 patients with active polyarticular psoriatic arthritis with 2 g/day of sulfasalazine for 16 weeks. Significant improvement was seen in joint count score, morning stiffness, and patient/physician assessment of disease activity. Toxicity requiring drug cessation was seen in only 1 patient. Patients with psoriatic arthritis had elevated B cells and immunoglobulin levels which fell with sulfasalazine therapy. Minimal changes were seen in T cell subsets. Sulfasalazine appears to be an effective second line agent for the treatment of psoriatic arthritis. Its mechanism of action may in part relate to alteration of B cell number and function.

Endothelin (ET) effected a dose-dependent increment in atrial natriuretic peptide (ANP) secretion and ANP mRNA accumulation in neonatal rat atrial and ventricular cardiocytes but had no effect on the processing of the ANP prohormone to the mature ANP product. The secretagogue effect was not limited by cell density. Both basal and ET-dependent secretory activity were abrogated by the calmodulin antagonist calmidazolium but were unaffected by meclophenamate or pertussis toxin. The magnitude of the ET-dependent increment in ANP secretion was amplified by culturing the cells in a dynamically pulsating (vs. static) environment, implying an interaction between mechanical and agonist-mediated secretory stimuli in this system. ET also promoted immunoreactive ANP release from primary cultures of fetal rat hypothalamic cultures, suggesting that this regulatory function may be generally employed in ANP gene-expressing cells. These findings demonstrate that ET has parallel effects on ANP synthesis and secretion and support a role for this peptide in the regulation of local and circulating levels of the natriuretic hormone.

We have previously shown that neonatal exposure of rats to pharmacologic doses of diethylstilbestrol via daily injections resulted in a significant decrease in the estrogen-binding capacity of the uterine estrogen receptor (ER). In this study, we examined the effects of physiologic and pharmacologic doses of estradiol (E2) administered to adult ovariectomized rats via Silastic implants. Two days after implantation, uteri were removed, weighted, and homogenized, and ER levels were determined in the supernatant (hydroxylapatite assay) and low-speed pellet (nuclear exchange assay). Implants containing E2 concentrations of 0.005 or 0.05 mg/ml increased cytosolic but not total ER-binding capacity, whereas 0.5 or 5.0 mg of E2/ml implants decreased the binding capacity of cytosol ER to 40% and total ER to 50% of control values. The 0.005-mg/ml dose increased cytosol ER without increasing uterine weight; all higher doses significantly increased uterine weight. Determination of ER protein by an ER radioimmunoassay showed the same extent of reduction of ER concentration as the binding assays, demonstrating that the loss in E2 binding capacity is homologous down-regulation. The down-regulation of ER was maximal at 24 hr and was completely reversible after implant removal, although the time required to recover from down-regulation was dose dependent. Uterine weight also returned to control levels slowly after implant removal. Neither the sedimentation rate of the down-regulated ER nor the Kd of the cytosolic ER changed following long-term implantation; however, the Kd of the nuclear ER decreased significantly. This is the first demonstration of in vivo homologous down-regulation of uterine ER. ER down-regulation may play a role in several biologic processes.

The human placenta and its associated membranes are vital to the maintenance, nutrition, and protection of the developing fetus. During placental development some cytotrophoblasts give rise to the chorionic membrane whereas others fuse to form a differentiated syncytium of cells that are responsible for placental protein and steroid hormone production. The mechanisms involved in the differentiation of the trophoblasts are unknown; however, an intermediate stage with a characteristic phenotype has been documented in vivo. We have observed that chemically dissimilar xenobiotic agents induced BeWo choriocarcinoma cells to change their usual cytotrophoblastic phenotype and acquire morphologic and functional characteristics typical of intermediate trophoblast. Incubation of BeWo cell cultures in the presence of 1 mumol/L methotrexate for 48 hours stimulated human chorionic gonadotropin secretion (2.3-fold) and aromatase activity (4.9-fold). Morphologic findings associated with these hormonal changes, including increased nuclear size and cytoplasmic expansion, were also observed. With the use of a computer-interfaced image analyzer, planimetric morphometry of the nuclear area of the cells revealed a 1.8-fold increase after incubation with methotrexate. The effect of forskolin, a direct activator of adenylyl cyclase, was also evaluated by means of this model of cytotrophoblast differentiation. The addition of 10 mumol/L forskolin to BeWo cultures also resulted in dramatic changes in trophoblast cell phenotype. Increases in human chorionic gonadotropin synthesis (7.3-fold), aromatase activity (13.5-fold), and nuclear area (3.0-fold) were induced over those of untreated cells. In addition, increases in [3H]thymidine incorporation (1.7-fold) were afforded by both treatments. These results suggest that biochemical and cytologic changes associated with human trophoblast differentiation can be induced in vitro via activation of the adenylyl cyclase pathway by forskolin and through unknown and apparently independent signals by methotrexate.