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Personalized tools relevant to an individual patient’s unique characteristics may be an important component of personalized health care. We randomized 97 patients hospitalized with acute decompensated heart failure to receive a printout of an ultrasound image of their inferior vena cava (IVC) with an explanation of how the image is related to their fluid status (n = 50) or to receive no image and only generic heart failure information (n = 47). Adherence to medications, low-sodium diet, and daily weight measurement at baseline and 30 days after discharge were assessed using the Medical Outcomes Study Specific Adherence Scale, modified to a three-item version for heart failure (HF), (MOSSAS-3HF, maximum score = 15, indicating adherence all of the time). The baseline MOSSAS-3HF scores (mean ± standard deviation (SD)) were similar for intervention and control groups (7.4 ± 3.4 vs. 6.4 ± 3.7, p = 0.91). The MOSSAS-3HF scores improved for both groups but were not different at 30 days (11.8 ± 2.8 vs. 11.7 ± 3.0, p = 0.90). Survival without readmission or emergency department (ED) visit at 30 days was similar (82.6% vs. 84.1%, p = 0.85). A personalized HF tool did not affect rates of self-reported HF treatment adherence or survival without readmission or ED visit.

Three sisters of Northern European descent provided an opportunity to examine the longterm course and possible familial aspects of a rare disease, polyarteritis nodosa (PAN). Approval and consent was obtained from each patient. The sisters have been cared for in the Johns Hopkins Vasculitis Center for the past 3 decades. At age 7, the first sister developed rash, fever, lethargy, and a deviated right eye and was treated empirically for tuberculosis. At age 8, these symptoms recurred along with abdominal pain, hypertension, and stroke. Renal angiography demonstrated microaneurysms and a skin biopsy confirmed the diagnosis of PAN. She was initiated on prednisone and azathioprine (AZA). Within the following year, she developed 2 bowel perforations with pathology demonstrating PAN and was treated with cyclophosphamide (CYC). By age 17, methotrexate was added for abdominal pain in the context of worsening mesenteric angiography. At age 19, she developed mononeuritis multiplex, and acute abdominal pain led to discovery of a ruptured renal aneurysm and upper gastrointestinal (GI) bleed, necessitating gastrectomy. She was treated with prednisone, CYC, and total parenteral nutrition. From ages 19 to 25 years, she had 8 hospitalizations for abdominal angina and GI bleeding and 1 for stroke. Angiography during this period showed 95–99% stenosis of the celiac artery and diffuse changes of PAN in the superior and inferior mesenteric arteries as well as active skin lesions. … Address correspondence to Dr. J. Liebowitz, Johns Hopkins University, Division of Rheumatology, 5200 Eastern Ave., MFL Bldg., Center Tower, Suite 4100, Baltimore, Maryland 21224, USA. E-mail: jliebow3{at}jhmi.edu

Background : Teaching interns patient-centered communication skills, including making structured telephone calls to patients following discharge, may improve transitions of care. Objective : To explore associations between a patient-centered care (PCC) curriculum and patients’ perspectives of the quality of transitional care. Methods : We implemented a novel PCC curriculum on one of four inpatient general medicine resident teaching teams in which interns make post-discharge telephone calls to patients, contact outpatient providers, perform medication adherence reviews, and engage in patient-centered discharge planning. Between July and November of 2011, we conducted telephone surveys of patients from all four teaching teams within 30 days of discharge. In addition to asking if patients received a call from their hospital physician (intern), we administered the 3-Item Care Transitions Measure (CTM-3), which assesses patients’ perceptions of preparedness for the transition from hospital to home (possible score range 0–100). Results : The CTM-3 scores (mean±SD) of PCC team patients and standard team patients were not significantly different (82.4±17.3 vs. 79.6±17.6, p =0.53). However, regardless of team assignment, patients who reported receiving a post-discharge telephone call had significantly higher CTM-3 scores than those who did not (84.7±16.0 vs. 78.2±17.4, p =0.03). Interns exposed to the PCC curriculum called their patients after discharge more often than interns never exposed (OR=2.78, 95% CI [1.25, 6.18], p =0.013). Conclusions : The post-discharge telephone call, one element of PCC, was associated with higher CTM-3 scores – which, in turn, have been shown to lessen patients’ risk of emergency department visits within 30 days of discharge. Keywords: patient-centered care; graduate medical education; post-discharge telephone call; care transitions (Published: 29 April 2015) Citation: Med Educ Online 2015, 20: 26701 - http://dx.doi.org/10.3402/meo.v20.26701

BACKGROUND Access to hand-carried ultrasound technology for noncardiologists has increased significantly, yet development and evaluation of training programs are limited. OBJECTIVE We studied a focused program to teach hospitalists image acquisition of inferior vena cava (IVC) diameter and IVC collapsibility index with interpretation of estimated central venous pressure (CVP). METHODS Ten hospitalists completed an online educational module prior to attending a 1-day in-person training session that included directly supervised IVC imaging on volunteer subjects. In addition to making quantitative assessments, hospitalists were also asked to visually assess whether the IVC collapsed more than 50% during rapid inspiration or a sniff maneuver. Skills in image acquisition and interpretation were assessed immediately after training on volunteer patients and prerecorded images, and again on volunteer patients at least 6 weeks later. RESULTS Eight of 10 hospitalists acquired adequate IVC images and interpreted them correctly on 5 of the 5 volunteer subjects and interpreted all 10 prerecorded images correctly at the end of the 1-day training session. At 7.4 ± 0.7 weeks (range, 6.9–8.6 weeks) follow-up, 9 of 10 hospitalists accurately acquired and interpreted all IVC images in 5 of 5 volunteers. Hospitalists were also able to accurately determine whether the IVC collapsibility index was more than 50% by visual assessment in 180 of 198 attempts (91% of the time). CONCLUSIONS After a brief training program, hospitalists acquired adequate skills to perform and interpret hand-carried ultrasound IVC images and retained these skills in the near term. Though calculation of the IVC collapsibility index is more accurate, coupling a qualitative assessment with the IVC maximum diameter measurement may be acceptable in aiding bedside estimation of CVP. Journal of Hospital Medicine 2013;8:711–714. © 2013 Society of Hospital Medicine

Immune modulators used to treat rheumatologic disease have diverse endocrine effects in patients with diabetes. Providers should be aware of these effects given that diabetes and rheumatologic disease overlap in prevalence and cardiovascular morbidity. In patients with type 1 diabetes, clinical trials have demonstrated that immune modulators used early in the disease can improve pancreatic function, though their efficacy in adults with longstanding autoimmune diabetes is unknown. In patients with type 2 diabetes, hydroxychloroquine is an effective antihyperglycemic and may be preferred for rheumatologic use in patients with difficult glycemic control. In patients without diabetes, hydroxychloroquine and tumor necrosis factor (TNF) inhibitors have been found to decrease diabetes incidence in observational studies. Additionally, dapsone and sulfasalazine alter erythrocyte survival resulting in inaccurate HbA1c values. These multifaceted effects of immune modulators create a need for coordinated care between providers treating patients with diabetes to individualize medication selection and prevent hypoglycemic events. More research is needed to determine the long-term outcomes of immune modulators in patients with diabetes.

Background and Objective:Background and Objective: Patient-centered care, which is dependent on knowing each patient as an individual, has been identified as a critical aspect of healthcare. The most effective and efficient methods to get to know patients as individuals have not been defined. Our aim was to identify questions and phrases that can be used by physicians to get to know their patients. Abstract: Methods:Methods: We surveyed 15 physicians who have been formally recognized for their clinical excellence to determine what questions or phrases they use when interviewing patients to get to know them as individuals. Abstract: Results:Results: A total of 28 questions or phrases were received from 13 physicians and were qualitatively analyzed and grouped into six major themes: (i) appreciation of the patient's concerns; (ii) personal relationships; (iii) hobbies and pleasurable activities; (iv) open-ended questions to learn about the patient; (v) work; and (vi) the patient's perspective on the patient-physician relationship. Abstract: Conclusion:Conclusion: This work identifies questions and phrases used by clinically excellent physicians to get to know their patients as people. Future work should focus on obtaining the perspectives of patients, and on examining whether using the identified questions and phrases results in an improved patient experience as demonstrated by improved satisfaction ratings, ratings on the quality of physician-patient interaction, or patient outcomes.

If Irving Stone had written a book about giant-cell arteritis rather than about Michelangelo, he might have chosen The Ecstasy and the Agony as the more appropriate title. As many physicians know, diagnosing giant-cell arteritis and witnessing the patient’s dramatic initial response to treatment are much more fulfilling than managing a disease that lasts for months or years and that leads to the use of glucocorticoids in doses that result in a litany of side effects, including weight gain, hypertension, diabetes, and osteoporosis. This vexing challenge of treating giant-cell arteritis explains why doctors and patients will welcome the results of . . .

Objective The traditional physical examination of the heart is relatively inaccurate. There is little information regarding whether cardiac hand-carried ultrasound performed by noncardiologists adds to the accuracy of physical examinations. The purpose of this study was to determine whether hand-carried ultrasound can add to the accuracy of hospitalists' cardiac physical examinations. Methods During a focused training program in hand-carried echocardiography, 10 hospitalists performed cardiac examinations of 354 general medical inpatients first by physical examination and then by hand-carried ultrasound. Eligible inpatients included those for whom a conventional hospital echocardiogram was ordered. We measured how frequently the hospitalists' cardiac examination with or without hand-carried ultrasound matched or came within 1 scale level of an expert cardiologist's interpretation of the hospital echocardiogram. Results Adding hand-carried ultrasound to the physical examination improved hospitalists' assessment of left ventricular function, cardiomegaly, and pericardial effusion. For left ventricular function, using hand-carried ultrasound increased the percentage of exact matches with the expert cardiologist's assessment from 46% to 59% ( P =.005) and improved the percentage of within 1-level matches from 67% to 88% ( P =.0001). The addition of hand-carried ultrasound failed to improve the assessments of aortic stenosis, aortic regurgitation, and mitral regurgitation. Conclusion Adding hand-carried ultrasound to physical examination increases the accuracy of hospitalists' assessment of left ventricular dysfunction, cardiomegaly, and pericardial effusion, and fails to improve assessment of valvular heart disease. The clinical benefit achieved by improved immediacy of this information has not been determined. An important limitation is that the study assessed only 1 level of training in hand-carried ultrasound.

PURPOSE: Because the training that noncardiologists require to perform cardiac hand-carried ultrasound has not been defined, we studied how well hospitalists perform hand-carried echocardiography after limited training. METHODS: Ten hospitalists completed a focused training program that included performing an average of 35 hand-carried echocardiograms. Hospitalists' echocardiograms were compared with gold-standard conventional echocardiograms, and hospitalists were compared with 5 certified echocardiography technicians in their ability to acquire, measure, and interpret hand-carried ultrasound images and with 6 senior cardiology fellows in their ability to interpret echocardiograms. RESULTS: Echocardiography technicians had significantly higher performance scores for image acquisition, measurement, and interpretation than hospitalists. Senior cardiology fellows outperformed hospitalists in most aspects of image interpretation. For hospitalists, learning image acquisition was more difficult than image interpretation. CONCLUSIONS: Hospitalists can learn aspects of hand-carried echocardiography, but after 35 training echocardiograms cannot replicate the quality of conventional echocardiography. Whether the lower performance skills are important will depend on the clinical context of hand-carried echocardiography performed by hospitalists.

Objective To determine aspects of quality of life (QOL) important to people with giant cell arteritis (GCA). Methods We explored the domains of QOL affected by GCA in audiotaped focus groups. We then created an Importance Rating Questionnaire (IRQ) by constructing questions related to the domains most frequently mentioned. Of 214 GCA patients to whom the IRQ was sent, 145 (68%) responded. We calculated frequencies of responses and then ranked items by the proportion selecting the top category of importance and also according to a mean item rank. We compared the domains of QOL covered by the IRQ with those in the Short Form 12 (SF-12). Results The highest rated QOL item was “losing sight in both eyes permanently.” Of the top 20 items, 12 were in domains not covered directly by the SF-12. Conclusion We have identified aspects of QOL important to GCA patients. Assessment of QOL in GCA should include vision and other domains that are not included in standard QOL questionnaires.

Objective To evaluate treatment with methotrexate (MTX) in patients with newly diagnosed giant cell arteritis (GCA) to determine if MTX reduces GCA relapses and cumulative corticosteroid (CS) requirements and diminishes disease- and treatment-related morbidity. Methods This was a multicenter, randomized, double-blind study. Over 4 years, 16 centers from the International Network for the Study of Systemic Vasculitides enrolled patients with unequivocal GCA. The initial treatment was 1 mg/kg/day (≤60 mg every day) prednisone, plus either 0.15 mg/kg/week MTX (increased to 0.25 mg/kg/week, for a maximum weekly dosage of 15 mg) or placebo. Two physicians, both blinded to treatment allocation, evaluated each patient at every trial visit. One physician was responsible for providing global medical care. The other assessed GCA status according to a standard protocol. Treatment failure was defined as 2 distinct relapses or persistence of disease activity after the first relapse, in spite of increased CS therapy. Results Ninety-eight patients were enrolled. No significant differences between treatment groups were noted with regard to age, frequency of positive findings on temporal artery biopsy (placebo 87%, MTX 79%), or comorbidities at the time of enrollment. The median dosage of MTX was 15 mg/week. The incidence of treatment failure was comparable between groups after 12 months: 57.5% in the MTX group failed treatment (95% confidence interval [95% CI] 41.6–73.4%) compared with 77.3% in the placebo group (95% CI 61.9–92.8%) (P = 0.26). In a Cox regression analysis, MTX was not associated with a reduced risk of treatment failure (relative risk 0.72; 95% CI 0.41–1.28). There were no significant differences between groups with regard to abnormal elevations of the erythrocyte sedimentation rate following initial remissions, serious morbidity due to GCA, cumulative CS dose, or treatment toxicity. In the MTX group, there were fewer cases of GCA relapse heralded by symptoms of isolated polymyalgia rheumatica (1 case versus 5 in the placebo group; P = 0.05). Conclusion The results of this randomized, multicenter trial do not support the adjunctive use of MTX to control disease activity or to decrease the cumulative dose and toxicity of CS in patients with GCA.

Background/Aims: We examined the correlations between renal biopsy findings in Wegener’s granulomatosis and renal function at baseline and 1 year in 22 patients who presented between 1988 and 1999. Methods: Renal histology was independently reviewed by 2 pathologists who were masked to the clinical data. The primary outcome was the relationship of the percentage of normal glomeruli to reciprocal serum creatinine at baseline and 1 year. Other histologic data were collected using a series of ordinal rating scales. Acute and chronic sum scores were calculated. Results: The median serum creatinine (SCr) at baseline was 3.9 mg/dl (0.8–14 mg/dl). Seven patients initially required hemodialysis, of whom 4 subsequently regained independent renal function. By 1 year of follow-up, the median (SCr) for patients with independent renal function was 1.9 mg/dl (0.9–6.8 mg/dl). Reciprocal SCr at baseline correlated with the percentage of normal glomeruli (r = 0.584, p = 0.005), crescent score (r = –0.595, p = 0.003), interstitial fibrosis as a percentage of cortical surface area (r = –0.669, p = 0.002), interstitial inflammation (r = –0.439, p = 0.041), eosinophil (r = –0.495, p = 0.019), neutrophil score (r = –0.557, p = 0.005), and acute sum score (r = –0.499, p = 0.018). However, only the percentage of normal glomeruli (r = 0.493, p = 0.023), the crescent score (r = –0.452, p = 0.035), and interstitial fibrosis as a percentage of cortical surface area (r = –0.466, p = 0.052) correlated with reciprocal SCr at 1 year of follow-up. Conclusion: In this relatively small data set, the percentage of normal glomeruli, the crescent score, and interstitial fibrosis as a percentage of cortical surface area correlated with renal function at both baseline and 1 year of follow-up.

Giant cell arteritis (GCA), the most common form of systemic vasculitis in adults, preferentially involves large and medium-sized arteries in patients over the age of 50. The classic manifestations are headache, jaw claudication, polymyalgia rheumatica (PMR), and visual symptoms, but 40% of patients present with a wide range of occult manifestations. Early diagnosis and treatment with prednisone can prevent blindness, the most feared complication of GCA. The pathogenesis of GCA is T-cell dependent and antigen driven. Clinical subsets of GCA appear to result from variable cytokine expression. The risk of developing thoracic aortic aneurysm is increased more than 17-fold in patients with GCA. GCA can also involve large arteries, especially the subclavian and axillary arteries. Color Doppler ultrasound, magnetic resonance imaging, and positron-emission tomography scanning are providing insights into the extent and pathogenesis of the disease but have not replaced temporal artery biopsy as the gold standard for securing the diagnosis. Two recently completed double-blind, placebo-controlled trials concerning whether methotrexate plus prednisone is more effective than prednisone alone reached conflicting conclusions.

Spiral computed tomographic angiography (CTA) coupled with three-dimensional volume-rendering image processing is a less invasive alternative to conventional catheter angiography. The technique has been used successfully in a variety of vascular diseases. In this pictorial essay, we review the CTA findings in selected cases of vasculitis. Technical considerations and the potential clinical value of this method are discussed.

Objective To evaluate the safety of etanercept (Enbrel) in patients receiving conventional treatment for Wegener's granulomatosis (WG). Methods We performed a 6-month open-label trial of etanercept (25 mg subcutaneously twice weekly) which was added to standard therapies for WG (glucocorticoids, methotrexate, cyclophosphamide, azathioprine, cyclosporine) and prescribed according to disease severity. Evaluations of clinical response were determined by the Birmingham Vasculitis Activity Score for WG (BVAS/WG) in 20 patients with persistently active disease or with new flares of previously established WG. Fourteen of the 20 patients (70%) had etanercept added as the only new therapeutic variable. Results Injection site reactions (ISRs) were the most common adverse event related to etanercept (8 episodes in 5 patients [25%]

Objective To refine and validate the Birmingham Vasculitis Activity Score (BVAS) as a disease-specific activity index for Wegener's granulomatosis (WG). Methods Sixteen members of the International Network for the Study of the Systemic Vasculitides (INSSYS) revised the BVAS, with 3 goals: to reduce the redundancy of some component items, to enhance its ability to capture important disease manifestations specific to WG, and to streamline the instrument for use in clinical research. We defined the items and weighted them empirically as either minor (e.g., nasal crusting = 1 point) or major (e.g., alveolar hemorrhage = 3 points). We then validated the new, disease-specific BVAS/WG in 2 simulation exercises and a clinical case series that involved 117 patients with WG. Results We removed 38 items from the original BVAS, revised 9 items, and added 7 new items. Correlations between the scores on the BVAS/WG and the physician's global assessment (PGA) of disease activity were high, even when patients in remission were excluded. In the clinical case series, Spearman's rank correlation coefficient between the BVAS/WG and the PGA was r = 0.81 (95% confidence interval 0.73–0.87). The interobserver reliability using intraclass (within-case) correlation coefficients in the 2 simulation exercises was r = 0.93 for the BVAS/WG and r = 0.88 for the PGA in the first and r = 0.91 for the BVAS/WG and r = 0.88 for the PGA in the second. There was no significant observer effect in the scoring of the BVAS/WG or the PGA. The discriminant validity of the BVAS/WG was good: r = 0.73 (95% confidence interval 0.43–0.83). Conclusion The BVAS/WG is a valid, disease-specific activity index for WG. Tested in simulation exercises and in actual patients, the BVAS/WG correlates well with the PGA, is sensitive to change, and has good inter- and intraobserver reliability. The INSSYS will use the BVAS/WG to assess the primary outcome in a phase II/III trial of etanercept in WG.

Objective To examine the test characteristics of immunofluorescence (IF) and enzyme-linked immunosorbent assays (ELISA) in a consecutive series of patients under evaluation for anti-neutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV). Methods Using stored sera, we performed a cross-sectional study on 856 consecutive patients tested prospectively for ANCA by IF. Based on guidelines from the 1994 Chapel Hill Consensus Conference (CHCC), we determined each patient's underlying diagnosis by a medical records review without regard to their ANCA status (the CHCC guidelines do not require ANCA as a prerequisite for diagnosis). We grouped patients with forms of vasculitis commonly associated with ANCA into one of 4 types of AAV: Wegener's granulomatosis (n = 45), microscopic polyangiitis (n = 12), Churg-Strauss syndrome (n = 4), and pauci-immune glomerulonephritis (n = 8). We also classified patients without clinical evidence of AAV (92% of all patients tested) into 5 predefined categories of disease (including “other”) and an additional category for no identifiable disease. In a blinded fashion, we then performed ELISAs on the stored serum for antibodies to proteinase-3 (PR3) and myeloperoxidase (MPO) and calculated the test characteristics for both ANCA assay techniques. Results Sixty-nine of the 856 patients (8.1%) had clinical diagnoses of AAV based on CHCC guidelines. The positive predictive value (PPV) of ELISA for AAV was superior to that of IF, 83% versus 45%. For patients with both positive IF tests and positive ELISA tests, the PPV increased to 88%. Both IF and ELISA had high negative predictive values (97% and 96%, respectively). Positive ELISA tests were associated with higher likelihood ratios (LR) than IF (54.2 [95% CI = 26.3, 111.5] versus 9.4 [95% CI = 6.9, 12.7]). The LR of both a positive IF and a positive ELISA was 82.1 (95% CI = 33.3, 202.5). Conclusions Compared with IF, an ELISA test for ANCA was associated with a substantially higher PPV and LR for AAV. This fact, combined with the greater sensitivity of IF, suggests that an effective testing strategy is to perform ELISA tests only on samples that are positive for ANCA by IF.

(2000). Wegener's Granulomatosis: Spectrum of CT Findings in Diagnosis, Disease Progression, and Response to Therapy. Critical Reviews in Diagnostic Imaging: Vol. 41, No. 4, pp. 279-313.

Objective To establish the validity of an index designed to measure activity in systemic necrotizing vasculitis (SNV). Methods The Vasculitis Activity Index (VAI) was designed to incorporate appropriately weighted clinical measurements that reflect disease activity in SNV. We performed a pilot study to guide the modification and subsequent testing of the initial design. The data necessary to calculate the VAI are direct ratings by a clinical observer of the degree of activity in 9 organ systems and 3 indirect measures of vasculitis activity. These data are recorded on 0–4 visual analog scales. Physician's global assessment (PGA) is used as the “gold standard” measurement of disease activity. The VAI was validated using 2 independent data sets: the questionnaire data set, derived from test case histories (“paper cases”) sent to 100 practicing rheumatologists, and the clinic data set, obtained from use of the VAI in 204 regular care visits of 74 patients with SNV. Results The VAI correlated highly with the PGA: Pearson's correlation coefficient R = 0.84 (95% confidence interval [95% CI] 0.80–0.88) for the questionnaire data set, and R = 0.92 (95% CI 0.90–0.94) for the clinic data set. The mean of the interobserver coefficients of variation for the test case histories was lower for the VAI than for the PGA (mean difference 0.45; P = 0.002), indicating that the VAI has less interobserver variation than does the PGA. The change in VAI between clinic visits for individual patients correlated highly with the change in PGA (R = 0.88, 95% CI = 0.83–0.91). The VAI data collection form requires about 1 minute to complete, including computation of the score. Conclusion The VAI is a valid measure of vasculitis activity that correlates highly with the PGA. In addition, the VAI has less interobserver variation than the PGA and has a high level of sensitivity to change over time. Additional testing of the VAI appears warranted.

Objective. To develop a compulsory ambulatory rotation in rheumatology for internal medicine residents and to evaluate the educational effect of this rotation. Method. All year 2 residents in the program participated in the rotation. The perceived quality of the learning experience was assessed by resident self-evaluation using a visual analog scale. Residents used a similar scale to rate their confidence in managing common rheumatologic problems. A validated multiple choice test was used to measure pre- and post-rotation knowledge of rheumatology and skill in interpreting bone radiographs. Results. On a 1 (no learning) to 5 (superior learning) scale, the mean rating of the rheumatology learning experience was 4.7 ± 0.5. Residents' confidence in clinical skills significantly increased as well. Scores on the post-rotation rheumatology knowledge test increased 15% compared with prerotation scores (P < 0.0001). Confidence and skills in interpreting bone radiology films also significantly improved (P < 0.0001 for both items). Conclusion. A relatively brief, subspecialty ambulatory rotation can be developed to expose all residents to a rheumatology curriculum. Such a rotation can increase their confidence, knowledge, and skills in important domains of outpatient rheumatology.

Although hindfoot pathology in rheumatoid arthritis is a significant cause of disability for patients, the etiology of the planovalgus deformity is controversial. The present study surveys 99 patients with clinically proven rheumatoid arthritis for the presence and severity of hindfoot pathology. Specific attention was directed at the function of the posterior tibial tendon, as disruption of this structure has been implicated by some investigators as a cause of hindfoot deformity in rheumatoid arthritis. Assessment of posterior tibial function was by manual testing using two different grading scales, as well as by examination for several signs associated with posterior tibial tendon dysfunction. Between 13% and 64% of the study population could be considered to have posterior tibial tendon dysfunction, depending upon the specific diagnostic criteria used. Using the presence of all three of the most stringent criteria for diagnosis, 11% of patients were believed to have posterior tibial tendon dysfunction. These criteria were loss of the longitudinal arch, inability to perform a heel-rise, and lack of a palpable posterior tibial tendon. This study demonstrates that planovalgus deformity in rheumatoid arthritis can be due to clinically evident dysfunction of the posterior tibial muscle-tendon unit. There is a complex interplay between hindfoot joint disruption due to the inflammatory process and deformity due to tendinous dysfunction. If there is primary subtalar joint instability secondary to the inflammatory process, the posterior tibial tendon is rendered dysfunctional due to deranged hindfoot mechanics, as with primary posterior tibial tendon rupture. Since treatment of either condition (i.e., primary hindfoot instability or primary posterior tibial tendon rupture) is similar, the distinction is not important clinically. What is important is that attention to the specific cause of planovalgus would be expected to improve the overall treatment of rheumatoid feet.

Background Asymptomatic left ventricular systolic dysfunction is an important risk factor for heart failure and death. Given the availability of patients, trained personnel, and equipment, the hospital is an ideal setting to identify and initiate treatment for left ventricular systolic dysfunction. The purpose of this study was to determine the prevalence of asymptomatic left ventricular systolic dysfunction in patients 45 years of age or older with at least one clinical heart failure risk factor admitted to a general medical service. Methods Bedside, hand-carried echocardiography provided quantitative assessment of left ventricular systolic function in 217 medical inpatients 45 years of age or older who had at least one heart failure risk factor. Patients with known or suspected heart failure or with an assessment of left ventricular function in the past 5 years were excluded. We measured the prevalence of asymptomatic left ventricular systolic dysfunction, defined by left ventricular ejection fraction of 50% or lower, and its association with heart failure risk factors. Results Of 207 patients with interpretable images, 11 (5.3%) had a left ventricular ejection fraction of 50% or lower. Patients with left ventricular systolic dysfunction had more heart failure risk factors than those without left ventricular systolic dysfunction (3.09±0.8 vs 2.5±1.0, P =.04). The total number of heart failure risk factors trended towards an association with a greater prevalence of asymptomatic left ventricular systolic dysfunction, but this did not reach significance (odds ratio 1.74; 95% confidence interval, 0.97-3.12, P =.06). Conclusions Asymptomatic left ventricular systolic dysfunction is present in about 1 of every 20 general medical inpatients with at least one risk factor for heart failure. Because treatment of asymptomatic left ventricular systolic dysfunction may reduce morbidity, further studies examining the costs and benefits of using hand-carried ultrasound to identify this important condition in general medical inpatients are warranted.

Patient-centered care is an important aspect of medicine. The Johns Hopkins Initiative Curriculum aims to teach residents to view patients as individuals and to consider the context of their lives outside the hospital in order to provide patient-centered, and safe, care across transitions. Specifically, this curriculum was developed to reform graduate medical education by teaching residents to: (1) provide more patient-centered care by understanding the psychosocial context of each patient's illness, and knowing patients as individuals; (2) optimize care across transitions from the hospital to postdischarge setting; and, (3) educate and empower patients in shared decision-making about treatments. This curriculum is meant to be administered over a 4-week period with residents receiving both didactic instruction and observation by attending physicians during resident-patient interactions. Instruction is provided across seven content areas consisting of: (1) medication adherence, (2) pharmacy curriculum, (3) call to next provider of care, (4) patient-centered discharge, (5) telephone contact with patients after discharge, (6) follow-up visits with patients after discharge (home and sub-acute visits), and (7) the challenging provider-patient relationship. Each area is accompanied by detailed administration directions in an accompanying Instructor's Guide. We are currently engaged in studies of the impact of this curriculum on learners and patients. In addition to providing more patient-centered care, we hope to demonstrate benefits in other educational outcomes, including medical knowledge, self-awareness, systems-based practice, and communication skills. We plan to assess whether patients report better relationships with their physicians and an improved understanding of their medical conditions and care. We also hope to demonstrate improved clinical outcomes such as reduced 30-day readmission rates and increased follow-through with recommended treatments and studies after discharge.

Although the causes of giant cell arteritis, polymyalgia rheumatica, and Takayasu's arteritis are not known, the immune system participates in the pathogenesis of these disorders. Many reports indicate that cellular immunity is active in giant cell arteritis. The diagnosis of Takayasu's arteritis has been advanced by magnetic resonance imaging and ultrasonography. Neither technique has replaced angiography as the gold standard for diagnosis, but both may help monitor the response of Takayasu's arteritis to therapy. The diagnosis of giant cell arteritis with a low erythrocyte sedimentation rate is unusual but is more frequent in patients with a history of polymyalgia rheumatica or corticosteroid use. One recent retrospective study suggests that 20 mg/d of prednisolone is as effective as higher doses in treating giant cell arteritis. Other preliminary studies indicate that methotrexate may effectively spare corticosteroid use in giant cell arteritis and Takayasu's arteritis. Angioplasty for renal artery stenosis in Takayasu's arteritis initially cures 30% of patients and improves the condition in 70%; however, the long-term success rate is 50%. New studies demonstrate that patients with giant cell arteritis and polymyalgia rheumatica have an increased risk of hypothyroidism.

Primary angiitis of the central nervous system (PACNS) is a rare form of vasculitis of unknown cause. The mean age of onset is 50 years, and men are affected twice as often as women. Headache and encephalopathy are the most frequent initial symptoms. Stroke or focal symptoms develop in less than 20% of patients at the onset of disease and are uncommon in the absence of headache or encephalopathy. Symptoms or signs of vasculitis outside of the central nervous system are rare; serologic markers of inflammation are typically normal. Magnetic resonance imaging of the brain is abnormal in more than 90% of patients, but the pattern of abnormal findings is not specific. Cerebrospinal fluid analysis is also usually abnormal because of modest, nonspecific elevations in total protein level or white blood cell count. Angiography has a low sensitivity and low specificity. Most patients suspected of having PACNS have another disease. The diagnosis of PACNS is established by brain biopsy. The differential diagnosis of PACNS is broad and includes reversal cerebral vasoconstriction. In contrast to patients with PACNS, patients with reversal cerebral vasoconstriction are more often young women who experience a thunderclap headache and have a normal cerebrospinal fluid analysis. Patients with biopsy-proven PACNS are treated with cyclophosphamide and prednisone.

OBJECTIVE. Delayed contrast-enhanced MRI is increasingly being used for cardiac viability imaging. Takayasu's arteritis is a rare inflammatory disorder of unknown cause that affects the aorta, its major branches, and the pulmonary artery; it is characterized by inflammation and fibrosis in the arterial wall. We report our initial experience with seven patients (six women, one man; age range, 25–62 years) with delayed (20 min) gadolinium-enhanced MRI (inversion recovery prepared gated fast gradient-echo pulse sequence) in patients with known Takayasu's arteritis.CONCLUSION. Patients with Takayasu's arteritis (particularly those with abnormal laboratory values) have evidence of delayed hyperenhancement on delayed contrast-enhanced MRI. Thus, delayed contrast-enhanced MRI might be a useful technique to identify inflammation in arterial wall.

Purpose Because there is little information about the training that general internists require to perform hand-carried cardiac ultrasonography (HCU), we studied the rate of learning of a group of medical residents performing HCU after minimal formal training. Methods Medical residents on the inpatient services at Johns Hopkins Bayview Medical Center received formal training in HCU consisting of 15-30 minutes of didactic instruction about the principles of echocardiography, followed by ongoing one-on-one instruction in performing HCU and subsequent ongoing one-on-one training from a certified echocardiography technician as they were doing scans. The residents were shown how to position the patient to obtain 2-dimensional echo images from the parasternal short and long axes and apical 4-chamber views, and how to obtain color-flow Doppler images across the mitral and aortic valves. Residents were asked to determine whether pericardial effusion was present and to assess left ventricular size, left ventricular function, and the mitral and aortic valves. The residents performed cardiac physical examination and HCU independently on patients who had a conventional transthoracic echocardiogram (CTTE) performed within 24 hours of the HCU. The residents' HCU results were compared with the CTTE results by a cardiologist specializing in echocardiography. The rates at which residents gained technical proficiency and skills in interpreting their studies were measured by linear regression to fit various outcome variables against their experience at scanning as gauged by the number of scans performed. Results Thirty medical residents performed a total of 231 HCU studies. Linear regression models showed that the residents' overall technical proficiency skills improved at the rate of 0.79 (95% confidence interval [CI] 0.53-1.04) points on an overall assessment index (0-3 scale) per 10 scans completed. Interpretation accuracy improved at a rate of 1.01 (95% CI 0.69-1.39) points per 10 scans as measured by an interpretation accuracy index (0-3 scale). Because scanning efforts and instruction in HCU occurred during residents' usual rotation duties, some residents gathered experience in HCU slowly and sporadically. Conclusion This study, the first prospective, experimental effort of its kind, shows that residents as a group learned important aspects of HCU scanning and interpretation at a reasonably rapid rate.

This year's Update in Rheumatology includes discussions of advances related to rheumatoid arthritis, systemic lupus erythematosus, Wegener granulomatosis, antineutrophil cytoplasmic antibody–associ...

Objective To examine the reported correlation between the presence of Chlamydia pneumoniae in temporal artery biopsy specimens and the diagnosis of temporal arteritis (TA). Methods Among 90 possible cases of TA identified at our institution between 1968 and 2000, 79 of the positive biopsy specimens (88%) demonstrated giant cells and the other 11 cases (12%) had other histopathologic features compatible with TA; by chart review, all 90 patients were confirmed to have met the American College of Rheumatology classification criteria for TA. Controls had negative temporal artery biopsy specimens during the same 32-year time period and their postbiopsy disease courses were not compatible with TA. Controls were matched with each case by sex, year of biopsy, and age within 10 years. The biopsy specimens from all cases and controls were reevaluated and readings were confirmed in a masked manner by an experienced eye pathologist. Polymerase chain reaction (PCR) analyses for C pneumoniae were performed on the 180 samples using 2 different sets of PCR primers (which target 2 different genes). A primer set targeting the ompA gene (CP1-CP2/CPC-CPD) was used to perform a nested PCR, followed by confirmation of the findings with primers targeting the 16S ribosomal RNA (rRNA) gene (Cpn90/Cpn91) in a touchdown-enzyme time-release PCR. We used positive and negative controls, as well as controls made from infected and noninfected HEp-2 cells, suspended in a formalin-fixed, paraffin-embedded matrix. Results Seventy-six percent of the 180 cases and controls were women. The mean age of the cases was 72.0 years (range 53–90), and that of the controls was 70.4 years (range 51–86). Eighty percent of the control samples were obtained by temporal artery biopsy performed within 1 year of the biopsies performed on the matched cases. Using the CP1-CP2/CPC-CPD primer set, only 1 TA case sample (1% of all case samples) was positive for the ompA gene. One control sample was also positive using these primers. With the Cpn90/Cpn91 primers, none of the cases and none of the controls were positive for the 16S rRNA gene. Conclusion The results of this study using sensitive and specific PCR analyses do not support a role for C pneumoniae in the pathogenesis of TA.

The two principal aims in the treatment of Wegener's granulomatosis (WG) are to limit the extent and severity of permanent organ damage by controlling the disease promptly and to minimize the short- and long-term morbidity that often results from therapy. Remission is considered to be the absence of disease activity in any organ system. Once the disease has been controlled by the initial treatment regimen, which is dictated by the degree of disease severity, the focus of therapy shifts to maintaining disease remission, often with medications less toxic than those used to induce remission. The description of WG treatments in terms analogous to cancer chemotherapy (i.e., those designed to induce remissions and those intended to maintain them) is useful in the formulation of current disease management strategies and in the investigation of new therapies for WG.

OBJECTIVE. We attempt to determine whether angiography is indicated in patients with suspected central nervous system (CNS) vasculitis who present with negative findings on MR imaging studies.CONCLUSION. MR imaging findings may be negative in the setting of CNS vasculitis confirmed on angiography, indicating that advanced imaging techniques tailored to detect infarction (i.e., fluid-attenuated inversion recovery, diffusion-weighted, and perfusion imaging) may be necessary to enhance the sensitivity of an MR study and that despite the high sensitivity of MR imaging for CNS vasculitis, angiography may still be required to render an accurate diagnosis.

PURPOSE: To report the development of a bilateral ocular ischemic syndrome despite corticosteroid treatment in a patient with giant cell arteritis. METHOD: Case report. RESULTS: Despite receiving high-dose intravenous methylprednisolone and oral prednisone for biopsy-proven giant cell arteritis that presented as a severe anterior ischemic optic neuropathy in the right eye, a patient developed progressive ocular ischemia in that eye as well as an ocular ischemic syndrome in the fellow eye. CONCLUSIONS: Some patients with giant cell arteritis, possibly patients with other underlying systemic vasculopathies, are refractory to what should be adequate treatment with systemic corticosteroids and may develop a bilateral ocular ischemic syndrome.

We report the case of a 41-year-old man with chronic meningitis and cranial neuropathies in whom leptomeningeal biopsy demonstrated Wegener's granulomatosis. The literature on meningitis in this disease is reviewed. This case illustrates that Wegener's granulomatosis can cause chronic meningitis.

The purpose of this study was to examine the prevalence of foot and ankle problems in 99 patients with clinically proven rheumatoid arthritis. Patients were recruited from outpatient rheumatology clinics; no attempt was made to select patients on the basis of the severity of their disease, duration of disease, or symptom constellation. Each patient was examined by an investigator utilizing a predesigned protocol to assess their functional status, functional capacity, and overall joint involvement. Ninety-three of 99 patients had complaints referable to the foot or ankle at some time since diagnosis of rheumatoid arthritis. Ankle problems were paramount in 42%, forefoot difficulties in 28%, and equal ankle and forefoot problems in another 14%. Only four patients had had any treatment involving foot orthotic devices or special shoe wear. The prevalence of foot and ankle symptoms was related to the duration of systemic illness, but was present in > 50% of patients at any time after diagnosis of rheumatoid arthritis. Patients with longstanding rheumatoid arthritis have a high prevalence of foot and ankle symptoms. Unlike previous reports, the present study found a high prevalence of ankle and hindfoot symptoms, as opposed to forefoot complaints. Despite this finding, the patients had been treated infrequently by either conservative nonoperative management directed at accommodating footwear or surgical intervention to favorably alter their foot and ankle mechanics.

We sought to determine whether creatinine clearance (C cr ) gives an accurate measurement of the glomerular filtration rate in patients with systemic lupus erythematosus (SLE) with no clinical evidence of renal disease. Eighteen such SLE patients underwent measurement of C cr and 99m technetium-DTPA clearance (C DTPA ). The mean ±SD C cr :C DTPA ratio was 1.12±0.15 (95% confidence interval 1.04-1.20). These results indicate that C cr gives an accurate measurement of the glomerular filtration rate in the majority of SLE patients who do not have clinical evidence of renal disease

Inflammatory abdominal aortic aneurysm (AAA) accounts for 5% to 10% of all cases of AAA and differs from typical atherosclerotic AAA in many important ways. Although both inflammatory and atherosclerotic AAA most commonly affect the infrarenal portion of the abdominal aorta, patients with the inflammatory variant are younger and usually symptomatic, chiefly from back or abdominal pain. Unlike patients with atherosclerotic AAA, most with the inflammatory variant have an elevated erythrocyte sedimentation rate or abnormalities of other serum inflammatory markers. Computed tomography and magnetic resonance imaging are both sensitive for demonstrating the cuff of soft tissue inflammation surrounding the aneurysm that is characteristic of inflammatory AAA. In contrast to atherosclerotic AAA, the inflammatory variant is characterized pathologically by marked thickening of the aneurysm wall, fibrosis of the adjacent retroperitoneum, and rigid adherence of the adjacent structures to the anterior aneurysm wall. An extraordinary expansion of the adventitia due to inflammation also distinguishes inflammatory from atherosclerotic AAA. Although the pathogenesis of inflammatory AAA appears to involve an immune response localized to the vessel wall, the etiology of the inflammatory reaction is unknown. Inflammatory AAA is almost never associated with inflammation of other arteries. Male sex and smoking, the main risk factors for atherosclerotic AAA, are even stronger risk factors for the inflammatory variant. Smoking cessation is the first step of medical therapy. Corticosteroids or immunosuppressive therapies may also have roles. Although inflammatory AAA appears less likely to rupture than atherosclerotic AAA, surgical intervention appears prudent once the diameter of the aneurysm exceeds 5.5 cm. Knowing the features of inflammatory AAA should allow physicians to distinguish it from atherosclerotic AAA or from systemic vasculitis and to treat it with the appropriate combination of medical and surgical therapies.