Update in rheumatology.
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Biomedical subjects
Publications and source records attributed to David B Hellmann.
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Being a physician is rewarding but also challenging in the complex health care system. As physicians, we are continually trying to deliver more effective and higher quality care to our patients. With improvement in mind, a list of precepts has been generated as a tool to remind all of us in clinical medicine about the exemplary characteristics, behaviors, and attitudes that are expected as the norm in this profession. The list is organized into four categories: promotion of relationships with patients, principles of the effective clinician, growth and improvement, and values to guide one's career in medicine. The list is envisioned as an instrument that may be helpful to medical learners and physicians by promoting reflection about ways to consistently perform at a high level while more fully appreciating the joy of practicing medicine. The list of precepts may also be useful to medical educators who wish to successfully mold the physicians of tomorrow.
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.
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 hyper-enhancement on delayed contrast-enhanced MRI. Thus, delayed contrast-enhanced MRI might be a useful technique to identify inflammation in arterial wall.
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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.
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Temporal arteritis, the most common form of systemic vasculitis in adults, is a panarteritis that chiefly involves the extracranial branches of the carotid artery. The condition is illustrated in this article by the case of a 79-year-old woman with a dry cough, toothache, tongue infarction, and vision loss. The mean age of onset is 72 years and the disease rarely occurs in persons younger than 50 years. The most common presenting manifestations are headache, jaw claudication, polymyalgia rheumatica, and visual symptoms. Eighty-nine percent of patients have an erythrocyte sedimentation rate greater than 50 mm/h. However, about 40% of patients present with atypical manifestations, including fever of unknown origin, respiratory tract symptoms (especially dry cough), and large artery involvement. Familiarity with such unusual manifestations of temporal arteritis facilitates early diagnosis and treatment, thereby reducing the risk of vision loss.
PURPOSE: Gastrointestinal involvement in polyarteritis nodosa carries a poor prognosis. A 1982 review from our institution reported acute abdominal syndromes in 31% of patients with polyarteritis nodosa, and that all 5 patients with acute surgical abdomens died. We reviewed our more recent experience to determine if outcomes have changed since. SUBJECTS AND METHODS: We reviewed the records of all patients with polyarteritis nodosa in our vasculitis database between 1986 and 2000. Inclusion criteria were a diagnosis of polyarteritis nodosa, symptoms or signs of gastrointestinal involvement, and either a mesenteric angiogram consistent with polyarteritis nodosa or histopathologic proof of a medium-vessel vasculitis. We calculated a prognostic (5 factor) score for all patients. RESULTS: We identified 24 patients with polyarteritis nodosa who had gastrointestinal involvement during their illness. Thirteen (54%) of the patients developed acute surgical abdomens, 3 of whom died (P = 0.02 by comparison with the historical cohort). Mean (+/- SD) prognostic scores were higher among patients in the acute abdomen group compared with those who did not have acute abdominal syndromes (1.7 +/- 0.9 vs. 0.6 +/- 0.7, P = 0.002), corresponding with the observed mortality in these groups. CONCLUSION: Gastrointestinal involvement occurs commonly in polyarteritis nodosa and carries a poor prognosis. Compared with a historical cohort at our institution, mortality from this complication may have decreased, perhaps because of earlier diagnosis.
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 (<or=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.
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.
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.
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.