Abstract:
OBJECTIVE:To prospectively study the clinical features, pathophysiology, treatment and prognosis of Wegener granulomatosis. DESIGN:Of the 180 patients with Wegener granulomatosis referred to the National Institute of Allergy and Infectious Diseases during the past 24 years, 158 have been followed for 6 months to 24 years (a total of 1229 patient-years). MEASUREMENTS:Characteristics of clinical presentation, surgical pathology, course of illness, laboratory and radiographic findings, and the results of medical and surgical treatment have been recorded in a computer-based information retrieval system. SETTING:The Warren Magnuson Clinical Center of the National Institutes of Health. MAIN RESULTS:Men and women were equally represented; 97% of patients were white, and 85% were more than 19 years of age. The mean period of follow-up was 8 years. One hundred and thirty-three patients (84%) received "standard" therapy with daily low-dose cyclophosphamide and glucocorticoids. Eight (5.0%) received only low-dose cyclophosphamide. Six (4.0%) never received cyclophosphamide and were treated with other cytotoxic agents and glucocorticoids. Ten patients (6.0%) were treated with only glucocorticoids. Ninety-one percent of patients experienced marked improvement, and 75% achieved complete remission. Fifty percent of remissions were associated with one or more relapses. Of 99 patients followed for greater than 5 years, 44% had remissions of greater than 5 years duration. Thirteen percent of patients died of Wegener granulomatosis, treatment-related causes, or both. Almost all patients had serious morbidity from irreversible features of their disease (86%) or side effects of treatment (42%). CONCLUSIONS:The course of Wegener granulomatosis has been dramatically improved by daily treatment with cyclophosphamide and glucocorticoids. Nonetheless, disease- and treatment-related morbidity is often profound. Alternative forms of therapy have not yet achieved the high rates of remission induction and successful maintenance that have been reported with daily cyclophosphamide treatment. Despite continued therapeutic success with cyclophosphamide, our long-term follow-up of patients with Wegener granulomatosis has led to increasing concerns about toxicity resulting from prolonged cyclophosphamide therapy and has encouraged investigation of other therapeutic regimens.
journal_name
Ann Intern Medjournal_title
Annals of internal medicineauthors
Hoffman GS,Kerr GS,Leavitt RY,Hallahan CW,Lebovics RS,Travis WD,Rottem M,Fauci ASdoi
10.7326/0003-4819-116-6-488subject
Has Abstractpub_date
1992-03-15 00:00:00pages
488-98issue
6eissn
0003-4819issn
1539-3704journal_volume
116pub_type
杂志文章abstract::The clinical implications of newly acquired left bundle-branch block (LBBB) were examined prospectively in the Framingham Study population. During 18 years of observation 55 people developed LBBB. The mean age at the onset of LBBB was 62; LBBB occurred largely in people with antecedent hypertension, cardiac enlargemen...
journal_title:Annals of internal medicine
pub_type: 临床试验,杂志文章
doi:10.7326/0003-4819-90-3-303
更新日期:1979-03-01 00:00:00
abstract:BACKGROUND:Few recent data are available on risk factors for perioperative supraventricular arrhythmia (SVA) after noncardiac surgery or on the effect of SVA on clinical outcomes. OBJECTIVE:To determine the incidence, clinical correlates, and effect on length of stay of perioperative SVA in patients having major nonca...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-129-4-199808150-00003
更新日期:1998-08-15 00:00:00
abstract:BACKGROUND:Valid measurement of physician performance requires accurate identification of patients for whom a physician is responsible. Among all patients seen by a physician, some will be more strongly connected to their physician than others, but the effect of connectedness on measures of physician performance is not...
journal_title:Annals of internal medicine
pub_type: 杂志文章,多中心研究
doi:10.7326/0003-4819-150-5-200903030-00008
更新日期:2009-03-03 00:00:00
abstract::Three cases of carbamazepine-induced granulomatous hepatitis are reported. Each patient had ingested carbamazepine for less than 1 month before presenting with a febrile illness suggestive of biliary tract infection. After withdrawal of carbamazepine, symptoms disappeared rapidly. Histologically, all patients had gran...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-95-1-64
更新日期:1981-07-01 00:00:00
abstract:STUDY OBJECTIVE:To describe the clinical and epidemiologic features of Vibrio vulnificus infections. DESIGN:Case series based on notifiable disease report forms and patient medical records. SETTING:Cases reported to the Florida Department of Health and Rehabilitative Services from 1981 to 1987. PATIENTS:Sixty-two pa...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-109-4-318
更新日期:1988-08-15 00:00:00
abstract::Increased adrenocorticotropic hormone (ACTH) levels after bilateral adrenalectomy could be secondary to a pituitary tumor, under replacement with cortisol, or an abnormality in the hypothalamic-pituitary-adrenal feedback loop. To distinguish between these possibilities, ACTH levels were measured before and after corti...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-96-3-277
更新日期:1982-03-01 00:00:00
abstract::Fifty-three consecutive patients with inferior myocardial infarction were evaluated prospectively, by physical examination and right heart catheterization within 36 hours of the onset of symptoms, to determine whether physical findings can separate such patients into those with and without associated right ventricular...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-99-5-608
更新日期:1983-11-01 00:00:00
abstract:BACKGROUND:The risk for venous thromboembolism during pregnancy or postpartum is uncertain. OBJECTIVES:To estimate the relative and absolute risk for deep venous thrombosis and pulmonary embolism during pregnancy and postpartum and to describe trends in incidence. DESIGN:Population-based inception cohort study using ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-143-10-200511150-00006
更新日期:2005-11-15 00:00:00
abstract::The diagnostic and therapeutic value of the angiotensin converting enzyme inhibitor teprotide (SQ 20881) was assessed in 18 patients with hypertensive emergencies. Mean blood pressure fell 31 +/- 18 mm Hg in the 10 subjects who responded to 1 mg/kg body weight administered intravenously, whereas it fell 5 +/- 3 mm Hg ...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-90-1-43
更新日期:1979-01-01 00:00:00
abstract::Patients with diabetes mellitus and coronary artery disease are thought to have painless myocardial ischemia more often than patients without diabetes. We studied 50 consecutive patients with diabetes and 50 consecutive patients without diabetes, all with ischemia, on exercise thallium scintigraphy to show the reliabi...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-108-2-170
更新日期:1988-02-01 00:00:00
abstract::Physical symptoms account for more than half of all outpatient visits, but the specific causes and treatments of many common symptoms are frequently not well established. Because clinical research has primarily targeted specific diseases, symptoms-based research is still an emerging field of scientific inquiry, and op...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-134-9_part_2-200105011-00008
更新日期:2001-05-01 00:00:00
abstract::In deciding whether to undertake a dietary program to reduce serum cholesterol levels, a person must consider how much benefit to expect. We developed a model that assumes cholesterol reduction is effective and safe in reducing the risk for death from ischemic heart disease. In the model, we considered asymptomatic ad...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-106-4-605
更新日期:1987-04-01 00:00:00
abstract::Evidence on the safety and efficacy of methotrexate as a second- or third-line agent for treating patients with rheumatoid arthritis is reviewed. Four placebo-controlled clinical trials have documented short-term benefit from methotrexate; although true remission is rare, patients receiving methotrexate showed a 26% (...
journal_title:Annals of internal medicine
pub_type: 临床试验,杂志文章,评审
doi:10.7326/0003-4819-107-2-358
更新日期:1987-09-01 00:00:00
abstract::From 1969 to 1974, 19 cases of Serratia marcescens endocarditis were observed in the San Francisco Bay Area. Seventeen patients were intravenous drug users, and Serratia caused 14% of all addict-associated endocarditis in San Francisco. Serratia strains were nonpigmented and had typical antibiotic sensitivities, excep...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-84-1-29
更新日期:1976-01-01 00:00:00
abstract::Implantable cardioverter defibrillators are life-saving devices for many patients with cardiac disease. Recipients of these devices, nevertheless, often suffer from progressive comorbid and cardiac conditions. Therefore, physicians should anticipate situations in which the defibrillator is no longer desired by the pat...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-142-8-200504190-00012
更新日期:2005-04-19 00:00:00
abstract:BACKGROUND:Memphis and Shelby County, Tennessee, experienced an epidemic of hepatitis A in 1994 and 1995. More than 1700 cases were reported. OBJECTIVE:To characterize the clinical features of patients hospitalized during a large urban epidemic of hepatitis A. DESIGN:Retrospective chart review. SETTING:15 acute care...
journal_title:Annals of internal medicine
pub_type: 杂志文章,多中心研究
doi:10.7326/0003-4819-128-2-199801150-00006
更新日期:1998-01-15 00:00:00
abstract:OBJECTIVE:To evaluate the risk for chronic renal disease associated with regular use of nonaspirin nonsteroidal anti-inflammatory drugs (NSAIDs). DESIGN:Multicenter case-control study. PATIENTS:Patients were 554 North Carolina residents (age range, 30 to 79 years) hospitalized between 1980 and 1982 with a discharge d...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-115-3-165
更新日期:1991-08-01 00:00:00
abstract::Prospective data at Framingham and elsewhere have shown conclusively that risk of coronary heart disease in persons younger than age 50 is strikingly related to the serum total cholesterol level. Within so-called normal limits risk has been found to mount over a five-fold range. The impact has been found to be augment...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-90-1-85
更新日期:1979-01-01 00:00:00
abstract::Recently acquired knowledge about vitamin D metabolism has improved our understanding in different varieties of osteomalacia. Many new causes of osteomalacia continue to be found. Radiologic and biochemical changes are not always characteristic and may occasionally be misleading. Bone biopsy after a double tetracyclin...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-89-6-966
更新日期:1978-12-01 00:00:00
abstract::To assess whether echocardiographic and electrocardiographic detection of left ventricular hypertrophy could predict cardiovascular morbid events in patients with uncomplicated essential hypertension, we followed 140 men for a mean of 4.8 years. Initial echocardiographic measurements of left ventricular mass were norm...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-105-2-173
更新日期:1986-08-01 00:00:00
abstract:BACKGROUND:Although antiviral therapy is cost-effective in adults, its cost-effectiveness in older adults has not been studied. OBJECTIVE:To determine the cost-effectiveness of influenza testing and treatment strategies for older adults. DESIGN:Cost-utility decision model. DATA SOURCES:Clinical trials of antiviral d...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-139-5_part_1-200309020-00007
更新日期:2003-09-02 00:00:00
abstract:BACKGROUND:Neurologic complications of endocarditis can influence diagnosis, therapeutic plans, and prognosis. OBJECTIVE:To describe how early cerebral magnetic resonance imaging (MRI) affects the diagnosis and management of endocarditis in hospitalized adults. DESIGN:Single-center prospective study between June 2005...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-152-8-201004200-00006
更新日期:2010-04-20 00:00:00
abstract:STUDY OBJECTIVE:To test the hypothesis that work as a respiratory therapist is associated with an increased risk of developing asthma. DESIGN:Cross-sectional questionnaire study comparing respiratory therapists with controls (physical therapists and radiologic technologists). Subsequent validation of reported asthma w...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-110-10-767
更新日期:1989-05-15 00:00:00
abstract:STUDY OBJECTIVE:To determine the usefulness of the continuous intravenous dexamethasone suppression test in the differential diagnosis of patients with the Cushing syndrome. DESIGN:Comparison of the results of this test with those of other tests in the study population and with data from the literature. SETTING:Refer...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-112-10-738
更新日期:1990-05-15 00:00:00
abstract::A 42-year-old woman developed goitrous hypothyroidism while receiving ethionamide for treatment of an atypical mycobacterial infection. Five weeks after discontinuation of ethionamide therapy, thyroid function had returned to normal. The goitrogenic effect was studied in vitro by incubating ethionamide in various conc...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-100-6-837
更新日期:1984-06-01 00:00:00
abstract::Very low calorie diets used to treat moderate and severe obesity produce average weight losses of 20 kg in 12 weeks. This paper reviews the development of very low calorie diets from research on fasting in the late 1950s and examines data on the amount of dietary protein needed to achieve positive nitrogen balance. Th...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-99-5-675
更新日期:1983-11-01 00:00:00
abstract:BACKGROUND:Preventive therapy is an important element of syphilis control efforts. No currently recommended, single-dose alternatives to penicillin G benzathine are available for treatment of incubating syphilis. OBJECTIVE:To evaluate the use of a single 1.0-g dose of azithromycin for treatment of persons recently exp...
journal_title:Annals of internal medicine
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.7326/0003-4819-131-6-199909210-00007
更新日期:1999-09-21 00:00:00
abstract::Sideroblastic anemia after treatment for Hogdkin's disease was seen in two patients 3 years after completion of radiation therapy and chemotherapy. This was followed in both by the development of myelomonoblastic leukemia. No evidence of recurrent Hodgkin's disease was present in either patient. Our observation sugges...
journal_title:Annals of internal medicine
pub_type: 杂志文章
doi:10.7326/0003-4819-92-5-625
更新日期:1980-05-01 00:00:00
abstract:PURPOSE:To review the electropharmacology, clinical applications, side effects, and hemodynamic profile of intravenous amiodarone. DATA SOURCES:The MEDLINE database was searched for English-language material, including reports of clinical trials and in vivo studies, review articles, and abstracts presented at national...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-127-4-199708150-00007
更新日期:1997-08-15 00:00:00
abstract:PURPOSE:To integrate information from the various disciplines that contribute to the understanding of the cause and prevention of sudden cardiac death: identification of new approaches from applied clinical epidemiology; identification and control of transient risk factors; and evaluation of the results of intervention...
journal_title:Annals of internal medicine
pub_type: 杂志文章,评审
doi:10.7326/0003-4819-119-12-199312150-00006
更新日期:1993-12-15 00:00:00