Course and prognosis of sarcoidosis in African-Americans versus Caucasians.
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Biomedical subjects
Publications and source records attributed to J M Reich.
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STUDY OBJECTIVE: To determine whether persons with asymptomatic bilateral hilar lymphadenopathy (ABHL) and normal results of a physical examination should be observed with a presumptive diagnosis of stage 1 sarcoidosis (S1S) (ABHLps), its most frequent cause, or undergo mediastinoscopy to avoid overlooking an alternative diagnosis (AD) requiring treatment. DESIGN: We surveyed the English-language medical literature to estimate the proportion of persons with tuberculosis (TB), Hodgkin's disease (HD), and non-Hodgkin's lymphoma (NHL) who present with ABHL and calculated the number of mediastinoscopies required to identify each AD by computing the following ratio: incidence S1S/incidence of each AD presenting as ABHL (I(S1S)/I[ABHL-AD]). Risks of mediastinoscopy and benefits of earlier ascertainment of AD were derived from the published literature. Cost estimates were based on institutional charges. We conducted a regional survey of practicing pulmonologists to ascertain their diagnostic preferences. RESULTS: We estimate that if 33,000 persons with ABHL underwent mediastinoscopy, 32,982 (99.95%) would be found to have S1S or, very rarely, a disorder not requiring intervention; 407 would require hospitalization for complications at a cost in excess of $1 million; and 204 would experience major morbidity; 8 persons with TB, 9 with HD, and 1 with NHL would be identified at a cost of $100 to $200 million. The benefit for persons diagnosed as having AD would be minimal and likely offset by the procedural mortality. Seventy percent of pulmonologists responding to the survey favored observation over transbronchial lung biopsy or mediastinoscopy in patients with ABHL. CONCLUSION: A policy of continued observation of patients presenting with ABHL is preferable to diagnostic mediastinoscopy from both the risk/benefit and cost/benefit standpoint.
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The purpose of this study was to estimate the incidence of clinically identified (i.e., ascertained either because of symptoms or an incidental chest radiograph) sarcoidosis in a geographically and ethnically defined United States population. We employed a research-quality database of a large health maintenance organization (HMO), Kaiser Permanente, Northwest Region (KPNW), which collects morbidity and utilization data for a random sample of members sociodemographically representative of the service area of the HMO, to identify persons with suspected or confirmed sarcoidosis. Nine clinically identified incident cases of sarcoidosis were verified within the 1.87*10(5) person-year sample over a 21-year span, 1967-1987, an estimated annual all-ethnicity incidence rate of 4.8/10(5) (95 percent confidence interval ((CI)): 1.7, 7.9). Among persons of Caucasian ancestry, the estimated annual incidence was 2.8/10(5) (95 percent CI: 0.4, 5.2). If the incidence among Caucasians and the relative risk of developing sarcoidosis among African-Americans in the KPNW population is representative of the U.S. population, we estimate the incidence of clinically identified sarcoidosis in the latter to be 7.3. If, in addition, clinically identified cases constitute 42 percent of those that are clinically ascertainable (i.e., identified by mass population screening in addition to cases detected because of symptoms or incidental chest radiographs), as observed in the Swedish population, our estimate of the U.S. incidence of clinically ascertainable sarcoidosis would be 17.4.
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STUDY OBJECTIVE: To determine whether a reported association between sarcoidosis and malignancy can be supported. DESIGN: In this retrospective survey in a general community setting, we crossmatched the Kaiser Permanente Northwest Region (KPNW) Tumor Registry comprising 3 x 10(4) cases observed over 32 years against a sarcoidosis registry of 243 cases observed over 24 years. We used linkage criteria, eg, previously reported associated malignancies, late age onset of sarcoidosis, and close temporal proximity, to identify joint occurrences suggesting an etiologic relationship. We assessed the feasibility of detecting an association of sarcoidosis and Hodgkins disease (HD) by computing the required sample size based on incidence estimates of sarcoidosis and HD in this population. Medical records of 241 persons identified by the Tumor Registry as having HD were reviewed. SETTING: KPNW, a health maintenance organization. POPULATION: We studied 6.8 x 10(6) KPNW member-years from 1960 to 1992 at risk for malignancy; 5.6 x 10(6) member-years from 1971 to 1992 at risk for sarcoidosis. RESULTS: Six of the 11 (55%; 95% confidence interval, 26 to 84) correctly classified sarcoidosis and malignancy (S&M) cases met two or more linkage criteria. A seventh case, incorrectly classified as sarcoidosis, exhibited a necrotizing systemic granulomatous process, believed to represent a response to a fatal lymphoproliferative disorder. The mean age (40 years) of the 11 patients with S&M was 9 years higher than the mean age of the patients with sarcoidosis in the population from which they were drawn. The stage of sarcoidosis at diagnosis among the 11 S&M cases was exclusively 0 and I and thus divergent from expected values in this population. We found no instances of sarcoidosis accompanying HD. CONCLUSION: Linkage analysis provides evidence that S&M may be etiologically related in at least a quarter of cases in which both are present.