Mortality of Intrathoracic Sarcoidosis in Referral vs Population-Based Settings: Influence of Stage, Ethnicity, and Corticosteroid Therapy

doi:10.1378/chest.121.1.32

Chest

Volume 121, Issue 1, January 2002, Pages 32-39

https://doi.org/10.1378/chest.121.1.32 Get rights and content

Study objectives

To compare the sarcoidosis mortalityin referral settings (RS) and population-based settings (PS), and toidentify the contribution of stage, ethnicity, and corticosteroidtherapy (CST) to their disparate outcomes.

Design

Allobservational studies identified in a MEDLINE search and bibliographicreview published in the English language since 1960 dealing with thecourse and prognosis of sarcoidosis in large, unsorted, adult,ambulatory RS and PS providing long-term follow-up were reviewed andsubjected to meta-analysis.

Measurements and results

Sarcoidosis mortality in RS (4.8%), in which 17% of patients had themost unfavorable prognosis as judged by stage (stage III), was 10-foldthat reported in PS (0.5%), in which 11% of patients were identifiedat this stage. The magnitude of this disparity could not be accountedfor solely by adverse selection, as indicated by stage or by ethnicity.Patients in RS received CST with sevenfold the frequency of PS, and itsprovision was highly correlated with stage-normalized mortality.

Conclusion

The prognosis of patients with intrathoracicsarcoidosis in PS is far more favorable than that obtained in RS.Sarcoidosis mortality is largely independent of ethnicity. Thepossibility cannot be excluded that excessive employment of CST mayunfavorably influence the long-term course of the disease in someindividuals.

Section snippets

Materials and Methods

All reports dealing with the long-term course and prognosis ofsarcoidosis originating in large, outpatient adult practices in Westerncountries, unsorted by chronicity, classified according to stage, andidentified in a MEDLINE search and bibliographic survey of publicationsin the English language from 1960 through 2000, were reviewed andallocated by origin in RS or PS. The search terms employed weresarcoidosis AND course, AND prognosis, AND outcome. Compendia based onhospitalized patients were

Meta-analysis and Review

Ten reports fulfilling the inclusion criteria wereidentified⁴⁵⁶⁷⁸⁹¹⁰¹¹¹²¹³; they are summarized in Tables 1,2.

Discussion

Imprecision of the available data, in combination with varyingmeans of data presentation, constitute the major limitation of thisstudy. Cumulative mortality is an imprecise estimate of mortality rate(which was not supplied), neither the dose or duration of CST wasprovided, no study supplied details of physiologic impairment, and onlyone study¹⁰ detailed the radiographic extent of disease.Patient age and duration of follow-up were supplied by differentauthors in noncomparable formats: range,

Conclusions

(1) Sarcoidosis mortality representative of patients who arelikely to be encountered by community physicians in western countriesis 0.5%, less than one tenth of that reported from RS; (2) althoughethnic differences strongly influence the incidence and clinicalmanifestations of sarcoidosis, they have little appreciable influenceon its mortality; and (3) the possibility that a lower threshold foradministering CST is associated with an increased mortality risk,beyond the selection effect that

References (21)

LE Siltzbach et al.
Course and prognosis of sarcoidosis around the world
Am J Med
(1974)
M Sones et al.
Course and prognosis of sarcoidosis
Am J Med
(1960)
JM Reich et al.
Course and prognosis of sarcoidosis in a nonreferral setting: analysis of 86 patients observed for 10 years
Am J Med
(1985)
E Huhti et al.
Prognosis for sarcoidosis in a defined geographical area
Br J Dis Chest
(1987)
D Bunn et al.
A ten year study of sarcoidosis
Br J Dis Chest
(1972)
NM Gideon et al.
Sarcoidosis mortality in the United States, 1979–1991
Am J Med
(1996)
RG Crystal
Sarcoidosis
RS Fraser et al.
American Thoracic Society, European Respiratory Society, World Association of Sarcoidosis and Other Granulomatous Diseases. Statement on sarcoidosis
Am J Respir Crit Care Med
(1999)
H Smellie et al.
The natural history of pulmonary sarcoidosis
Q J Med
(1960)

There are more references available in the full text version of this article.

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Chest

Clinical InvestigationsSarcoidMortality of Intrathoracic Sarcoidosis in Referral vs Population-Based Settings: Influence of Stage, Ethnicity, and Corticosteroid Therapy

Study objectives

Design

Measurements and results

Conclusion

Section snippets

Materials and Methods

Meta-analysis and Review

Discussion

Conclusions

Am J Med

Am J Med

Am J Med

Br J Dis Chest

Br J Dis Chest

Am J Med

Sarcoidosis

American Thoracic Society, European Respiratory Society, World Association of Sarcoidosis and Other Granulomatous Diseases. Statement on sarcoidosis

Am J Respir Crit Care Med

The natural history of pulmonary sarcoidosis

Q J Med

Clinical Investigations
Sarcoid
Mortality of Intrathoracic Sarcoidosis in Referral vs Population-Based Settings: Influence of Stage, Ethnicity, and Corticosteroid Therapy