A Case-Control Etiologic Study of Sarcoidosis (ACCESS)
Objectives:
To determine the etiology of sarcoidosis by
establishig a case control, multi-center study. In addition to etiology, this
study also sought to examine socioeconomic variables and the clinical course of
patients with sarcoidosis, including quality of life.
Background:
Sarcoidosis is a chronic granulomatous disorder of
unkonwn cause that is characterized by activation of T-lymphocytes and
macrophages. For many years sarcoidosis was presumed to be an atypical
manifestation of tuberculosis because of the similarity between the
inflammatory responses of the two diseases. However, as culture techniques
became more widely employed to diagnose tuberculosis and it became less common,
it became clear that sarcoidosis was not simply a variation of tuberculosis.
Data on the etiology of sarcoidosis have come from diverse sources: in clinical
investigations, alveolitis has been found to precede granulomatous
inflammation; in case control studies, familial aggregation has been
identified; and in case reports, recurrence of granulomatous inflammation has
been observed after lung transplantation. The cause may not prove to be a
single, known exposure. Interactions of exposures with genetic dispositions
could have important implications for our understanding of immune responses as
well as the pathogenesis of sarcoidosis.
Subjects:
736 patients with sarcoidosis enrolled within 6
months of diagnosis from 10 clinical centers in the U.S. Using the ACCESS
sarcoidosis assessment system, organ involvement was determined for the whole
group and for subgroups differentiated by sex, race, and age (<40 or 40 and
older). Cases were matched with a control, and there was a two-year follow-up
on cases. The ACCESS group proposed an instrument fo defining organ involvement
in sarcoidosis. Biological specimens included DNA, plasma, and bronchoalveolar
lavage samples were obtained. The Limited Access Data set includes 718 cases,
686 controls, and two-year follow-up data on 210 cases.
Conclusions:
The initial presentation of sarcoidosis is related to
sex, race and age, and it tends to remain stable over two years in the majority
of patients. The etiology is probably multifactoral with both genetic and
environmental factors contributing.
Publications (as of June 9, 2004):
- Judson M. et al. Defining Organ Involvement in
Sarcoidosis: The ACCESS Proposed Instrument. Sarcoidosis, Vasculitis and
Diffuse Lung Diseases. 1999; 16:75-86.
- ACCESS Research Group. Design of a Case Control
Etiologic Study of Sarcoidosis (ACCESS). Journal of Clinical Epidemiology.
1999; 52:1173-1186.
- Baughman R, et al. Clinical Characteristics of
Patients in a Case Control Etiologic Study of Sarcoidosis. American Journal of
Respiratory and Critical Care Medicine, 2001; 164:1885-1889.
- Rybicki B, et al. Familial Aggregation of
Sarcoidosis: A Case Control Etiologic Study of Sarcoidosis (ACCESS). American
Journal of Respiratory and Critical Care Medicine, 2001; 164:2085-2091.
- Pandey JP, et al. TNF-a, IL1-ß, and
Immunoglobulin (GM and KM) Gene Polymorphisms in Sarcoidosis. Human Immunology
2002; 63:485-91.
- Judson M, et al. The Diagnostic Pathway to
Sarcoidosis. Chest 2003;' 123:406-12.
- Brown ST, et al. Recovery of Cell Wall-Deficient
Organisms from Blood Does Not Distinguish between Patients with Sarcoidosis and
Control Subjects. Chest 2003; 123: 413-17.
- Judson, MA, et al. Two-year Prognosis of
Sarcoidosis. The ACCESS experience. Sarcoidosis, Vasculitis and Diffuse Lung
Disease 2003; 20:204-11.
- Rossman MD et al. HLA-DRB1* 1101: A significant
risk factor for sarcoidosis in blacks and whites. Am J Hum Genet 2003; 73(4):
720-34.
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Study Website |
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Study Documentation |
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Data Distribution Agreement |
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