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Sarcoidosis—recent progress in etiology and pathogenesis

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Abstract

DESPITE its recognition more than ninety years ago, and the development of an extensive literature representing investigative studies, by means of roentgenograms of the chest, and the decreasing incidence of tuberculosis, new interest has arisen in this widespread granulomatous disease—sarcoidosis.

The clinical manifestations and current therapy of sarcoidosis are well known and are beyond the scope of this paper. Many excellent reviews1–6 are available which summarize these aspects in detail. What perhaps is not so well known are the many small bits of information that support some of the recent concepts about the etiology and possible pathogenesis of this masquerader of many diseases.

The development of our present knowledge about sarcoidosis has been described as occurring in three stages.7 Originally recognized in the second half of the nineteenth century as a skin disease, it was not until Boeck8 in 1899 noted involvement in the lungs and named the process “sarkoid”, because he believed it was in some way related to benign sarcomas, that a new stage began, stressing the internal manifestations of the disease. In 1917, Schaumann’s9 important work was published, noting the generalized systemic involvement of the disease, of which skin lesions were only a small part. In the next several decades, experience was gained through numerous clinical observations, but there was little advancement in the understanding of the basic pathophysiology. Interest flourished, especially in the Scandinavian countries, in what was thought to be an atypical form of tuberculosis.

The third stage occurred after World War II.


 

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