Carcinoma of the lung in progressive systemic sclerosis: a tabular review of the literature and a detailed report of the roentgenographic changes in two cases.

The association of various pathologic changes in the lung, especially interstitial fibrosis, in progressive systemic sclerosis is a recognized occurrence. Usually it is not the site of development of a malignancy. As a corollary the literature contains a number of examples of carcinoma developing in the lung in patients with idiopathic basilar fibrosis unrelated to PSS. Currently, we have reviewed the available literature and found a satisfactory description of 44 cases of carcinoma of the lung in patients with progressive systemic sclerosis. Also, there are brief descriptions of 10 cases of pulmonary carcinoma and PSS. The development of a malignancy superimposed upon the chronic changes in the lung, associated with the systemic sclerosing process may be related pathogenically to an altered immunologic response.

Progressive systemic sclerosis (PSS) and malignancy, pulmonary and non-pulmonary.

In contrast to the 15-20% incidence of the coexistence of acute dermatomyositis-polymyositis and malignancy, it has been accepted traditionally that the association of progressive systemic sclerosis, a disease with several features that may overlap the former condition, and malignancy is purely fortuitous. This experience has not been altered by the material presented in this review. However, the factual coexistence has been illuminated by a review of the pertinent literature and presentation of 12 previously unpublished case reports. Four cases concern pulmonary malignancies in PSS; eight are of an associated non-pulmonary malignancy. In the first group, the development of a malignancy superimposed on the chronic fibrosing changes in the lungs of PSS does not seem so strange, particularly in view of a possible immunologic reaction by collagen in considering pathogenesis. This immunologic process might be similar to a related immunologic process responsible for the development of malignant cells in pulmonary and other tissue, where normal cells usually are found. The high incidence of males is related to the high incidence of males in Veterans Administration Hospitals, the principal population source of these cases.