Pulmonary involvement in PSS is characterized by fibrosis of the lung, a condition that does not, normally, cause hemoptysis. Therefore, the manifestation of hemoptysis in a patient with PSS and lung involvement should not go unchecked. It is usually the presentation of a secondary lesion that is possibly malignant.
In 1953, the first report of an association between scleroderma and malignancy  described three patients, each with long- standing systemic sclerosis (PSS) and lung involvement. In all three patients carcinoma of the lung was found at necropsy.
Further case-reports [3-7] described a similar association between PSS and lung cancer, usually diagnosed in patients known to have had diffused scleroderma involving the lungs for many years prior to the appearance of the malignancy. . The most common type of lung cancer reported in connection with PSS is bronchiolar ("alveolar-cell") carcinoma [2-4, 9] (representing less than 4% of all forms of lung cancer). This type of cancer can be found in areas of chronic fibrosis and has been described in association with scarring.
Squamous cell carcinoma of the lung has been rarely reported in association with PSS (in nonsmok-ers) , and also oat-cell carcinoma . These cancers developed without long-standing pulmonary involvement with systemic sclerosis. Therefore, it has been suggested that these tumors were coincidental with the collagen disease, and not directly related to it. Another possibility raised regarding these cases was that the collagen disease represented an unusual paraneoplastic phenomenon ,
It is possible that cell products, including growth factors, might cause sclerodermatous changes , Scleroderma-like skin lesions have been described in some patients with metatstatic secreting carcinoid tumors , and also in multiple myeloma in which all test results for amyloid were negative .
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