Collagen Disease and Cancer

The association of cancer with other disease states has intrigued clinicians and investigators of recent years. Para-endocrine syndromes caused by carcinoma of the lung provide dramatic examples of this relationship (1). Recent reports in the literature (2–4) describe the successive occurrence in the same patient of a “collagen” disease such as lupus erythematosus, dermatomyositis, or scleroderma, and lymphoma, myeloma, or carcinoma. Furthermore, certain in-bred strains of mice in which spontaneous lymphoma and autoimmune hemolytic anemia arise have provided an experimental model for study of this association (5). One's interest in a correlation between collagen disease and malignant tumors quickens when we are informed that a disturbance in an immunoglobulin mechanism may be a common factor in both groups of diseases (3). Case Reports Case I: D. S., a 57-year-old Caucasian male, was treated in 1962 for dermatomyositis2 proved by skin biopsy. The chest film showed nothing abnormal. His symptoms were controlled by corticosteroids for two years. In 1964 a large mass developed in the right supraclavicular fossa, which proved to be metastatic anaplastic carcinoma. A recent chest film showed a tumor in the right hilus (Fig. 1). Case II: J. N., a 47-year-old Caucasian female, presented with systemic lupus erythematosus which began in 1953. Gastrointestinal examination in 1956 showed disordered motor function of the small bowel thought to be due to vascular lesions which may occur in this “collagen disease ”(Fig. 2). In 1964, enlarged lymph nodes and spleen developed, and the patient died of acute lymphocytic leukemia. Case III: In T. F., a 47-year-old Caucasian female, scleroderma was proved by skin biopsy in 1956. A chest film at that time showed diffuse interstitial pulmonary fibrosis (Fig. 3). In 1964, she died of carcinoma of the lung which infiltrated the left upper lobe (Fig. 4). Discussion Normal immune mechanisms depend on normally functioning lymphocytes and plasmacytes. These cells proliferate whenever “foreign” antigenic material invades the body and then produce globulins which act as specific antibodies to these antigens. The thymus is the main source of lymphocytes, and its major role in the phenomenon of immunity has been recognized only recently (3). It may happen that abnormal lymphocytes proliferate and subsequently produce abnormal globulins, some of which may react with the host's own tissues to produce an “autoimmune state.” Clinical diseases such as periarteritis nodosa, rheumatoid state, lupus erythematosus, and scleroderma are derived from this situation. Similarly, some malignant conditions such as multiple myeloma and chronic lymphocytic leukemia are usually accompanied by large amounts of “degraded“ globulins or by lymphocytes which react abnormally to antigen stimulation (Fig. 5).