| Summary: | The malignant pleural effusion in systemic sclerosis (SSc) is an uncommon condition than its common manifestation like interstitial lung fibrosis, pulmonary hypertension, and lung malignancy. We reported here a case of malignant pleural effusion in a 40-year-old female suffering from SSc with high serum tumor marker "cancer antigen-125 (CA-125)". Computed tomogram guided fine needle aspiration cytology from the lung nodule revealed adenocarcinoma lung. The frequency or reasons of an elevated level of a serum or pleural fluid CA-125 in this case are unclear. Excepting ovarian and lung malignancy, increased CA-125 level in serum, and pleural fluid is also detectable in nonmalignant conditions including SSc as a part of serositis. We emphasized here the malignant potential of SSc and the need for long-term follow-up to detect underlying malignancy. High CA-125 level might be interpreted cautiously.
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