Malignancy in the setting of the anti-synthetase syndrome

Malignancy and interstitial lung disease (ILD) are 2 conditions associated with dermatomyositis (DM) that are responsible for a significant portion of the morbidity and mortality related to this disease; however, they rarely occur in the same patient. The antisynthetase syndrome consists of several characteristics, including ILD, arthritis, Raynaud phenomenon, "mechanic's hands," and positive antibodies to tRNA synthetases, which have each been negatively associated with cancer. When patients with DM present with such characteristics, clinicians may be falsely reassured that a thorough malignancy screen is unnecessary. We describe a patient who presented with the antisynthetase syndrome and was subsequently found to have colon cancer. Removal of the cancer led to resolution of the myositis and lung disease, but the patient's rash and arthritis persisted and ultimately required immunosuppressive therapy. We provide a review of the literature describing the concurrence of both this syndrome and ILD alone, with malignancy. We conclude that a thorough and expedited age-appropriate malignancy work up is indicated in all patients with a new diagnosis of DM, despite the presence of disease characteristics that are usually not associated with cancer.