A controlled study of the clinical characteristics affecting survival in patients with rheumatoid arthritis (RA) with cervical spine involvement treated surgically demonstrated a strong association between disease severity, the frequency of severe extraarticular manifestations, especially interstitial lung disease, and a decreased probability of survival. These probabilities at 1 and 5 years postoperatively were 74 and 54%, respectively. Death resulted most often from infection or comorbid conditions. All surgery patients with interstitial lung disease died within 28 months postoperatively. In patients with RA undergoing cervical spine surgery, fatality rates appear to be increased in patients with severe extraarticular manifestations, especially interstitial lung disease.