PURPOSE: Polymyositis/Dermatomyositis (PM/DM) is an immunological disorder affecting skeletal muscles, skin, and other organs requiring prolonged treatment with immunosuppressive agents. We present findings in 34 consecutive autopsy cases encountered at Mayo Clinic Rochester.
METHODS: A computer-assisted database search identified 37 PM/DM patients with autopsy examination performed during a 25-year period (1981 to 2006) at our institution; 34 of these subjects had medical records available for current review.
RESULTS: Thirty-four patients consisted of 15 males and 19 females with a median age of 67 years (range, 21 to 88 years). All but 6 patients had a known diagnosis of PM-DM at the time of death. Immediate causes of death were: sepsis (7, 20%); bronchopneumonia (7, 21%); diffuse alveolar damage/ARDS (7, 21%); hemorrhagic shock (3, 9%); CHF (2, 6%); massive PE (2, 6%); myocardial infarction (2, 6%); progressive pulmonary fibrosis (3, 8%) and acute liver failure (1, 3%). Specific infectious causes included candida species (3), Pneumocystis jiroveci (3), cryptococcus neoformans (1), cytomegalovirus (1), and nocardia asteroides (1). Twenty-eight subjects with a known PM/DM had been diagnosed a median interval of 5 months (range, 1 to 144 months) before death. Prednisone was the most commonly used agent for treatment of PM-DM (25, 74%), followed by methotrexate (8, 24%). Pulmonary hypertension by echocardiography was found in 27% patients (6 of 22 with data available). Of all the patients dying of sepsis and /or pneumonia, 68 % were on immunosuppressive therapy, Respiratory causes of death (pneumonia, diffuse alveolar damage, and pulmonary fibrosis) accounted for 50% of all immediate causes of deaths.
CONCLUSION: Respiratory failure and infections are the most common causes of in hospital mortality in patients with PM-DM.
CLINICAL IMPLICATIONS: Better understanding of respiratory complications occurring in patients with PM/DM may lead to improved survival of these patients.
DISCLOSURE: Rajesh Patel, No Financial Disclosure Information; No Product/Research Disclosure Information