Diffuse alveolar damage as the initial presentation of dermatomyositis and successfully treated with intravenous immunoglobulin and steroids: A case report

Background: Severe rapid progressive respiratory involvement as the initial manifestation of dermatomyositis is rare, but may be life threatening. Diffuse alveolar damage is the worst of the pulmonary findings in dermatomyositis patients because of its poor response to therapy and poor prognosis; almost all patients die. Case: The subject is a 37-year-old female complaining of mild dyspnea lasting three weeks and followed with symmetrical polyarthritis and very mild proximal myalgia one week after the onset of dyspnea. Moreover, the heliotrope sign was transient and very faint. A chest X-ray revealed diffuse bilateral infiltration and laboratory findings showed significantly elevated muscle enzymes. The muscle biopsy revealed typical myositis change. One week after hospitalization, the respiratory condition rapidly progressed to respiratory failure and mechanical ventilation support was required. Open lung biopsy demonstrated interstitial pneumonia with diffuse alveolar damage. Intravenous immunoglobulin therapy was initiated in conjunction with high dose steroids. The patient was successfully weaned from the ventilator after 10 days and was treated with two more courses of monthly intravenous immunoglobulin and gradually tapering oral steroids. Conclusions: Diffuse interstitial pneumonitis is an unusual presenting pattern for dermatomyositis. The response of the symptoms and lung signs to intravenous immunoglobulin and high dose glucocorticoid, in this case, is important to note because it seemed to be an easy solution for a potentially lethal condition.