Summary:An unusual case of respiratory failure and dropped head syndrome as a complication of severe chronic graft-versus-host disease (GVHD)-related polymyositis is described. The patient required tracheostomy and mechanical ventilation but recovered following treatment with aggressive immunosuppression and intensive rehabilitation. The differential diagnoses of muscle weakness in the bone marrow transplant (BMT) patient and the dropped head syndrome are both discussed. To our knowledge, this is the first reported case of respiratory failure requiring mechanical ventilation occurring as a complication of GVHD-related polymyositis. Bone Marrow Transplantation (2000) 26, 1117-1120. Keywords: polymyositis; GVHD; respiratory failure; BMT; dropped head syndrome Respiratory failure in the setting of bone marrow transplantation (BMT) is usually acute in onset and occurs due to a number of well described but poorly understood clinical syndromes that occur in this patient population. The clinical syndromes include diffuse alveolar hemorrhage, 1 the idiopathic pneumonia syndrome, 2 septic shock, 3 pneumonia due to conventional and opportunistic organisms 4 or bronchiolitis obliterans.5 Chronic respiratory failure as a complication of muscle weakness in this patient population is not well described and appears to be very uncommon. Polymyositis is a recognized but unusual complication of chronic graft-versus-host disease (GVHD) due to BMT. 6 We report a patient who developed GVHD-related polymyositis that resulted in profound weakness of both the extensor muscles of the neck and muscles of respiration. The patient gradually developed respiratory failure and ultimately required a tracheostomy and mechanical ventilation. To our knowledge, GVHD as a cause of chronic respiratory failure has not been previously described. The differential diagnoses of respiratory muscle weakness in the BMT patient and the 'dropped head syndrome' are both discussed. Case reportA 37-year-old female who had undergone a matched unrelated allogeneic BMT 2 years prior to admission presented with a several month history of progressive weakness of the muscles of both shoulders, neck and thighs. She had difficulty performing simple tasks such as combing her hair and brushing her teeth. In addition, she had diffuse myalgias and dysphagia for both liquids and solids. Ten years previously she had been diagnosed with acute myeloid leukemia (M2) (AML) that responded to chemotherapy. The disease relapsed 7 years later and she was treated with induction chemotherapy, two cycles of consolidation followed by a matched (6/6) unrelated allogeneic BMT from an ABO incompatible donor. She received a standard conditioning regimen comprising cyclophosphamide and total body irradiation. Routine pulmonary function tests performed prior to BMT were all normal. Her course following BMT was complicated by grade 1 acute and then extensive chronic GVHD, involving primarily the oral mucosa and skin. This was treated with corticosteroids (prednisone 10-20 mg per day) and cyclos...

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