Chronic graft-versus-host disease-related polymyositis as a cause of respiratory failure following allogeneic bone marrow transplant

Abstract

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...

Chronic graft-versus-host disease manifesting as polymyositis: an uncommon presentation

“…Sensory and motor nerve conduction are preserved. 11,14,18,22,23,26 The diagnosis of polymyositis can be established on the basis of characteristic clinical and EMG findings. In atypical presentations, a muscle biopsy may be required to confirm the diagnosis.…”

“…In 16 cases the steroid was used in combination with a variety of immunosuppressants, including: cyclosporine, intravenous immunoglobulin, azathioprine, antilymphocyte globulin, cyclophosphamide, methotrexate, thalidomide and photopheresis. 2,12,[14][15][16][17][18]24,26,27 Thirteen of 30 evaluable patients failed initial immunosuppression and are summarized in Table 1. Six patients were primarily resistant and seven had recurrent polymyositis while steroids were being tapered.…”

“…In 30 of these patients, polymyositis was associated with dermatological changes of GVHD. 2,12,[14][15][16][17][18][19][20][21][22][23][24][25][26] The onset of GVHD-associated polymyositis has been reported to occur between 100 days and 69 months after allogeneic HSCT, and about half of the patients do not have a history of acute GVHD. 21,23 Clinical manifestations of GVHD-associated polymyositis are indistinguishable from those of the idiopathic form polymyositis.…”

Reduced respiratory and skeletal muscle strength in survivors of sibling or unrelated donor hematopoietic stem cell transplantation

“…The literature to date is comprised mainly of small case series and case reports. 2,[14][15][16][17][18][19] In a previous study, we demonstrated respiratory and skeletal muscle weakness in up to 30% of subjects 1 before undergoing HSCT. This study extends these observations and demonstrates that significant respiratory and skeletal muscle weakness can still be detected in those Table 2 Respiratory and skeletal muscle strength and submaximal exercise testing in subjects following HSCT subjects undergoing PFT following HSCT.…”

“…An evaluation of skeletal and respiratory muscle strength had been added to routine PFT as a result of our experience with severe skeletal muscle weakness occurring following HSCT in the posttransplant period. 2 The additional testing of respiratory and skeletal muscle function was considered part of the clinical management of the patient undergoing HSCT starting in January 2002. The Institutional Review Board at T-NEMC approved this analysis of de-identified data, without obtaining informed consent from the subjects.…”