2000
DOI: 10.1093/rheumatology/39.8.914
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Respiratory failure due to muscle weakness in inflammatory myopathies: maintenance therapy with home mechanical ventilation

Abstract: Polymyositis and dermatomyositis are idiopathic inflammatory myopathies. Respiratory complications are a common feature, but ventilatory insufficiency is rare in these patients. We describe here three patients diagnosed with inflammatory myopathy (polymyositis) with respiratory failure due to muscle weakness who did not respond to immunosuppressive therapy. Mechanical ventilation at home with nasal or tracheal intermittent positive pressure resulted in improved chronic hypoventilation. This treatment improves … Show more

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Cited by 45 publications
(28 citation statements)
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“…PM is a rare paraneoplastic syndrome of breast cancer. Moreover, respiratory muscle involvement in PM is uncommon, and fatal respiratory failure is especially extremely rare [19]. No similar cases have been described in literature, so we believe this is the first reported case of a patient developing respiratory failure requiring mechanical assistant ventilation as a complication of breast cancer related polymyositis.…”
Section: Discussionmentioning
confidence: 74%
See 1 more Smart Citation
“…PM is a rare paraneoplastic syndrome of breast cancer. Moreover, respiratory muscle involvement in PM is uncommon, and fatal respiratory failure is especially extremely rare [19]. No similar cases have been described in literature, so we believe this is the first reported case of a patient developing respiratory failure requiring mechanical assistant ventilation as a complication of breast cancer related polymyositis.…”
Section: Discussionmentioning
confidence: 74%
“…Respiratory muscle weakness and hypercapnea should be treated by mechanical ventilation and corticosteroids. When treatment with corticosteroids fails, the patient should be treated with agents such as methotrexate, azathioprine, cyclophosphamide, intravenous immune globulin, cyclosporine, tacrolimus, alkylating agents, and tumor necrosis factor inhibitors [19,21].…”
Section: Discussionmentioning
confidence: 99%
“…Maintenance therapy with noninvasive positive pressure ventilation improves quality of life, and, in severe cases, tracheostomy with home mechanical ventilation can be lifesaving. 14 …”
Section: Ventilatory Failure Caused By Muscle Weaknessmentioning
confidence: 99%
“…One case of antisynthetase syndrome had isolated respiratory muscle weakness [102] (although it should be emphasised that the more common cause of respiratory failure in these patients is interstitial lung disease). Other reports exist of patients having been admitted to the ICU for respiratory muscle weakness due to PM [103] or DM [104], (although it should be noted many of these presented acutely to hospital rather than with chronic ERF to ambulatory clinics); in the DM patients most of these were early in the course of their disease and the authors emphasise the high mortality for these patients [104]. There is also a case report of a patient with respiratory muscle weakness occurring 5 years after onset of limb symptoms in a patient with IBM [105].…”
Section: Inflammatory Myopathymentioning
confidence: 99%