This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Highlights A predominantly restrictive ventilatory pattern is common in myotonic dystrophy Respiratory dysfunction is associated with alveolar hypoventilation and chronic hypercapnia. There is no clear relationship between CTG repeat length and severity of respiratory dysfunction. The natural history and time-course of respiratory functional decline is very poorly understood. There is significant involvement of central respiratory drive but no established mechanism.
We report a case of severe lupus myocarditis with rapid, acute deterioration to cardiogenic shock and multiorgan failure, highlighting the importance of early identification and treatment of acute presentations in patients with systemic lupus erythematosus. A 31-year-old woman with previously diagnosed subacute cutaneous lupus erythematosus initially presented with abdominal pain and frank per-rectal bleeding. She deteriorated rapidly with type 1 respiratory failure and acute kidney injury requiring dialysis secondary to acute cardiac failure with a prolonged intensive care unit admission, over a month. A significantly elevated troponin, global hypokinesia on echocardiogram and normal coronary artery angiogram and cardiac MRI lead to the diagnosis of acute lupus myocarditis as the cause for the cardiogenic shock. She was treated with high-dose corticosteroids and eventually made a full recovery, with cardiac function returning to normal.
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