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Sleep Med. 2002 Jul;3(4):341-5.

Sleep disruption in systemic sclerosis (scleroderma) patients: clinical and polysomnographic findings.

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Department of Neurology, Johns Hopkins Sleep Disorders Center, Asthma and Allergy Building, The Johns Hopkins University, 5501 Hopkins Bayview Circle, Baltimore, MD 21224, USA.



Progressive systemic sclerosis (SSc) is a connective tissue disease characterized by endothelial lesions and fibrosis of the skin and other organs. Patients' quality of life and life expectancy are determined by the intensity of pulmonary, esophageal, cardiac, and renal involvement. Neurological involvement was considered to be rare, but increasing evidence indicates that peripheral and autonomic neuropathies commonly occur. Conditions seen with SSc, such as pulmonary fibrosis and gastro-esophageal reflux, have the potential to affect sleep. No systematic assessment of sleep-related problems associated with this disorder has been performed.


An all-night polysomnogram and a clinical interview blinded to sleep status were obtained for 27 consecutive SSc patients. Sleep data were compared to age adjusted published normative values. Correlations were determined for sleep and clinical data.


Patients with SSc had a reduced sleep efficiency (SE) (mean+/-SD: 82+/-12.3%) and rapid eye movement sleep (13.1+/-5.6%) and increased arousal index (26.1+/-13.0) and slow wave sleep (25.7+/-9.7%). The periodic leg movement index (PLMI) exceeded 5/h in 13 patients (48%) and 25/h in seven patients. Six patients had restless legs syndrome (RLS). Significant disordered breathing was absent. The RLS patients showed a greater arousal index than all other patients. PLMI was correlated with SE for RLS but not for non-RLS patients. Esophageal dyskinesia, dyspnea, and RLS were significantly associated with poor sleep.


Patients with SSc have significant disturbance of their sleep. Esophageal dyskinesia and dyspnea, which are common complications of SSc, were commonly associated with indices of sleep disruption. RLS but not sleep apnea appears to have an increased prevalence in SSc.

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