SESSION TITLE: Miscellaneous Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Monday, October 27, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: Diaphragm dysfunction is thought to contribute to dyspnea in the setting of Shrinking Lung Syndrome (SLS). Diaphragm function in this setting assessed by M-Mode Ultrasound has not been described previously.
CASE PRESENTATION: A 54 year old woman with SLE was referred for worsening dyspnea and pleuritic chest pain for eight months. Her exercise capacity dropped from 10 blocks to half a block. She was being treated with prednisone 10 mg and hydroxychloroquine. Exam revealed crackles at both lung bases. Laboratory findings showed normal BNP, positive lupus antibodies, normal complement levels with an elevated CRP of 15. Chest X ray revealed significantly reduced lung volumes with elevated hemidiaphragms worsening over 14 months. CT chest was negative for pulmonary embolism and interstitial abnormalities. Echocardiography and Right heart cath were normal. Patient was unable to perform Pulmonary function testing (PFT). M-Mode ultrasound revealed severely reduced diaphragmatic excursions on both sides, which was confirmed with fluoroscopy. Diagnosis of SLS was made and the patient was started on prednisone 60 mg daily. At 2 weeks and 8 weeks of steroid therapy, ultrasound evaluation of the hemidiaphragms revealed significant improvement in the diaphragmatic motion corresponding with the improvement in her symptoms.
DISCUSSION: We report the first case of diaphragm dysfunction in SLS confirmed and ongoing recovery followed up with M-Mode ultrasound. SLS is rare, with an estimated prevalence of <1% among patients with SLE. Diaphragmatic dysfunction is common in SLS. Chest imaging, fluoroscopy and PFT have traditionally been used to support the diagnosis and follow improvement with enhanced immune suppression. M-Mode ultrasound to assess diaphragm function is well described and normal ranges have been established. M-Mode ultrasound may allow exclusion of diaphragm dysfunction safely and noninvasively in the dyspneic patient with SLE.
CONCLUSIONS: M-Mode ultrasound may be used to assess diaphragm dysfunction in dyspneic patients with SLE and may be used to monitor recovery of diaphragm function in the setting of SLS especially among patients unable to perform PFTs. This case report describes, for the first time, diagnostic M-Mode ultrasound findings in diaphragmatic dysfunction in the setting of SLE/SLS.
Reference #1: Boussuges: Diaphragmatic motion studied by M-mode ultrasonography: Methods, reproducibility, and normal values. Chest 2009
Reference #2: Summerhill: Monitoring recovery from diaphragm paralysis with ultrasound. Chest 2008
DISCLOSURE: The following authors have nothing to disclose: Venkat Rajasurya, Sumeet Bhavsar, S Deepthi Gudivada, Vibhu Sharma
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