The impact of gastroesophageal reflux disease symptoms in scleroderma: effects on sleep quality.

Systemic scleroderma/sclerosis (SSc) is an autoimmune connective tissue disease, which can lead to esophageal motor dysfunction and gastroesophageal reflux disease (GERD). Nocturnal GERD symptoms may be associated with sleep disturbances, which in turn can drastically affect well-being and fatigue levels. We hypothesized that GERD symptoms would be associated with poorer sleep in patients with SSc. Rheumatologist established SSc patients completed the following questionnaires: the UCLA scleroderma clinical trial consortium gastrointestinal tract instrument (GIT) 2.0 questionnaire; the Pittsburgh sleep quality index (PSQI); the fatigue severity scale (FSS); the multidimensional gastrointestinal symptom severity index (GSSI). Poor sleep quality was defined by a PSQI total score >5. Questionnaires were completed by 287 patients [mean (SD) age = 59 (14) years; female = 243]. Poor sleep quality was identified in 194 (68%) patients. Patients with poor sleep quality reported less sleep time and increased fatigue compared to those with normal sleep scores. SSc patients with poor sleep had significantly higher GIT Reflux scores (P < .001), and poor sleep was more frequent in those with moderate/severe versus mild/no heartburn on GISSI (P < .001). Narcotic and antidepressant use was significantly more frequent in SSc patients with poor sleep quality. Multivariable logistic regression supported the association between GERD symptoms and poor sleep after controlling for age, sex, and body mass index (BMI) (2.53, 95% confidence interval (CI) 1.52-4.25; P < .001). The association remained after controlling for narcotic and antidepressant use (2.20, 95% CI 1.29-3.73; P < .001). SSc patients who reported GERD symptoms were also more likely to report poor sleep quality. Future studies should examine mechanisms underlying nocturnal GERD symptoms in SSc patients, and the impact of improved GERD symptom control on sleep quality.

Pulmonary artery sarcoma. Clinical features.

STUDY OBJECTIVES: To define characteristic clinical and radiographic presentations of patients found to have primary sarcoma of the pulmonary artery and differentiate this entity from acute and chronic pulmonary thromboembolism. To review pathologic characteristics of pulmonary artery sarcoma. DESIGN: A retrospective review of medical records at a large medical center and a review of the literature. SETTING: Tertiary care medical center. PATIENTS: Patients found to have pulmonary artery sarcoma based on surgical or autopsy findings. INTERVENTIONS: Review of medical records. MEASUREMENTS AND RESULTS: Presenting symptoms of patients with pulmonary artery sarcoma are similar to those of patients with other pulmonary vascular disease, including progressive dyspnea, cough, hemoptysis, and chest pain. Symptoms not commonly associated with pulmonary vascular disease consist of progressive weight loss, anemia, and fever, which are symptoms commonly associated with malignancy. The diagnosis is often not suspected prior to surgery, which is often undertaken with an alternative diagnosis such as bronchogenic carcinoma or chronic pulmonary thromboembolism. Prolonged survival is rare; most patients survive only a few months. Survival longer than 3.5 years was not observed. Recent experience with MRI of the thorax and transesophageal echocardiography suggests that these diagnostic entities may be rewarding in separating the abnormalities seen on chest radiographs and angiography from thrombus in the pulmonary artery, mediastinal adenopathy, or primary bronchogenic carcinoma. CONCLUSIONS: Although a rare entity, the key is to suspect the presence of pulmonary artery sarcoma in evaluation of patients with atypical presentations of pulmonary vascular disease especially if associated with symptoms such as weight loss, fever, or anemia.

Endobronchial thrombolysis with streptokinase for airway obstruction due to blood clots.

To our knowledge, only four reports have previously described endobronchial thrombolysis with streptokinase for airway obstruction due to blood clots; the highest dose used was 80,000 U. Herein we describe a 21-year-old woman with pulmonary embolism who experienced life-threatening airway obstruction due to a large blood clot in the distal trachea. Streptokinase (120,000 U), injected through a fiberoptic bronchoscope, partially dissolved the clot. The rest of the clot was removed easily with forceps and suctioning. No complications occurred.

Thoracoscopic excision of a peripheral corticotropin-secreting pulmonary carcinoid tumor.

Bronchial carcinoid tumors are rare neuroendocrine neoplasms that arise from the Kulchitsky cells of the bronchial epithelium. These tumors can manifest as central carcinoid tumors, pulmonary carcinoid tumorlets, or peripheral carcinoid tumors. Occasionally, the peripheral carcinoid tumors produce corticotropin and result in Cushing's syndrome. Herein we report the first case of Cushing's syndrome associated with a peripheral pulmonary carcinoid tumor that was excised by video-assisted thoracoscopy. After excision, the patient had complete remission. Video-assisted thoracoscopy may be ideal for resecting a peripheral pulmonary carcinoid, especially in patients with debilitation but no evidence of metastasis.

Cardiovascular effects of sleep disorders.

Normal sleep provides a period of physiologically reduced workload for the cardiovascular system for almost one third of the human life span. Snoring, the most common disorder of sleep, heralds the presence of an unstable upper airway and alerts perceptive clinicians to the possibility of OSA. Epidemiologic evidence has implicated snoring as an independent risk factor for the development of hypertension, ischemic heart disease, and cerebral infarction. However, many investigators would attribute these adverse cardiovascular effects to the substantial prevalence of OSA in habitual snorers. The detrimental effects of OSA on hemodynamics and cardiac rhythm have been well documented, and recent data have linked OSA with increased cardiovascular mortality. Worsening hypoxemia during sleep likely contributes to the nocturnal mortality observed in patients with severe COPD. Effective treatment to prevent nocturnal hypoxemia is available for OSA and COPD, with current evidence supporting beneficial effects on survival.

Differential mechanical ventilation in respiratory failure due to severe unilateral lung disease.

Severe unilateral lung disease that produces respiratory failure may necessitate mechanical ventilatory support to sustain gas exchange. This article describes the successful use of differential lung ventilation in the management of one patient with diffuse unilateral pneumonia and another with a postoperative bronchopleural fistula after standard methods of mechanical ventilation failed to provide adequate gas exchange for these patients.

Mediastinal masses. Clues to interpretation of radiologic studies.

The best approach to evaluation of a mediastinal mass begins with a history and a physical examination that are oriented toward discovery of evidence of compression or obstruction to any of the mediastinal organs. Currently, the most efficient radiographic approach, after the plain chest roentgenogram, is computed tomography of the thorax, supplemented as needed by selected plain tomograms or a barium swallow examination. Unless the mass is definitely a benign lesion of no consequence, such as a pericardial fat pad, thoracotomy by an experienced thoracic surgeon is usually necessary to make a specific diagnosis and excision is performed if possible.

Ovarian leiomyoma with hilus cell hyperplasia that caused virilization.

A 68-year-old woman had noted gradual virilization and depression for 3 years. Examination revealed a 10-cm right pelvic mass. Plasma testosterone was substantially elevated (1,082 ng/dl), but urinary ketosteroid and ketogenic steroid excretion was normal. Laparotomy revealed a 10-cm mass that replaced the right ovary and weighed 210 g. Histologic analysis revealed a leiomyoma and proliferation of hilus cells in the periphery of the mass. The plasma testosterone decreased postoperatively to 45 ng/dl. We believe that this is the first report of an ovarian leiomyoma associated with hilus cell hyperplasia that caused virilization.

Etiologic considerations in superior vena cava syndrome.

The Mayo Clinic experience with superior vena cava obstruction during the last 20 years was reviewed. The diagnosis of superior vena cava obstruction is often made at the bedside. Typical symptoms include suffusion, dyspnea, cough, and, less commonly, pain, syncope, dysphagia, and hemoptysis. The most important physical findings are the increased collateral veins covering the anterior chest wall and the dilated neck veins with edema of the face, arms, and chest. The chest x-ray film usually shows widening of the superior mediastinum. Of our 86 cases of superior vena cava obstruction, 67 (78%) were due to malignancy and 19 (22%) to benign causes. The cause of obstruction is usually established by bronchoscopy, open lung biopsy, or biopsy of the superficial lymph node. Radiotherapy remains the standard approach for the treatment of superior vena cava obstruction due to malignant disease. It is of particular interest to note that of the six benign cases resulting from thrombosis of the superior vena cava, three were due to the use of central venous catheters. Physicians should be aware of this association.