A new zebrafish model for experimental leukemia therapy.

OBJECTIVES

Severe oesophageal disease in patients with systemic sclerosis (SSc), referred as scleroderma oesophagus, is characterised by ineffective or absent peristalsis along with hypotensive oesophagogastric junction (hEGJ). The associations between scleroderma oesophagus and different clinical and laboratory manifestations of SSc is still controversial. In this study we aimed to assess associations between scleroderma oesophagus, diagnosed by high resolution manometry (HRM), and other manifestations of disease.

METHODS

Fifty-four consecutive SSc patients (49 women, mean age 50.6±11.6) with oesophageal symptoms underwent clinical interview, medical records review and HRM. HRMs were analysed according to the Chicago Classification in order to provide oesophageal motility diagnosis; EGJ <9 mmHg was considered hypotensive. Demographic characteristics, patient-reported symptoms, SSc subtypes, pulmonary fibrosis, cutaneous ulcers, and anti-Scl-70 positivity were compared between SSc patients with or without scleroderma oesophagus. Comparison was also performed in computed tomography (CT) findings of oesophageal lumen in 26 patients with available data. Oesophageal dilatation was deemed present when the diameter was >9 mm.

RESULTS

Absent contractility was present in 37 (68.5%) patients; among these patients hEGJP was found in 32, thus 32/54 (59.2%) patients had classic scleroderma oesophagus. There were no associations with gender, age, oesophageal symptoms, skin involvement extent, anti-Scl-70, pulmonary fibrosis and cutaneous ulcers. Notably, oesophageal dilation on chest CT was more frequent in patients with scleroderma oesophagus compared to those without (77% vs. 7%, p=0.04, respectively).

CONCLUSIONS

Scleroderma oesophagus diagnosed by HRM was present in less than 2/3 of symptomatic patients with SSc and associated only with oesophageal dilation in CT. Although further studies are needed, oesophageal dilation on chest CT may be a non-invasive alternative for evaluation of SSc patients with oesophageal symptoms.