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Background Pulmonary arterial hypertension (PAH) is a life-threatening and debilitating complication of several connective tissue diseases. We aimed to evaluate the effects of long-term treatment with bosentan, an oral dual endothelin ET A /ET B receptor antagonist, in a cohort of patients with PAH related to connective tissue diseases. Material and methods In the present prospective, noncontrolled study, 13 patients (nine with systemic sclerosis, two with systemic lupus erythematosus, one with mixed connective tissue disease and one with overlap syndrome including scleroderma and myositis), mostly nonresponders to prostanoids therapy, were treated for 1 year with bosentan. Cardiac haemodynamics and the diagnosis of PAH were performed by Doppler ultrasound examination. Exercise capacity was assessed by 6-min walking test at baseline and at 3, 6 and 12 months of therapy. Results During bosentan treatment, progressive improvement of exercise capacity was observed.Walk distance increased in seven patients, remained unchanged in three and slightly decreased in three patients. A progressive significant decrease of right ventricular systolic pressure was also observed, whereas pulmonary artery mean pressure remained unchanged. Adverse effects related to bosentan (elevation of hepatic aminotransferases) were noted in two patients. Conclusion Long-term treatment with bosentan was effective in improving exercise capacity and pulmonary haemodynamics in patients with PAH related to connective tissue diseases.

Objective: To define the prevalence and severity of esophageal involvement in systemic sclerosis (SSc) and its relationship with the different clinical forms and ANA specificities of the disease. Methods: A hundred consecutive patients with SSc, 48 with cutaneous limited, 26 with intermediate and 26 with diffuse form of disease, 49 with anti-centromere and 37 with anti-Scl70 ANA pattern, were submitted to scintigraphy using a semisolid orally ingested bolus to detect esophageal hypomotility. Results: An impairment of esophageal function has been observed in 68% of SSc patients. Esophageal dysmotility was significantly more frequent and severe in patients with cutaneous diffuse and intermediate forms of SSc and with anti- Scl70 ANA pattern. Conclusions: Esophageal involvement is very common in SSc. The scintigraphy confirms to be a useful and non invasive diagnostic method; moreover it permits to quantify the severity of the esophageal dysmotility by analyzing both global and segmental function.

OBJECTIVE: To define the prevalence of interstitial lung fibrosis in systemic sclerosis (SSc) and its relationship with the different clinical forms of disease and ANA specificities. METHODS: Fifty patients with SSc were submitted to pulmonary high resolution computed tomography (HRCT). Lung abnormalities were evaluated according to Warrick's score that considers both the severity and the extent of fibrotic lesions. RESULTS: Pulmonary HRCT abnormalities were observed in 84% of SSc patients. Ground glass aspects (60%), irregular pleural margins (56%) and septal/subpleural lines (68%) were the most common lesions. The distribution of these abnormalities favoured the posterior basilar segments of both lungs. HRCT findings were significantly more frequent in males and in patients with the cutaneous diffuse form of SSc and with the specific antibody anti-Scl70. CONCLUSIONS: HRCT is a very useful method for the diagnosis of interstitial lung fibrosis in SSc. Warrick's score permits to quantify the HRCT findings and to evaluate their relationship with the disease clinical forms and ANA specificities.

Background Esophagopathy is one of the most frequent visceral organ involvements in Systemic Sclerosis (SSc). Fibrous tissue substitution of the visceral wall progressively compromises peristalsis and leads to lower oesophageal sphincter incompetence, with dysphagia and reflux esophagitis. The process most frequently involves the lower two thirds of the oesophagus, but in many patients, oropharynx and the upper third of the oesophagus can be involved as well. Objectives In order to evaluate this aspect of the disease, we submitted a group of sclerodermic patients to videofluorography with contrast agent, a technique that allows to study the oropharyngeal phase of deglutition. Methods We evaluated 36 SSc patients, 33 female, 3 male, mean age 52 yrs. (range 27–75), mean disease duration 8.5 yrs., 23 with limited SSc, 13 with diffuse SSc. They underwent videofluorography with barium sulphate, which allows to study either the oropharyngeal or the oesophageal phase of deglutition. Results In 31 patients (86.1%) we detected radiographic abnormalities, in 11 (30.6%) of both phases, in 16 (44.4%) of the esophageal phase only, in 4 (11.1%) of the oropharingeal phase only. The oropharyngeal abnormalities, detected in 15(41.7%) patients, comprised: incompetence of palatoglossus histmus and palatopharyngeus histmus, stasis of the contrast agent in the valleculae and pyriform recesses, and its penetration inside laryngeal haditus. Conclusion Our study demonstrates that in many SSc patients, even the first phase of deglutition, the oropharyngeal one, is altered. Tissue fibrosis involves even oropharyngeal structures, compromising deglutition in the first phase with higher risk of ab ingestis phenomena.