Changes in pulmonary exercise haemodynamics in scleroderma: a 4-year prospective study.

Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years.

Sonographic assessment of interstitial lung disease in patients with rheumatoid arthritis, systemic sclerosis and systemic lupus erythematosus.

OBJECTIVES: The usefulness of transthoracic ultrasound in the evaluation of lung diseases has been highlighted in the past decades. The aim of our study is to determine the diagnostic value of lung ultrasound in the detection of interstitial pulmonary fibrosis in patients with a rheumatic disease. Furthermore, we studied the possible correlation between the underlying disease and the frequency of pathological ultrasound findings. METHODS: A sample of 45 consecutive patients with RA (n=25), SSc (n=14) and SLE (n=6) and 40 healthy volunteers were enrolled into the study. Every study patient underwent both, lung sonography and HRCT. The following ultrasound findings were documented in each study patient: B- lines, subpleural nodes and irregularities of the pleura. HRCT was analysed by an experienced radiologist blind to sonography findings. RESULTS: Twenty-eight percent of the RA cohort, 64% of the SSc patients and four out of 6 SLE patients showed ILD on HRCT. Pathological ultrasound patterns were significant more frequent in the ILD group than in the non-ILD group (comet tail artifacts/B-pattern: 100% vs. 12%, p<0.001; subpleural nodes: 55 % vs. 17%, p=0.006; thickenings of the pleural line: 95% vs. 12.5%, p<0.001). Subpleural nodes were present in 100% of the RA patients vs. 22% the SSc patients (p=0.003) and 50% of the SLE patients (p=0.049) with ILD. An irregular pleural line>3 mm was documented in 100% of SSC and SLE patients with ILD, vs. 86% of ILD patients suffering from RA (p=ns). CONCLUSIONS: Transthoracic ultrasound of the lung might be a sensitive non-invasive tool to observe early stage interstitial lung disease in rheumatic diseases.

Helpful, albeit hazardous! Esophageal stem-cell injection in systemic sclerosis.

BACKGROUND: Over 90% of patients with systemic sclerosis suffer from gastroesophageal reflux. Esophageal motility disturbances are associated with a reduced life quality and may force interstitial lung disease progression. We wanted to determine whether we can improve gastroesophageal reflux in these patients by esophageal stem-cell injection. METHODS: We performed a pilot study including eights patients with systemic sclerosis and symptomatic gastroesophageal reflux. Sampling of adipose tissue was performed by an experienced plastic surgeon under local anesthesia. The collected fat was injected into the submucosa of the distal esophagus, each time 1 ml in all four quadrants starting 2, 4 and 6 cm proximal to the Z line (ending up to a total volume of 12 ml). Before the intervention, 3, 6 and finally 12 months after the procedure, patients answered the Gastroesophageal Reflux Disease Health-Related Quality of Life Questionnaire (GERD HRQL) and a high-resolution manometry was performed to quantify changes in motility function. RESULTS: All patients showed an improvement in the GERD HRQL score after the stem-cell injection and a lower dosage of proton-pump inhibitors. The manometric findings showed no change throughout the time. A serious adverse event occurred, as one patient developed multiple cerebellar embolic infarcts. CONCLUSION: Because of the favorable effect in all patients, a safe route for esophageal fat injection needs to be developed.