Prognostic value of N-terminal natriuretic peptides in systemic sclerosis: a single centre study.

OBJECTIVES: Cardiac involvement is an important determinant of prognosis in systemic sclerosis (SSc). The identification of patients with high risk is of great importance. Our aim was to investigate the diagnostic and prognostic value of circulating concentrations of N-terminal fragments of A- and B-type natriuretic peptides (NT-proANP and NT-proBNP) in patients with SSc. METHODS: We prospectively studied 144 patients with SSc and followed them up for five years. Blood was collected for natriuretic peptide measurement at the time of the yearly scheduled cardiological check-up. The occurrence of clinically significant cardiac disease was measured as the composite of pulmonary arterial hypertension, cardiac revascularisation, development of left ventricular dysfunction or death. RESULTS: Patients diagnosed with heart involvement during the study had significantly higher levels of NT-proANP and NT-proBNP (791.4 ± 379.9 pmol/l vs. 608.0 ± 375.8 pmol/l, p<0.05 and 183.1 ± 162.6 vs. 125.7 ± 117.5 pmol/l, p<0.05, respectively). Receiver-operator-characteristic analysis identified <822.5 pmol/l as the best NT-proANP and <154.5 pmol/l as the best NT-proBNP threshold (sensitivity 56.3%, specificity 79.5%, negative predictive value: 86.4% and sensitivity 50.0%, specificity 76.8%, negative predictive value: 83.7%, respectively). During the follow-up, lower NT-proANP levels were significantly associated with a longer event-free survival (p<0.05), similar but a non-significant trend regarding NT-proBNP levels was also shown (p=0.052). CONCLUSIONS: In our cohort, NT-proANP had a supplementary prognostic value for cardiac involvement in systemic sclerosis. In addition, the high negative predictive value of natriuretic peptides supports the more extensive use in identifying SSc patients with high risk of future cardiac involvement.

Overlap of coronary disease and pulmonary arterial hypertension in systemic sclerosis.

OBJECTIVES: Pulmonary arterial hypertension (PAH) is a common complication of systemic sclerosis (SSc). Symptoms of coronary artery disease (CAD) and PAH are closely related and cardiac catheterisation is needed to confirm their diagnosis. The aim of the present work was to investigate of the extent of overlap between CAD and PAH in patients with SSc. METHODS: Based on non-invasive investigations, 20 patients out of 120 were suspected to have PAH ("suspected PAH" group). Another 10 patients showed signs of coronary disease ("suspected CAD" Group). In these 30 patients, right heart catheterisation and coronary angiography were performed, and the coronary flow reserve (CFR) was assessed by thermodilution technique. RESULTS: In the "suspected PAH" and the "suspected CAD" groups, PAH was found in 12/20 and 2/10 cases, and coronary artery stenosis in 9/20 and 6/10 cases, respectively. Severely reduced CFR was revealed in 7/20 and 3/10 cases, respectively. CONCLUSIONS: PAH, CAD and reduced CFR all show a considerable overlap in symptomatic patients with SSc. The current non-invasive investigations are neither sensitive nor specific enough to make an appropriate distinction between these different disease manifestations. A more invasive approach, such as coronary angiography at the initial catheterisation, is required to properly characterise and treat the different forms of cardiac involvement in SSc.

[Iron deficiency anemia caused by an arteriovenous malformation in the distal ileum]. / A distalis ileum arteriovenosus malformatiója által okozott vashiányos anaemia.

The case of a young woman with chronic iron deficiency anemia is described. Her consequent guaiac-positive stool suggested a gastrointestinal bleeding behind her anemia. The use of the conventional diagnostic techniques did not result in a definitive diagnosis. The source of the hemorrhage was later detected by the aid of selective mesenteric arteriography as an unusual form of arteriovenous malformations localized to the small intestine. 20 months passed since the resection of the affected intestinal segment. During this period of close follow up no clinical signs of recurrent gastrointestinal hemorrhage was observed. The authors briefly review the relevant literature.