atrial-natriuretic-factor and Raynaud-Disease

A 65-year-old woman was admitted because of dyspnea at rest and peripheral edema due to scleroderma-associated pulmonary fibrosis and hypertension, as well as Raynaud's phenomenon.. She had a marked restrictive ventilatory disorder with severe impairment of diffusion capacity. Right heart catheterization demonstrated a mean pulmonary artery pressure of 50 mmHg. She was able to walk only 220 m. All usual methods of treatment failed to give satisfactory results so that sildenafil (phospherodiesterase type-5 |PDE-5| inhibitor; Viagra ((R)) was given, even though it is not licensed for this indications ("off-label", as a therapeutic attempt. This achieved definite reduction in pulmonary arterial pressure and significantly improved the clinical symptoms. In particular, it drastically reduced the level of atrial natriuretic peptide, an important prognostic marker in right heart failure. Sildenafil also significantly raised peripheral perfusion and the signs of Raynaud's syndrome.. PDE-5 inhibitors are efficacious in scleroderma-associated pulmonary hypertension and may also provide a new option in the treatment of Raynaud's disease.

Topics: 3',5'-Cyclic-GMP Phosphodiesterases; Aged; Atrial Natriuretic Factor; Cyclic Nucleotide Phosphodiesterases, Type 5; Female; Fingers; Humans; Hypertension, Pulmonary; Laser-Doppler Flowmetry; Phosphodiesterase Inhibitors; Phosphoric Diester Hydrolases; Piperazines; Prognosis; Pulmonary Wedge Pressure; Purines; Raynaud Disease; Regional Blood Flow; Scleroderma, Systemic; Sildenafil Citrate; Sulfones; Vasodilator Agents

The effect of a standardized cold pressure test (CPT) on the venous concentration of immunoreactive atrial natriuretic peptide (irANP) was studied in 12 females with primary Raynaud's phenomenon (PRP) and 12 female age-matched controls. The test was performed at the end of three stages. During the first stage no medication was given. During the second stage a magnesium infusion was given. After fourteen days of medication with a calcium antagonist (Nifedipine) the third stage of the study was performed. The venous irANP increased significantly (P < 0.05) 10 min after the start of the CPT both in the PRP group and in the control group (136 +/- 39 to 159 +/- 54 and 153 +/- 45 to 179 +/- 40 pg ml-1, given as mean and SD). Baseline irANP did not change in the PRP group after treatment with magnesium or nifedipine. In the control group nifedipine treatment significantly (P < 0.01) lowered venous irANP compared to the no treatment or magnesium sulphate infusion stages (128 +/- 31 vs. 153 +/- 45 and 160 +/- 41 pg ml-1). After the CPT in both PRP group and control group the venous irANP did not increase either during magnesium sulphate infusion or nifedipine treatment. In conclusion the study has demonstrated that a standardized CPT results in a delayed increase in irANP in venous plasma and that magnesium sulphate infusion and nifedipine treatment prevent this increase. Furthermore, our data do not suggest a role for irANP in the symptomatology of primary Raynaud's phenomenon.

Topics: Adult; Atrial Natriuretic Factor; Blood Pressure; Cold Temperature; Female; Humans; Magnesium Sulfate; Middle Aged; Nifedipine; Peptide Fragments; Pressure; Radioimmunoassay; Raynaud Disease