[Long-term effects of sildenafil in a patient with scleroderma-associated pulmonary hypertension and Raynaud's syndrome].
METHODS
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.


CONCLUSIONS
PDE-5 inhibitors are efficacious in scleroderma-associated pulmonary hypertension and may also provide a new option in the treatment of Raynaud's disease.
Klinik III für Innere Medizin, Universität zu Koln. stephan.rosenkranz@medizin.uni-koeln.de
S
S
Rosenkranz
E
E
Caglayan
F
F
Diet
T
T
Karasch
J
J
Weihrauch
K
K
Wassermann
E
E
Erdmann
D015106:3',5'-Cyclic-GMP Phosphodiesterases; D000368:Aged; D009320:Atrial Natriuretic Factor / Q000097:blood; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; D005260:Female; D005385:Fingers / Q000098:blood supply; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D017078:Laser-Doppler Flowmetry; D010726:Phosphodiesterase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use*; D010727:Phosphoric Diester Hydrolases / Q000187:drug effects; D010879:Piperazines / Q000494:pharmacology / Q000627:therapeutic use*; D011379:Prognosis; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D011687:Purines; D011928:Raynaud Disease / Q000150:complications / Q000188:drug therapy*; D012039:Regional Blood Flow / Q000187:drug effects; D012595:Scleroderma, Systemic / Q000150:complications* / Q000188:drug therapy; D000068677:Sildenafil Citrate; D013450:Sulfones; D014665:Vasodilator Agents / Q000494:pharmacology / Q000627:therapeutic use
D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D009320:Atrial Natriuretic Factor; D000068677:Sildenafil Citrate; D010727:Phosphoric Diester Hydrolases; D015106:3',5'-Cyclic-GMP Phosphodiesterases; D054706:Cyclic Nucleotide Phosphodiesterases, Type 5; C517381:PDE5A protein, human