Sildenafil Improved Pulmonary Hypertension and Peripheral Blood Flow in a Patient with Scleroderma-Associated Lung Fibrosis and the Raynaud Phenomenon

Rosenkranz, Stephan; Diet, Frank; Karasch, T; Weihrauch, Julia; Waßermann, Klaus; Erdmann, Erland

doi:10.7326/0003-4819-139-10-200311180-00030

Cited by 76 publications

(35 citation statements)

References 9 publications

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“…In scleroderma patients intra-arterial infusions of L-arginine (the substrate for NO) and sodium nitroprusside (a direct donor of NO) significantly decreased the occurrence of laboratory-induced Raynaud's phenomenon. 30 Rosenkranz et al 31 published a case report of a 65-year-old woman with severe Raynaud phenomenon related to systemic scleroderma. Treatment with sildenafil improved severity and frequency of acrocyanosis, and laserDoppler flowmetry demonstrated improved perfusion of the fingertips.…”

Section: Raynaud's Diseasementioning

confidence: 99%

PDE5 inhibitors beyond erectile dysfunction

et al. 2007

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The phosphodiesterase type-5 (PDE5) inhibitors sildenafil, vardenafil and tadalafil are widely used first-line therapy for erectile dysfunction (ED). Since the advent of sildenafil in 1998, more than 40 million men worldwide have been successfully treated with these compounds. The safety and high tolerability of PDE5 inhibitors make them an attractive tool to investigate further physiological functions of PDE5, for example the modulation of intracellular cyclic GMP (cGMP) pools. As cGMP is a key component of intracellular signaling this may provide novel therapeutic opportunities beyond ED even for indications in which chronic administration is necessary. The approval of sildenafil for the treatment of pulmonary hypertension in 2005 was a notable success in this area of research. A number of other potential new indications are currently in various phases of preclinical research and development. In recent years, extensive but very heterogeneous information has been published in this field. The aim of this review is to summarize existing preclinical and clinical knowledge and critically discuss the evidence to support potential future indications for PDE5 inhibitors.

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Section: Raynaud's Diseasementioning

confidence: 99%

PDE5 inhibitors beyond erectile dysfunction

et al. 2007

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“…2,3 It was recently reported that medical treatment of pulmonary hypertension with sildenafil in a patient with lung fibrosis secondary to SSc was associated with a significant improvement of coexisting RD. 4 Because PDE5 inhibitors are known to exert vasodilatory properties, particularly in diseased tissue, 5 and, furthermore, were recently shown to exhibit antiproliferative effects, 6 a therapeu-tic potential of PDE5 inhibitors in patients with RD may indeed be expected. Therefore, we conducted an openlabel pilot study, in which we investigated the effects of the novel PDE5 inhibitor vardenafil 7 on clinical symptoms and peripheral blood flow in patients with RD of various causes.…”

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confidence: 99%

Phosphodiesterase Type 5 Inhibition Is a Novel Therapeutic Option in Raynaud Disease

Caglayan

Huntgeburth²,

Karasch³

et al. 2006

Arch Intern Med

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“…Bei einem auf die o.g. Vasodilatanzien nicht ansprechenden SSc-assoziierten RP kann der Einsatz von Sildenafil (1-3 × 50 mg/ Tag) zu einer Abnahme der Frequenz und Dauer der Raynaud-Attacken füh-ren [78,79]. Unter einer Sildenafil-Medikation wurde auch auf die Abheilung akraler Ulzerationen ein guter Effekt beobachtet [78,80].…”

Section: Medikamentöse Therapieunclassified

Therapeutisches Management akraler Manifestationen der systemischen Sklerose

et al. 2007

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Acral manifestations of systemic sclerosis include Raynaud's phenomenon, calcinosis cutis, and sclerodactyly. In the later stages of the disease, contractures of the skin and joints as well as obliterative vasculopathy leading to digital ulcers and necrotic lesions may occur. Patients with acral manifestations of systemic sclerosis are ideally treated by a team that includes a rheumatologist, dermatologist, hand surgeon, physiotherapist, and, eventually, a psychologist. Calcium channel antagonists, alpha(1)-adrenergic blockade with prazosin, and prostacyclin analogs were proven to be effective in the treatment of scleroderma-related Raynaud's phenomenon. Losartan, an angiotensin II receptor inhibitor, and fluoxetine, a selective serotonin reuptake inhibitor, have been beneficial for systemic sclerosis-associated Raynaud's phenomenon in pilot studies. Parenteral prostacyclin analogs, e. g., iloprost, can be recommended as first-line treatment of ischemic digital ulcers. When prostacyclin analogs fail, the phosphodiesterase type 5 inhibitor sildenafil can be tried to improve ulcer healing. Bosentan, an endothelin receptor antagonist, may prevent new digital ulcers. At present, there are no medical agents agreed to be generally effective in the reduction of calcinotic deposits or cutaneous fibrosis, although some drugs have been identified as potentially beneficial. Surgical treatment of acral manifestations consists of excision or curettage of symptomatic calcific deposits, digital sympathectomy, arterial reconstruction, and amputation in rare cases. Flexion contractures of the proximal interphalangeal joints, with secondary hyperextension of the metacarpophalangeal joints, can be treated by arthrodesis of the proximal interphalangeal joints and resection arthroplasty or prostheses at the metacarpophalangeal joints to improve hand function.

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Sildenafil Improved Pulmonary Hypertension and Peripheral Blood Flow in a Patient with Scleroderma-Associated Lung Fibrosis and the Raynaud Phenomenon

Cited by 76 publications

References 9 publications

PDE5 inhibitors beyond erectile dysfunction

PDE5 inhibitors beyond erectile dysfunction

Phosphodiesterase Type 5 Inhibition Is a Novel Therapeutic Option in Raynaud Disease

Therapeutisches Management akraler Manifestationen der systemischen Sklerose

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