sildenafil-citrate and Raynaud-Disease

Episodes of excessive vasospasm are common in patients with Raynaud's phenomenon (RP). Pharmacological treatment may often result in side-effects such as hypotension, leading to discontinuation of treatment. Review of therapeutic interventions with regard to tendency towards hypotension was done in medical databases including PubMed, Scopus, and Medline to summarize the current state of the knowledge. Despite the episodes of blood pressure drops caused by hypotension, calcium channel blockers (CCB) have been widely used in RP as first-line treatment medication. The use of other CCB apart from nifedipine is controversial due to the variety of results in clinical trials. A clinical study comparing the efficacy and tolerability of losartan with nifedipine revealed a significant reduction in RP severity, frequency of episodes, and reported adverse effects. Application of oral sildenafil 100 mg/d as an add-on therapy increased microvascular blood flow in secondary RP, while being well-tolerated and with no withdrawal from the study. Topical vasodilators may be applied as an adjuvant therapy for patients with RP. Clinical studies approved 10% nifedipine cream and 10% nitroglycerine gel as an efficient RP therapy with side-effects comparable with placebo usage. Non-pharmacological interventions, such as cold avoidance, stress management, and smoking cessation are recommended in reducing episodes of RP. Calcium channel blockers, with a particular emphasis on nifedipine, in combination with non-pharmacological management seem to be the optimal way to treat the patients with a tendency to hypotension.

Topics: Antihypertensive Agents; Calcium Channel Blockers; Humans; Hypotension; Losartan; Nifedipine; Nitroglycerin; Raynaud Disease; Sildenafil Citrate; Smoking Cessation; Stress, Psychological; Vasodilator Agents

Systemic sclerosis (SSc)-related digital vasculopathy can progress from severe Raynaud's phenomenon to digital ulceration, is a major cause of pain and disability, and impacts negatively on quality of life. Current treatments are often ineffective and poorly tolerated. This review summarises some of the progress which has been made in the last 12 to 18 months in terms of our understanding of disease process, measurement and treatment.. The most important findings include that we can now better predict which patients with SSc are most likely to develop digital ulcers. In terms of treatment, a multicentre trial showed that the phosphodiesterase inhibitor sildenafil confers some benefit in SSc-related digital ulceration. Topical therapies are being explored: iontophoresis of vasodilators increases local blood flow, and in an avian model, VEGF121 fibrin applied in a gel matrix improved wound healing.. Progress is being made. Advances in our understanding of SSc-related vasculopathy continue to lead to exploration of new treatment approaches. Clinical trials and observational studies are challenging, but are being facilitated by developments in outcome measures and improved infrastructures and networking, allowing trials in much larger numbers of patients than have previously been possible.

Topics: Fingers; Humans; Pain; Raynaud Disease; Scleroderma, Systemic; Sildenafil Citrate; Skin Ulcer; Vascular Endothelial Growth Factor A; Vasodilator Agents

Recent controlled trials have assessed the efficacy of phosphodiesterase-5 (PDE-5) inhibitors in secondary Raynaud's phenomenon (RP). However, the conclusions are conflicting, and whether these drugs are effective remains unclear. The objective of this meta-analysis was to determine the efficacy of PDE-5 inhibitors on Raynaud's Condition Score (RCS) and frequency and duration of attacks.. A systematic review of articles was performed (sources included Medline, Embase, Web of Science, the Cochrane Central Register of Controlled Trials). Only double-blind, randomised controlled trials (RCTs) were included. Studies were selected independently by two authors using predefined data fields, including study quality indicators.. Six RCTs were included (one with sildenafil, one with modified-release sildenafil, three with tadalafil and one with vardenafil). PDE-5 inhibitors significantly decreased mean RCS by -0.46 (-0.74 to -0.17) (p=0.002), the daily frequency of ischaemic attacks by -0.49 (-0.71 to -0.28) (p<0.0001), and daily duration of RP attacks by -14.62 (-20.25 to -9.00) min (p<0.0001).. PDE-5 inhibitors appear to have significant but moderate efficacy in secondary RP. A further large RCT is needed.

Topics: Carbolines; Humans; Imidazoles; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Randomized Controlled Trials as Topic; Raynaud Disease; Sildenafil Citrate; Sulfones; Tadalafil; Treatment Outcome; Triazines; Vardenafil Dihydrochloride

The therapy of systemic sclerosis (SSc) remains a challenge for dermatology, rheumatology, internal medicine, and other disciplines. Organ involvement, above all kidney and lungs, is a key therapeutic issue. The current developments in organ-specific therapy are the main topic of the article. Finally, possibilities of disease-modifying drugs and value of HSCT are discussed.

Topics: Angiotensin-Converting Enzyme Inhibitors; Cardiovascular Diseases; Fibrosis; Fingers; Gastrointestinal Agents; Hematopoietic Stem Cell Transplantation; Humans; Hypertension, Pulmonary; Iloprost; Kidney Transplantation; Piperazines; Purines; PUVA Therapy; Raynaud Disease; Recurrence; Scleroderma, Systemic; Sildenafil Citrate; Skin Ulcer; Sulfones; Ultraviolet Therapy; Vasodilator Agents

Topics: Adolescent; Adult; Aged; Dosage Forms; Double-Blind Method; Female; Humans; Middle Aged; Nifedipine; Pilot Projects; Prospective Studies; Raynaud Disease; Sildenafil Citrate; Vasodilator Agents; Young Adult

Treatment of Raynaud phenomenon (RP) with phosphodiesterase-5 inhibitors has shown moderate efficacy. Adverse effects decrease the risk-benefit profile of these drugs, and patients may not be willing to receive long-term treatment. On-demand single doses before or during exposure to cold may be a good alternative.. To assess the efficacy and safety of on-demand sildenafil in RP.. Series of randomized, double-blind, n-of-1 trials. (ClinicalTrials.gov: NCT02050360).. Outpatients at a French university hospital.. Patients with primary or secondary RP.. Each trial consisted of a multiple crossover study in a single patient. Repeated blocks of 3 periods of on-demand treatment were evaluated: 1 week of placebo, 1 week of sildenafil at 40 mg per dose, and 1 week of sildenafil at 80 mg per dose, with a maximum of 2 doses daily.. Raynaud Condition Score (RCS) and frequency and daily duration of attacks. Skin blood flow in response to cooling also was assessed with laser speckle contrast imaging. Mixed-effects models were used and parameters were estimated in a Bayesian framework to determine individual and aggregated efficacy.. 38 patients completed 2 to 5 treatment blocks. On the basis of aggregated data, the probability that sildenafil at 40 mg or 80 mg was more effective than placebo was greater than 90% for all outcomes (except for RCS with sildenafil, 80 mg). However, the aggregated effect size was not clinically relevant. Yet, substantial heterogeneity in sildenafil's efficacy was observed among participants, with clinically relevant efficacy in some patients.. The response to sildenafil was substantially heterogeneous among patients.. Despite a high probability that sildenafil is superior to placebo, substantial heterogeneity was observed in patient response and aggregated results did not show that on-demand sildenafil has clinically relevant efficacy. In this context, the use of n-of-1 trials may be an original and relevant approach in RP.. GIRCI (Groupement Interrégional de Recherche Clinique et d'Innovation) Auvergne Rhône-Alpes (academic funding) and Pfizer.

Topics: Adult; Cross-Over Studies; Data Interpretation, Statistical; Double-Blind Method; Drug Administration Schedule; Female; Humans; Male; Middle Aged; Phosphodiesterase 5 Inhibitors; Prospective Studies; Raynaud Disease; Sildenafil Citrate; Treatment Outcome; Vasodilator Agents

Pulmonary artery hypertension (PAH) remains the leading cause of morbidity and mortality in systemic sclerosis, while Raynaud's phenomenon and digital ulcers significantly add to the morbidity in systemic sclerosis (SSc). This study was undertaken to evaluate the role of sildenafil in PAH, Raynaud's phenomenon, and digital ulcers in systemic sclerosis patients. A prospective, open-label, uncontrolled pilot study was done at a tertiary care centre in India to study the safety and efficacy of oral sildenafil in PAH, Raynaud's phenomenon, digital infarcts, and ulcers in SSc. Seventeen patients fulfilling ACR classification criteria for scleroderma and having PAH were recruited. Six-minute walk test, WHO class of dyspnoea, severity of Raynaud's phenomenon, and 2D ECHO were performed in all the study subjects at baseline and at 3 months post-treatment. All patients were treated with oral sildenafil 25 mg three times a day for a period of 3 months. The pre- and post-treatment values of mean pulmonary artery pressure (PAP), 6-min walk test, WHO class of dyspnoea, and severity of Raynaud's phenomenon were compared to look for any significant change. Sixteen patients who completed 3-month follow-up had shown statistically significant improvement in 6-min walk test, WHO class of dyspnoea, severity of Raynaud's phenomenon, and mPAP. Also, there was no occurrence of new digital infarcts or ulcers, and existing ulcers showed signs of healing. Sildenafil is highly efficacious cheaper and safe alternative to other available therapies for SSc-associated PAH, Raynaud's phenomenon, and digital infarcts/ulcers.

Topics: Adult; Arterial Pressure; Female; Humans; Hypertension, Pulmonary; Male; Middle Aged; Pilot Projects; Piperazines; Prospective Studies; Pulmonary Artery; Purines; Raynaud Disease; Scleroderma, Systemic; Sildenafil Citrate; Skin; Skin Ulcer; Sulfones; Treatment Outcome; Vasodilator Agents

To examine the effect of sildenafil in patients with Raynaud's phenomenon (RP) secondary to limited cutaneous systemic sclerosis (lcSSc).. In this double-blind, placebo-controlled study, 57 patients with RP secondary to lcSSc were randomized to receive modified-release sildenafil 100 mg once daily for 3 days followed by modified-release sildenafil 200 mg once daily for 25 days or placebo. The primary assessment was the percentage change in the number of RP attacks per week in the per-protocol population. Secondary end points included Raynaud's Condition Score, duration of attacks, RP pain score, endothelial dysfunction assessed by a peripheral arterial tonometric (PAT) device, and serum biomarker levels.. The mean percentage reduction from baseline to day 28 in attacks per week was greater for modified-release sildenafil than for placebo (-44.0% versus -18.1%, P = 0.034); the mean number of attacks per week improved from 25.0 at baseline to 19.3 after placebo treatment and from 30.5 to 18.7 after modified-release sildenafil treatment (P = 0.244). Decreases from baseline in Raynaud's Condition Score, duration of attacks, and RP pain score were not significantly different between groups. Mean values and changes from baseline in PAT responses and serum biomarker levels were similar between groups. The most frequent adverse events were headache and dyspepsia; the majority of adverse events were mild or moderate.. Our findings indicate that modified-release sildenafil reduced attack frequency in patients with RP secondary to lcSSc and was well tolerated. Modified-release sildenafil may be a treatment option in this patient population.

Topics: Adult; Aged; Biomarkers; Delayed-Action Preparations; Double-Blind Method; Female; Humans; Male; Middle Aged; Piperazines; Purines; Raynaud Disease; Scleroderma, Limited; Secondary Prevention; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents

Topics: Humans; Male; Penile Erection; Piperazines; Purines; Raynaud Disease; Sildenafil Citrate; Sulfones; Vasodilator Agents

Vasodilatory therapy of Raynaud's phenomenon represents a difficult clinical problem because treatment often remains inefficient and may be not tolerated because of side effects.. To investigate the effects of sildenafil on symptoms and capillary perfusion in patients with Raynaud's phenomenon, we performed a double-blinded, placebo-controlled, fixed-dose, crossover study in 16 patients with symptomatic secondary Raynaud's phenomenon resistant to vasodilatory therapy. Patients were treated with 50 mg sildenafil or placebo twice daily for 4 weeks. Symptoms were assessed by diary cards including a 10-point Raynaud's Condition Score. Capillary flow velocity was measured in digital nailfold capillaries by means of a laser Doppler anemometer. While taking sildenafil, the mean frequency of Raynaud attacks was significantly lower (35+/-14 versus 52+/-18, P=0.0064), the cumulative attack duration was significantly shorter (581+/-133 versus 1046+/-245 minutes, P=0.0038), and the mean Raynaud's Condition Score was significantly lower (2.2+/-0.4 versus 3.0+/-0.5, P=0.0386). Capillary blood flow velocity increased in each individual patient, and the mean capillary flow velocity of all patients more than quadrupled after treatment with sildenafil (0.53+/-0.09 versus 0.13+/-0.02 mm/s, P=0.0004). Two patients reported side effects leading to discontinuation of the study drug.. Sildenafil is an effective and well-tolerated treatment in patients with Raynaud's phenomenon.

Topics: Adult; Aged; Blood Flow Velocity; Connective Tissue Diseases; Cross-Over Studies; Double-Blind Method; Drug Resistance; Female; Humans; Male; Middle Aged; Patient Selection; Piperazines; Purines; Raynaud Disease; Scleroderma, Systemic; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilation; Vasodilator Agents

Topics: Female; Humans; Male; Piperazines; Purines; Raynaud Disease; Sildenafil Citrate; Sulfones; Vasodilator Agents

Topics: Aged; Female; Hand; Humans; Raynaud Disease; Scleroderma, Systemic; Sildenafil Citrate; Vasodilator Agents

The Raynaud's phenomenon (RP) is characterized by an exaggerated vascular response to cold temperature or emotional stress causing temporary ischemia. It is more prevalent in the digits of the hands and feet, and when occurring in conjunction with a rheumatological condition, it is also termed Raynaud's syndrome, or secondary RP. Healing following a burn requires appropriate tissue perfusion to promote primary restoration of the skin, prevent further burn progression, and to promote skin graft take in wounds requiring autologous split skin grafting. The addition of vascular compromise caused by RP to a burn wound is therefore hypothesized to impair burn wound healing and worsen burn wound progression. The authors describe a 51-year-old female with digital burns on a background of scleroderma and Raynaud's syndrome successfully treated with oral sildenafil therapy and autologous split skin grafting. The case report further highlights the potential role for sildenafil therapy in wound healing and patients requiring autologous skin grafting or local skin flaps. In future cases, we plan to involve rheumatology services early in the course of the injury aiming to improve outcomes.

Topics: Burns; Combined Modality Therapy; Female; Hand Injuries; Humans; Middle Aged; Phosphodiesterase 5 Inhibitors; Raynaud Disease; Sildenafil Citrate; Skin Transplantation; Surgical Flaps

Topics: Humans; Raynaud Disease; Sildenafil Citrate; Vasodilator Agents

Calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST) syndrome is a form of a rare, clinical subtype of systemic sclerosis, known as limited systemic sclerosis. Limited systemic sclerosis, including CREST syndrome, manifests as fibrotic skin changes restricted to the hands and face, with vascular, musculoskeletal, and visceral involvement. We present a case of a 75-year-old woman with a longstanding history of CREST syndrome complicated by a digital ulceration and persistent pain associated with recalcitrant Raynaud phenomenon. After failing a number of first-line pharmacologic therapies such as diltiazem, sildenafil, and topical nitropaste, the patient was started on a trial of botulinum toxin for the left second digit, with 10 unit injections into both webspaces for a total of 20 units. Following injection, the patient reported no further baseline pain in the affected finger and an over fifty-percent improvement in discomfort with manipulation of the digit at a follow-up time of one week. The ulceration started healing within the following three weeks. This result was maintained at a follow-up time of six weeks.

Topics: Acetylcholine Release Inhibitors; Administration, Topical; Aged; Botulinum Toxins; CREST Syndrome; Diltiazem; Female; Humans; Nitroglycerin; Pain; Raynaud Disease; Sildenafil Citrate; Treatment Failure; Ulcer; Vasodilator Agents

The aim of this study was to evaluate in systemic sclerosis (SSc) retrospectively the effect of Bosentan and Sildenafil and their combination on Raynaud's phenomenon (RP), function, and capillaroscopic patterns. One hundred and twenty-three SSc patients (mean age ± sd, 57.69 ± 14.07 years) were retrospectively evaluated and divided into two groups according to American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification score: group 1 score < 10, group 2 score > 10. Each group was divided into three subgroups according to treatment: Bosentan, Sildenafil, and Bosentan + Sildenafil. Nailfold videocapillaroscopy (NVC), Scleroderma Health Assessment Questionnaire (SHAQ) and Raynaud Condition Score (RCS) were performed at baseline and after 3 and 6 months. In Bosentan (29 patients: 12, group 1; 17, group 2), NVC changed significantly in both groups, after 3 and 6 months (p = 0.00439, group 1; p = 0.00035, group 2). In group 1, the "active" and the "late" patterns reduced, and the "aspecific" increased. In group 2, there was a reduction of late patterns, a worsening of SHAQ (p < 0.005) and an improvement of RCS (p = 0.00014). In Sildenafil (63 patients: 35, group 1; 28, group 2), after 3 months, NVC patterns changed significantly in both groups(p = 0.042 group 1, p = 0.00089 group 2). In group 1, the late and early patterns increased, and the aspecific decreased. In group 2, a significant change of NVC pattern was observed also after 6 months (p = 0.00089): the late pattern increased while the active one reduced. After 6 months, SHAQ was significantly reduced in group 1 (p = 0.00027) and in group 2 (p = 0.0043). RCS improved in both groups (p = 0.0042, group 1; p = 0.0016, group 2). Combination therapy (Bosentan + Sildenafil) (31 patients: 14, group 1; 17, group 2) induced significant changes on NVC only in group 1 after 3 (p = 0.00256) and 6 months (p = 0.000349) with a reduction of the late and active patterns and an increase of the early pattern. In both groups, after 6 months, SHAQ (p < 0.05, group 1; p = 0.00049, group 2) and RCS significantly reduced (group 1, p = 0.00024; group 2, p = 0.0021). Patients treated with Bosentan + Sildenafil show a significant improvement of RCS and NVC. This combination therapy may exert a vascular activity achieving an amelioration of the structure of microvasculature in SSc.

Topics: Adult; Aged; Bosentan; Capillaries; Drug Therapy, Combination; Female; Humans; Male; Microscopic Angioscopy; Microvessels; Middle Aged; Nails; Raynaud Disease; Retrospective Studies; Scleroderma, Systemic; Sildenafil Citrate; Sulfonamides; Treatment Outcome; Vasodilator Agents

Systemic sclerosis (SSc) is a connective tissue disease frequently associated with Raynaud's Phenomenon (RP). Among possible pharmacological treatments, phosphodiesterase 5 inhibitors are considered in cases of severe non -responsive RP. We present the case of a male SSc patient wh presented with critical finger ischemia and concomitant appearance of myocardial fibrosis after sudden interruption of sildenafil treatment.

Topics: Antirheumatic Agents; Cardiomyopathies; Fingers; Humans; Ischemia; Male; Middle Aged; Myocardium; Raynaud Disease; Risk Factors; Scleroderma, Systemic; Sildenafil Citrate; Substance Withdrawal Syndrome; Time Factors

Raynaud's phenomenon is a microvascular disorder that results in exaggerated vasoconstriction over vasodilatation secondary to an alteration in autonomic control. Though benign, it can result in severe ulceration and ultimately gangrene associated with disfiguration and permanent deformity. We present a case of severe secondary Raynaud's phenomenon in a black-African patient from a resource-limited setting, with focus on the difficulties encountered in the diagnosis and treatment.. A 43-year-old female Cameroonian farmer with a 7-year history of episodic paresthesia in her fingers and toes (when exposed to cold) presented to our emergency department with severe pain, ulceration, and "darkening" of her fingertips over a period of 2 days. An examination revealed bilateral ulceration and dry gangrene of her fingers and toes, based on which a diagnosis of secondary Raynaud's phenomenon due to a connective tissue disease was proposed. Results of paraclinical investigations were normal. Lifestyle modification along with a calcium channel blocker and phosphodiesterase type 5 inhibitor provided significant relief.. An early diagnosis and knowledge on appropriate treatment of Raynaud's phenomenon is of vital importance to prevent permanent tissue damage and disability. Relying on biphasic color change for the diagnosis of Raynaud's phenomenon in black Africans can be potentially misleading.

Topics: Adult; Analgesics, Opioid; Anti-Bacterial Agents; Black People; Calcium Channel Blockers; Cloxacillin; Connective Tissue Diseases; Directive Counseling; Female; Fingers; Gangrene; Humans; Life Style; Microcirculation; Nifedipine; Phosphodiesterase 5 Inhibitors; Raynaud Disease; Risk Reduction Behavior; Severity of Illness Index; Sildenafil Citrate; Toes; Tramadol; Treatment Outcome

Raynaud's phenomenon (RP) is commonly observed in fingers and toes of patients with connective tissue diseases (CTDs). However, existing vasodilators have very limited efficacy. In this study, phosphodiesterase type 5 inhibitors (PDE-5Is) were administered to evaluate efficacy on RP. Three patients with mixed connective tissue disease and three patients with systemic sclerosis having RP were enrolled. Oral sildenafil, vardenafil, or tadalafil was administered. The fingertip temperature was measured by thermography before and 120 min after administration. To evaluate longer effects, vardenafil was administered daily for 12 weeks; the fingertip temperature was measured by thermography before and 12 weeks after administration. As compared with the pre-administration of sildenafil, vardenafil, and tadalafil, the mean fingertip temperature increased by 2.17, 3.47, and 3.59 °C, respectively, in 120 min. In the 12-week trial with vardenafil in 3 patients, the mean fingertip temperature increased by 3.04, 7.96, and 3.32 °C from baseline in each patient. PDE-5Is significantly increased fingertip temperature within 120 min, and the effect of vardenafil lasted for 12 weeks under daily use. PDE-5Is were safe and would be an effective treatment for RP with CTDs.

Topics: Administration, Oral; Adult; Aged; Carbolines; Female; Fingers; Humans; Imidazoles; Male; Middle Aged; Phosphodiesterase 5 Inhibitors; Pilot Projects; Piperazines; Prospective Studies; Purines; Raynaud Disease; Regional Blood Flow; Sildenafil Citrate; Skin Temperature; Sulfonamides; Sulfones; Tadalafil; Thermography; Time Factors; Treatment Outcome; Triazines; Vardenafil Dihydrochloride; Vasodilator Agents

We report a rare case of diffuse systemic sclerosis (SSc) evolving into diffuse SSc/systemic lupus erythematosus (SLE) overlap syndrome. A 15-year-old boy was diagnosed as diffuse SSc with initial presentations of Raynaud's phenomenon and skin tightening. He underwent Chinese herbal treatment and clinical symptoms deteriorated in the following 3 years. On admission to our ward, serositis with pleural effusion, severe pulmonary fibrosis with moderate pulmonary hypertension, swallowing difficulty, and polyarthritis were observed. Autoantibody profiles revealed concurrence of anti-double-stranded DNA, anti-Smith, anti-topoisomerase I, and anti-ribonucleoprotein antibodies. The patient fulfills the criteria for both diffuse SSc and SLE. After drainage for pleural effusion accompanied by oral prednisolone and sildenafil, there were improvement of respiratory distress, swallowing difficulty, and pulmonary hypertension. In conclusion, connective tissue diseases may overlap with each other during the disease course. Serial follow-up for clinical symptoms as well as serological changes is recommended.

Topics: Adolescent; Antihypertensive Agents; Arthritis; Autoantibodies; Biomarkers; Deglutition Disorders; Disease Progression; Drainage; Drugs, Chinese Herbal; Glucocorticoids; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Male; Piperazines; Pleural Effusion; Prednisolone; Pulmonary Fibrosis; Purines; Raynaud Disease; Scleroderma, Diffuse; Serositis; Sildenafil Citrate; Sulfones; Treatment Outcome

Digital skin vasoconstriction on local cooling is exaggerated in primary Raynaud's phenomenon (RP) as compared with controls. A significant part of such vasoconstriction relies on the inhibition of the nitric oxide (NO) pathway. We tested the effect of the phosphodiesterase 5 (PDE5) inhibitor sildenafil, which potentiates the effect of NO, on skin blood flow. We recruited 15 patients with primary RP, performing local cooling without sildenafil (day 1), after a single oral dose of 50 mg (day 2), and after a dose of 100 mg (day 3). Skin blood flow, skin temperature, and arterial pressure were recorded, and data were expressed as cutaneous vascular conductance (CVC). Sildenafil at 100 mg, but not 50 mg, significantly lessened the cooling-induced decrease in CVC. It also increased resting CVC and skin temperature. These data suggest that 100 mg sildenafil improves digital skin perfusion during local cooling in primary RP. The benefit of sildenafil "as required" should be confirmed in a randomized, controlled trial.

Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Phosphodiesterase 5 Inhibitors; Piperazines; Purines; Raynaud Disease; Regional Blood Flow; Sildenafil Citrate; Skin; Skin Temperature; Sulfones

Topics: Arginine; Bosentan; Botulinum Toxins; Calcium Channel Blockers; Humans; Piperazines; Prazosin; Purines; Raynaud Disease; Sildenafil Citrate; Sulfonamides; Sulfones

Topics: Altitude Sickness; Erectile Dysfunction; Heart Diseases; Humans; Hypertension, Pulmonary; Male; Piperazines; Purines; Raynaud Disease; Sildenafil Citrate; Stroke; Sulfones; Vasodilator Agents

Topics: Humans; Piperazines; Purines; Raynaud Disease; Sildenafil Citrate; Sulfones; Vasodilator Agents

Topics: Humans; Piperazines; Purines; Raynaud Disease; Sildenafil Citrate; Sulfones; Vasodilator Agents

Topics: Adult; Dose-Response Relationship, Drug; Feasibility Studies; Female; Humans; Male; Middle Aged; Piperazines; Purines; Raynaud Disease; Scleroderma, Systemic; Sildenafil Citrate; Skin Temperature; Sulfones; Treatment Outcome; Vasodilator Agents

The Raynaud's phenomenon often accompanies systemic rheumatic diseases and is also known as a vascular side effect of chemotherapy. Therapy of the Raynaud's phenomenon with nitrates or calcium-channel-blockers is rarely beneficial. In contrast, the PDE-V-inhibitor sildenafil seems to be effective in these patients. For the first time we report on a patient with Raynaud's phenomenon due to chemotherapy not responding to sildenafil but to the new PDE-V-inhibitor tadalafil in an equivalent dosage. Measurement with a laser Doppler revealed an increased blood flow and a reduction of symptoms. Therefore, therapy of Raynaud's phenomenon with the new PDE-V-inhibitor tadalafil seems to be an effective treatment option in patients not responding to sildenafil.

Topics: 3',5'-Cyclic-GMP Phosphodiesterases; Brachial Artery; Capillaries; Carbolines; Fingers; Humans; Laser-Doppler Flowmetry; Male; Middle Aged; Phosphodiesterase Inhibitors; Piperazines; Purines; Raynaud Disease; Regional Blood Flow; Sildenafil Citrate; Sulfones; Tadalafil; Treatment Outcome; Ultrasonography

Topics: Aged; Alprostadil; Female; Humans; Phosphodiesterase Inhibitors; Piperazines; Purines; Raynaud Disease; Scleroderma, Systemic; Sildenafil Citrate; Sulfones; Vasodilator Agents

Topics: Adult; Female; Fingers; Humans; Male; Middle Aged; Piperazines; Purines; Raynaud Disease; Retrospective Studies; Scleroderma, Systemic; Sildenafil Citrate; Skin Ulcer; Sulfones; Vasodilator Agents

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

Topics: Adult; Aged; Female; Fingers; Humans; Hypertension, Pulmonary; Ischemia; Phosphodiesterase Inhibitors; Piperazines; Purines; Raynaud Disease; Sildenafil Citrate; Sulfones; Toes

Topics: 3',5'-Cyclic-GMP Phosphodiesterases; Aged; Female; Hemodynamics; Humans; Hypertension, Pulmonary; Natriuretic Peptide, Brain; Piperazines; Pulmonary Fibrosis; Purines; Raynaud Disease; Scleroderma, Systemic; Sildenafil Citrate; Sulfones