carboprostacyclin and Raynaud-Disease

Raynaud's phenomenon has been described more than 140 years ago, its prevalence in the general population is high, and yet the underlying pathological mechanisms are only partly understood. In children, female preponderance has been reported, as it is also the case in adults, and the disease usually starts after the age of 10. Diagnosis and differential diagnosis, in particular with erythromelalgia, are usually made through patient's or parent's interrogatory. It is important to distinguish primary Raynaud's phenomenon, which is usually benign, from Raynaud's phenomenon secondary to connective tissue disease. To this regard, nailfold capillaroscopy is of great help, as may be more recently introduced techniques such as thermography and digital blood pressure response to cooling. Primary Raynaud's phenomenon usually only requires simple physical measures (avoidance of exposure to coldness, gloves in winter, ...) plus sometimes oral calcium blockers. By contrast, Raynaud's phenomenon secondary to connective tissue disease requires systemic investigations, assessment and treatment of the underlying disease and, in some cases, more aggressive treatments such as intravenous carboprostacyclin (Iloprost).

Topics: Age of Onset; Calcium Channel Blockers; Child; Cold Temperature; Connective Tissue Diseases; Epoprostenol; Female; Humans; Male; Platelet Aggregation Inhibitors; Raynaud Disease; Sex Factors

The authors propose a promising new therapy for the treatment of Raynaud's phenomenon, with parenteral prostacyclin (carbaprostacyclin) and a serotonergic S2-receptor antagonist (ketanserin) given orally. They studied 31 patients, 22 treated with ketanserin and carbaprostacyclin, 9 with carbaprostacyclin alone. Both groups demonstrated successful results and a significant improvement in measurements performed by photoplethysmography and transcutaneous pulse oximetry.

Topics: Adult; Aged; Blood Gas Monitoring, Transcutaneous; Drug Therapy, Combination; Epoprostenol; Female; Humans; Ketanserin; Male; Middle Aged; Photoplethysmography; Prostaglandins, Synthetic; Raynaud Disease; Treatment Outcome

In a single-blind trial 25 patients with progressive scleroderma and Raynaud's phenomenon intravenous infusions of iloprost, a prostacyclin derivative (carbaprostacyclin), were given daily for five hours during a six-day hospital stay, after a comparable initial single placebo infusion. Duration, frequency and intensity of Raynaud symptoms improved in more than 75% of the patients. This improvement was objectified by telethermometry which demonstrated acral hyperthermia and significantly briefer rewarming after standardized cooling of the hands. In addition, there was more rapid healing of ulcerations and necroses of the digital pulp. A significant inhibition of ADP- and collagen-dependent platelet aggregation was demonstrated during the iloprost infusion. Side effects, such as headache, nausea and tiredness occurred only transitorily during the infusion, were individually highly variable, and then only at higher concentrations. A dosage of 2 ng/kg X min was tolerated by all patients.

Topics: Adult; Aged; Epoprostenol; Female; Humans; Male; Middle Aged; Raynaud Disease; Scleroderma, Systemic

We investigated post-ischemic hyperreactive cutaneous blood flow in patients with primary Raynaud's phenomenon and Raynaud's phenomenon secondary to systemic sclerosis (SSc). Reactive hyperemia was measured over a locally warmed area of skin, using a laser Doppler flowmeter, following 5 minutes of suprasystolic occlusion of blood flow. We found that patients with primary Raynaud's phenomenon had normal post-ischemic blood flow compared with normal controls. In contrast, patients with SSc had reduced levels of baseline and peak blood flow compared with either the primary Raynaud's phenomenon patients or the normal subjects. Infusion of carbaprostacyclin, a potent prostacyclin analog vasodilator, did not increase blood flow in the SSc patients, nor did it restore the reactive hyperemic response. These findings are consistent with the hypothesis that patients with the nonvasoconstricted condition of SSc have fixed structural defects that limit cutaneous microvascular blood flow.

Topics: Adult; Aged; Analysis of Variance; Constriction; Epoprostenol; Female; Fingers; Humans; Male; Microcirculation; Middle Aged; Raynaud Disease; Reference Values; Regional Blood Flow; Scleroderma, Systemic; Skin; Vasodilator Agents