Raynaud’s phenomenon and peripheral ischemic syndromes


Authors: D. Karetová
Authors‘ workplace: II. interní klinika 1. LF UK a VFN, Praha
Published in: Čes. Revmatol., 20, 2012, No. 2, p. 54-61.
Category: Overview Reports

Overview

Raynaud’s syndrome (or Raynaud’s phenomenon, RP) is an episodic disorder caused by vasospasm of peripheral arterioles. Exposure to cold temperatures or emotional stress is the most common underlying cause. The clinical manifestations include a sudden change in the colour of the fingers (less often of the toes), edema, paresthesia, and during a severe course of the disease, atrophy may occur in the involved tissues with ulcers or gangrene. The primary Raynaud’s phenomenon (Raynaud’s disease) that is characterized by an increased tendency to vasospasms due to an increased tone of sympaticus, can often be successfully treated with a change of lifestyle and the disease can completely remit. Secondary Raynaud’s phenomenon (Raynaud’s syndrome) is caused by an underlying systemic disease (mostly systemic sclerosis), or by various types of involvement of the arterial system (vasculitis, etc.). The diagnosis is based on a typical clinical picture. Laboratory and functional tests aim to establish the actual disorder of blood circulation (using Doppler ultrasonography, capillaroscopy etc.) and especially to exclude a systemic disease. Basic laboratory tests include determination of the activity of inflammation - C-reactive protein, rheumatoid factor, erythrocyte sedimentation rate, blood counts and antinuclear antibodies. The patient should be monitored by an angiologist to exclude concurrent macroangiopathy, or by a rheumatologist to monitor the patient for the development of any manifestations of autoimmune rheumatic diseases over time. Pharmacotherapy of RP is based on calcium channel blockers, as the drugs of first choice. However, their use is limited in some patients with RP due to tendency to hypotension. Endothelin receptor antagonists (especially bosentan) and phosphodiesterase-5 inhibitors (such as sildenafil, or longer-acting tadalafil) are the new available drugs that were introduced in the prevention of vasospasm and formation of ulcers in systemic sclerosis. The most severe cases with prolonged vasospasms leading to acral ulcerations can be treated with local or intravenous prostaglandin E1 and pharmacological or surgical sympathectomy should be considered.

Key words:
Raynaud’s phenomenon, Raynaud’s disease, digital ulcers, vasodilators, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostaglandins


Sources

1. Bakst R, Merola JE, FranksJE Jr, et al. Raynaudęs phenomenon: pathogenesis and management. J Am Acad Dermatol 2008, 59:633-653.

2. Bunker C, Goldsmith P, Leslie T, et al. Calcitonin gene-related peptide, endothelin-1, the cutaneous microvasculature and Raynaudęs phenomenon. Br J Dermatology 1996; 134:399-406.

3. Brhel P. Raynaudův syndrom způsobený prací s vibrujícími nástroji. Interní medicína pro praxi 2007; 9(10):444-447.

4. Hofírek I, Sochor O, Šárník S, et al. Vazoneurózy a angiologická vyšetření. In: Roztočil K (ed). Angiologie 2006, Trendy soudobé angiologie, sv. 1, Galén 2006:57-62.

5. Zufferey P, Depairon M, Chamot Am, et al. Prognostic significance of nailfold capillary microscopy in patients with Raynaudęs phenomenon and scleroderma-pattern abnormalities. A six-year follow-up study. Clin Rheumatol 1992,11:536-41.

6. Heidrich H (ed). Raynaudęs syndrome. VASA 2008; S/73,3-25.

7. Heidrich H. Functional vascular diseases: Raynaudęs syndrome, acrocyanosis and erythromelalgia. VASA 2010; 39:33-41.

8. Dziadzio M, Denton CP, Smith R, et al. Losartan therapy for Raynaud’s phenomenon and scleroderma: clinical and biochemical findings in a fifteen-week, randomized, parallel-group, controlled trial. Arthritis Rheum 1999 Dec; 42(12):2646-55.

9. Vinjar B, Stewart M. Oral vasodilators for primary Raynaud’s phenomenon. Cochrane Database Syst Rev 2008 Apr 16;(2):CD006687.

10. Thompson AE, Pope JE. Calcium channel blockers for primary Raynaud’s phenomenon: a meta-analysis. Rheumatology 2004 Feb, 44(2):145-50.

11. Pope J, Fenlon D, Thompson A, et al. Iloprost and cisaprost for Raynaud’s phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev 2000;(2):CD000953.

12. Korn JH, Mayes M, Cerinic MM, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum 2004;50:3985–3993.

13. Matucci-Cerinic M, Denton CP, Furst DE, et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial. Ann Rheum Dis 2011 Jan;70(1):32-8.

14. Zamiri B, Koman AL, Smith BP, et al. Double-blind, placebo-controlled trial of sildenafil for the management of primary Raynaudęs phenomenon. Ann Rheum Did 2004;63(Suppl.I):484-485.

15. Carlino G. Treatment of Raynaudęs phenomena with PDE-5 inhibitor Cialis (tadalafil) in patients with scleroderma and lupus. Arthritis Rheum 2005;52(Suppl.):S169.

16. Schiopu E, Hsu VM, Impens AJ, et al. Randomized placbo-controlled crossover trial of tadalafil in Raynaud’s phenomenon secondary to systemic sclerosis. J Rheumatol 2009;36:2264–2268.

17. Caglayan E, Huntgeburth M, Karasch T, et al. Phosphodiesterase type 5 inhibition is a novel therapeutic option in Raynaud disease. Arch Intern Med 2006;166:231-233.

18. Abou-Raya A, Abou-Raya S, Helmii M. Statins: potentially useful in therapy of systemic sclerosis-related Raynaudęs phenomenon and digital ulcers. J Rheumatol 2008;35:1801-1808.

Labels
Dermatology & STDs Paediatric rheumatology Rheumatology
Login
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account