Elsevier

Autoimmunity Reviews

Volume 8, Issue 1, October 2008, Pages 62-68
Autoimmunity Reviews

Treatment of Raynaud's phenomenon

https://doi.org/10.1016/j.autrev.2008.07.002Get rights and content

Abstract

Raynaud's phenomenon is an episodic vasospasm of the peripheral arteries, causing pallor followed by cyanosis and redness with pain and sometimes paraesthesia, and, rarely, ulceration of the fingers and toes. Primary or idiopathic Raynaud's phenomenon (Raynaud's disease) occurs without an underlying disease. Secondary Raynaud's phenomenon (Raynaud's syndrome) occurs in association with an underlying disease. Initially conservative, non-pharmacologic approach is important for these patients, although pharmacologic therapy may ultimately be necessary. Advances in vascular physiology have showed the role of the endothelium as well as endothelium-independent mechanisms in the altered vasoregulation of Raynaud's phenomenon. This has opened promising therapeutic avenues, and it is likely that therapies targeted towards specific pathophysiologic steps become available in the near future.

Introduction

Raynaud's phenomenon (RP) is an episodic vasospasm of the peripheral arteries due to an exaggerated reaction to cold weather or emotional stimuli. Fingers and toes are typically involved. The classic triad of symptoms and color variation was first described by Maurice Raynaud in 1862. They consist of pallor of the skin (secondary to vasospasm), followed by cyanosis (produced by de-oxygenated blood), and finally blushing (produced by the return of blood flow from the fingers followed by a reactive increase in temperature) [1], [2].

Raynaud's phenomenon is classified as primary or secondary. Patients presenting with primary Raynaud's phenomenon (PRP) most likely have a history of recurrent events, with symptoms that vary from mild to severe, but it lacks complications or ischemic damage. [3], [4] Approximately, 25% of PRP have also a family history of familiar recurrence in first degree relatives. The average age at PRP appearance is around 14 years old, only 27% of the cases starts after 40 years. Secondary Raynaud's phenomenon (SRP) mainly develops as a consequence of a systemic autoimmune disorder [5], [6], particularly systemic sclerosis [7] or mixed connective tissue disease [8].

RP can manifest itself as an isolated, acute or sub acute disorder as well, presenting with cold, pain and numbness of the fingers, secondary to ischemia that can lead to ulcerations and/or gangrene [1]. The main criteria to distinguish PRP and SRP are as follows: characteristic color variations caused by cold or stress, presence or absence of an underlying diseases that causes RP (i.e. a systemic autoimmune disease), digital ulcers or gangrene, elevated erythrocyte sedimentation rate, antinuclear antibodies (ANA), rheumatoid factor, C3 and C4 complement factors, chest X-ray, hand X-ray and nail-fold capilaroscopy. Patients presenting asymmetric attacks, absent pulse, and or asymmetric blood pressure, need more specialized studies, such as digital pletismography and arterial Doppler ultrasound or angiography to establish the diagnosis and rule out vascular diseases (i.e., thromboangiitis obliterans, embolic disease, Paget–Schoetter syndrome, or atherosclerosis) [9].

Section snippets

Treatment of Raynaud's phenomenon

Treatment for mild disease is almost exclusively conservative, changing life-style conditions that precipitate the illness [10]. Pharmacologic treatment is used when the manifestations are severe (Table 1). Surgical treatment is reserved for complicated conditions that do not respond to pharmacological treatment [1], [2], [3]. A great variety of drugs have been tested for the treatment of RP, questioning their utility, since none of them show efficiency in clinical trials, and most of them have

Non-pharmacologic therapy

Mild RP can be treated almost exclusively with conservative measures. The main objective is to try to make the patient able to change his or her life-style [3]. Patients have to learn how to recognize reflex vasospasm as a main thermo-regulative mechanism, and to identify circumstances that can cause acute attacks like sudden changes in temperature, digital trauma, or some drugs [2], [11].

Drugs with a high potential for vasospasm in peripheral arteries should be avoided, all in patients with

Pharmacological treatment

If non-pharmacologic treatment alone does not succeed, drug therapy may be necessary. The objective of using drugs in RP consists in reducing the frequency and severity of the acute vasospastic episodes, maintaining blood flow and preventing ulcers, ischemia and necrosis [16]. In the more severe cases, where chronic ischemia has caused an alteration in vascular structure, the therapeutic strategy will be to maintain adequate blood flow and to prevent vasoconstriction and avoid necrosis.

A wide

Sympathectomy

Surgical treatment is reserved for patients with severe and complicated RP, in which pharmacological treatment was not successful [1], [10]. The recommended surgical treatment consists of open decompression with resection of fibrotic layering along with digital sympathectomy, which is helpful in some patients who are unresponsive to medical therapy and patients with secondary RP with critical ischemia or active digital ulcers are likely to have some immediate improvement in blood flow following

Vascular reconstruction

On the other hand, in patients with systemic sclerosis, vascular occlusion most commonly occurs in the ulnar artery and the digital arteries; so revascularization of ulnar artery occlusive disease in this setting may improve RP and improve healing of digital ulcers [40].

Take home-messages

  • Avoid cold exposure. Use warm gloves, hats, and garments during the winter months or before going into cold environments.

  • Calcium channel blockers are the most effective treatment for Raynaud's phenomenon.

  • Patients who do not tolerate or fail to respond to calcium channel blocker therapy can try other vasodilator drugs alone or in combination.

  • Chemical or surgical sympathectomy has been reported to be effective in relief of symptoms for very severe, refractory cases, however, results of this

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