Raynaud syndrome

Primary

Raynaud's disease, or primary Raynaud's, is diagnosed if the symptoms are idiopathic, that is, if they occur by themselves and not in association with other diseases. Some refer to primary Raynaud's disease as "being allergic to coldness". It often develops in young women in their teens and early adulthood. Primary Raynaud's is thought to be at least partly hereditary, although specific genes have not yet been identified.^[8]

Smoking increases frequency and intensity of attacks, and a hormonal component exists. Caffeine, estrogen, and nonselective beta-blockers are often listed as aggravating factors, but evidence that they should be avoided is not solid.^[9]

Secondary

Raynaud's phenomenon, or secondary Raynaud's, occurs secondary to a wide variety of other conditions.

Secondary Raynaud's has a number of associations:

• Connective tissue disorders:
  • scleroderma^[10]
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Sjögren's syndrome
  • Dermatomyositis
  • Polymyositis
  • Mixed connective tissue disease
  • Cold agglutinin disease
  • Ehlers-Danlos syndrome
• Eating disorders:
  • Anorexia nervosa
• Obstructive disorders:
  • Atherosclerosis
  • Buerger's disease
  • Takayasu's arteritis
  • Subclavian aneurysms
  • Thoracic outlet syndrome
• Drugs:
  • Beta-blockers
  • Cytotoxic drugs – particularly chemotherapeutics and most especially bleomycin
  • Cyclosporin
  • Bromocriptine
  • Ergotamine
  • Sulfasalazine
  • Anthrax vaccines whose primary ingredient is the Anthrax Protective Antigen
  • Stimulant medications, such as those used to treat ADHD (amphetamine and methylphenidate)^[11]
  • OTC pseudoephedrine medications (Chlor-Trimeton, Sudafed, others)^[12]
• Occupation:
  • Jobs involving vibration, particularly drilling and prolonged use of a string trimmer (weed whacker), suffer from vibration white finger
  • Exposure to vinyl chloride, mercury
  • Exposure to the cold (e.g., by working as a frozen food packer)
• Others:
  • Physical trauma to the extremities
  • Lyme disease
  • Hypothyroidism
  • Cryoglobulinemia
  • Cancer
  • Chronic fatigue syndrome
  • Reflex sympathetic dystrophy
  • Carpal tunnel syndrome
  • Magnesium deficiency
  • Multiple sclerosis
  • Erythromelalgia (clinically presenting as the opposite of Raynaud's, with hot and warm extremities, often co-exists in patients with Raynaud's^[13])
  • Chilblains (also clinically presenting as the opposite of Raynaud's, with hot and itchy extremities, however it affects smaller areas than erythromelalgia, for instance the tip of a toe rather than the whole foot)

Raynaud's can precede these other diseases by many years, making it the first presenting symptom. This may be the case in the CREST syndrome, of which Raynaud's is a part.

Patients with secondary Raynaud's can also have symptoms related to their underlying diseases. Raynaud's phenomenon is the initial symptom that presents for 70% of patients with scleroderma, a skin and joint disease.

When Raynaud's phenomenon is limited to one hand or one foot, it is referred to as unilateral Raynaud's. This is an uncommon form, and it is always secondary to local or regional vascular disease. It commonly progresses within several years to affect other limbs as the vascular disease progresses.^[14]

Medications

Medications can be helpful for moderate or severe disease.

• Vasodilators – calcium channel blockers, such as the dihydropyridines nifedipine or amlodipine, preferably slow-release preparations – are often first-line treatment.^[21] They have the common side effects of headache, flushing, and ankle edema, but these are not typically of sufficient severity to require cessation of treatment.^[22] The limited evidence available shows that calcium-channel blockers are only slightly effective in reducing how often the attacks happen.^[23] Although, other studies also reveal that CCBs may be effective at decreasing severity of attacks, pain and disability associated with Raynaud's phenomenon.^[24] People whose disease is secondary to erythromelalgia often cannot use vasodilators for therapy, as they trigger 'flares' causing the extremities to become burning red due to too much blood supply.
• People with severe disease prone to ulceration or large artery thrombotic events may be prescribed aspirin.^[21]
• Sympatholytic agents, such as the alpha-adrenergic blocker prazosin, may provide temporary relief to secondary Raynaud's phenomenon.^[21][25]
• Losartan can, and topical nitrates may, reduce the severity and frequency of attacks, and the phosphodiesterase inhibitors sildenafil and tadalafil may reduce their severity.^[21]
• Angiotensin receptor blockers or ACE inhibitors may aid blood flow to the fingers,^[21] and some evidence shows that angiotensin receptor blockers (often losartan) reduce frequency and severity of attacks,^[26] and possibly better than nifedipine.^[27][28]
• The prostaglandin iloprost is used to manage critical ischemia and pulmonary hypertension in Raynaud's phenomenon, and the endothelin receptor antagonist bosentan is used to manage severe pulmonary hypertension and prevent finger ulcers in scleroderma.^[21]
• Statins have a protective effect on blood vessels, and SSRIs such as fluoxetine may help symptoms, but the data is weak.^[21]
• PDE5 inhibitors are used off-label to treat severe ischemia and ulcers in fingers and toes for people with secondary Raynaud's phenomenon; as of 2016, their role more generally in Raynaud's was not clear.^[29]

Surgery

• In severe cases, an endoscopic thoracic sympathectomy procedure can be performed.^[30] Here, the nerves that signal the blood vessels of the fingertips to constrict are surgically cut. Microvascular surgery of the affected areas is another possible therapy, but this procedure should be considered as a last resort.
• A more recent treatment for severe Raynaud's is the use of botulinum toxin. The 2009 article^[31] studied 19 patients ranging in age from 15 to 72 years with severe Raynaud's phenomenon of which 16 patients (84%) reported pain reduction at rest; 13 patients reported immediate pain relief, three more had gradual pain reduction over 1–2 months. All 13 patients with chronic finger ulcers healed within 60 days. Only 21% of the patients required repeated injections. A 2007 article^[32] describes similar improvement in a series of 11 patients. All patients had significant relief of pain.

Alternative medicine

Evidence does not support the use of alternative medicine, including acupuncture and laser therapy.^[3]