Raynaud's Phenomenon (RP) or White Finger Disease

Essentials

• Most patients have benign primary Raynaud's phenomenon.
• Identify the patients who may have a treatable underlying disease. Secondary Raynaud's phenomenon should be diagnosed as early as possible and the underlying cause effectively treated.
• An extended RP episode will cause hypoxia in target organ tissues.
• Prepare to treat the patient in cooperation with specialized care and primary health care. Severe RP should be treated in specialized care.
• Remember to provide the patient with lifestyle guidance.

Definition

• Maurice Raynaud described Raynaud's phenomenon in 1862. The phenomenon is called by several names, such as Raynaud's phenomenon, Raynaud's disease and Raynaud's syndrome.
• RP is a phenomenon where, due to paroxysmal constriction of small vessels, most often in the fingers or toes, the skin turns first pale, then bluish and finally red (”tricolour”).
• Primary RP (pRP) is an independent condition that is usually benign.
• Secondary RP (sRP) is due to some other disease (see Table T3).

Aetiology and prevalence

• Primary RP often runs in the maternal side of the family, and there is often also migraine in the family.
• The exact mechanism behind the phenomenon is unknown but there may be abnormal self-regulation of capillary function or increased secretion of transmitters constricting blood vessels.
• In pulmonary RP, in particular, endothelial function is disturbed.
• The prevalence of primary RP is 3-15%.
  • More than 20% of young women may have RP.
  • Its prevalence is higher in women than in men.
• The prevalence of secondary RP is high in patients with systemic autoimmune diseases, in particular: for example, 95% of patients with systemic sclerosis have sRP.

Symptoms

• Both pRP and sRP usually occur symmetrically in both hands and/or both feet. Nevertheless, RP may occur in just a few or one finger or toe, the nose, the navel or nipples.
• RP with a mechanical cause or nerve entrapment often occurs in organs innervated by that nerve (e.g. median nerve entrapment in patients with carpal tunnel syndrome).
• In patients with systemic autoimmune diseases, such as systemic sclerosis, RP may also occur in visceral organs, such as kidneys or lungs, or in coronary vessels.
• Stages of RP episodes
  • The skin first turns white and, at the same time, numbness and tingling occur in the area, and the finger, for instance, becomes clumsy.
  • When the vasospasm starts to subside, the skin becomes warm. At the same time, it turns blue, sometimes really deep blue, and severe pain occurs in the area.
  • When the spasm subsides completely, the skin turns bright red.
  • The various stages may last only a moment but may sometimes continue for several hours.
• Factors triggering RP episodes
  • Wind, cold exposure, change of temperature (e.g. going into an air-conditioned space or into a refrigerated department at a store)
  • In the summer, wind blowing from the sea, even if not cold, is a common triggering factor.
  • Ingesting cold food or beverages may trigger an episode, as can handling frozen goods or coins with bare hands.
  • Emotional stress

History and examination

• Careful history taking
  • Age at onset of symptoms
  • Medication
  • Smoking
  • Working conditions
  • Family background
• RP in the hands or any other visible area of the body is easy to detect by careful observation, interview and clinical examination (see Symptoms here). Visceral RP is hard to detect.
• Check the general condition of the skin and check for ulceration on fingertips.
• Note possible sausage fingers.
• Check the blood pressure and consider cardiac disorders.
• The formerly recommended test where, in the doctor's office, the patient immersed his/her hands or feet in ice water should not be used. There is a risk of severe vasospasm and development of gangrene.
• It is important to distinguish between primary and secondary RP and, in the case of the latter, to target the treatment at its cause. Secondary RP should be suspected if
  • the symptom appears at an age of over 30 years
  • the patient has abnormal autoantibodies in his/her blood
  • the patient is found to have or develops during follow-up other symptoms suggestive of an autoimmune disease, such as ulceration on fingertips or butterfly rash on the face.
  • In patients with autoimmune diseases, videocapillaroscopy shows typical changes in eponychial capillaries (see Table T1).
  • sRP may also be associated with many other diseases and conditions (Table T3).

Differential diagnosis

Laboratory tests

• If a young woman has RP with mild symptoms and none suggesting a systemic connective tissue disease or other underlying disease, and if the symptom runs in the family, no laboratory investigations are needed. Primary health care can be responsible for her treatment.
  • Depending on the local guidelines, antinuclear antibodies, basic blood count with platelet count and ESR may be determined.
  • It should be kept in mind that about 10% of patients with scleroderma do not have antinuclear antibodies.
• Examinations should be performed and treatment chosen in specialized care if
  • the patient is a child below the age of 16 years
  • the symptom appeared at an age of over 30 years
  • the symptom is severe, frequently recurring and slowly reversible
  • ulcers occur on fingertips.
• In specialized care, laboratory examinations focus on the disease suspected based on the patient's clinical symptoms and examinations.
  • ESR: often elevated in patients with autoimmune disease or chronic inflammatory disease
  • Antinuclear antibodies (ANAAb): systemic autoimmune disease, SLE, systemic sclerosis, MCTD
  • Chemical screening of urine: changes in kidney function, such as proteinuria
  • RF: autoimmune disease, rheumatoid arthritis, cryoglobulinaemia, Sjögren's syndrome
  • Complete blood count: polycythaemia, autoimmune disorder
  • Creatinine: change in kidney function
  • fP-Gluc: diabetes
  • TSH: disorder of thyroid function
  • CK: polymyositis, dermatomyositis
  • Hepatitis serology: if there is clinical suspicion of hepatitis (cf. Viral Hepatitis)
  • Serum and urinary protein: to detect paraprotein

Videocapillaroscopy

• Videocapillaroscopy of nailfold capillaries provides a picture of the capillaries and their function and may help to identify the underlying disease.
• It can also be used to monitor the response to treatment and to define the prognosis.

Symptoms of systemic rheumatic disease

• If RP is associated with a systemic disease (a rheumatic disease, for example), the treatment and the prognosis will depend on the disease.
• Table T4 lists some of the most common autoimmune diseases with RP and their typical symptoms.

Treatment

• Holistic treatment is needed.
  • In secondary RP, it is essential to treat the underlying cause.
  • In primary RP, treatment should be targeted at the triggering factors that are known.
    • The same instructions apply to the treatment of sRP, as well.
• The whole body should be kept warm and exposure to cold and sudden temperature changes avoided.
• Smoking cessation is essential.
• Work involving vibration should be stopped.
• Release of nerve entrapment will usually cure RP restricted to a certain area.
• Protection of the skin from knocks, inflammation and dryness
• Careful treatment of the nails and cuticle skin
• Effective treatment of underlying diseases, such as arteriosclerosis or diabetes, usually lessens the symptoms.
• Work-related causes sustaining the symptom should be eliminated; for example, work that involves taking care of and reaching into a freezer is unsuitable for a patient with RP.
• Air flowing with force from air-conditioning equipment towards the shoulders should be redirected.
• If any drug (e.g. non-selective beta blocker, triptan, sympathomimetic; cf. Table T3) aggravates the symptom, its use should be avoided. However, drugs do not aggravate/trigger the symptoms in all patients.

Referral

• Impending gangrene: the patient should be referred to a hospital as an emergency case.
• The following patients should be non-urgently referred to a rheumatologist.
  • Patients with symptoms, findings or abnormal laboratory test results (e.g. ANA) suggestive of an underlying disease, such as a systemic connective tissue disease
  • Patients with symptoms constantly disturbing their everyday life
  • Patients with symptoms not responding to treatment
  • Patients who need assessment of working ability
  • Children below the age of 16 years
• It is important to identify patients benefiting from treatment and requiring consultation of a rheumatologist.
• There are treatments available to slow down or to prevent the development of finger ulceration and other late changes.

Prognosis

• Primary RP will turn into secondary RP in 14-37% of cases, especially if antinuclear antibodies have been detected. About 99% of these patients will develop some systemic autoimmune disease, most commonly systemic sclerosis Systemic Sclerosis.
• Particularly patients who have antinuclear antibodies at an early stage, elevated ESR or changes detected by videocapillaroscopy, have an increased risk of systemic sclerosis.
• The prognosis of patients with secondary RP depends on the prognosis of the underlying cause or disease.