History taking in Raynaud's phenomenon (RP)

1. Confirm it is true Raynaud's

• Digits involved: fingers and toes. Ask about the nose tip, earlobes and, in women, the nipples.
• Color sequence: white (pallor, ischemia), then blue (cyanosis), then red (reactive hyperemia on rewarming). Ask about each phase. Up to 5% of the general population has RP, and not everyone has all three colors (Harrison's Principles of Internal Medicine 22E; Andrews' Diseases of the Skin).
• Pattern of attacks: they should be sudden, sharply demarcated, episodic and fully reversible, affecting one or several digits.
• Triggers: cold exposure (including air conditioning, cold water, handling frozen food), emotional stress, and vibration.
• Symptoms during an attack: numbness, coldness, tingling, throbbing or pain. Painful attacks suggest more severe or secondary disease.
• Duration: minutes to an hour is typical for primary RP. Prolonged attacks suggest secondary disease.
• Distribution: symmetric or asymmetric, and which fingers. The thumb is often spared in primary RP. Asymmetric or single-digit disease should prompt a search for a structural vascular cause.

2. Features that favor primary vs secondary RP

3. Complications (red flags)

• Digital ulcers, especially on the fingertips
• Pitting scars or ulcers on the pulp
• Gangrene or loss of digits
• Persistent cyanosis between attacks
• Non-healing wounds or infection
• Asymmetry, or a single digit repeatedly affected

4. Systematic review for an underlying cause

• Systemic sclerosis (scleroderma): skin tightening or puffy fingers, sclerodactyly, telangiectasia, calcinosis, and tight facial skin or a small mouth.
  • GI: heartburn, dysphagia, early satiety, bloating, constipation or diarrhea.
  • Lung: exertional dyspnea and dry cough, which suggest interstitial lung disease or pulmonary hypertension.
  • Renal/cardiac: new headache, hypertension, edema or palpitations.
• SLE: photosensitive rash, oral ulcers, arthritis, pleuritic chest pain, and hair loss.
• Sjogren's: dry eyes and dry mouth.
• Dermatomyositis/polymyositis: proximal weakness, heliotrope rash, and Gottron papules.
• Mixed connective tissue disease: overlap features, puffy hands and myositis.
• Rheumatoid arthritis: joint pain, swelling and morning stiffness.
• Vasculitis: fever, weight loss, purpura, mononeuritis, and sinus or renal symptoms.

• Use of vibrating tools (jackhammers, chainsaws), or hand-arm vibration syndrome.
• Work with vinyl chloride, or in cold environments.
• Repetitive hand trauma, such as a pianist, typist, or someone who works with frozen food.
• Symptoms of thoracic outlet syndrome or carpal tunnel syndrome, which point to a compressive cause.

• Beta blockers, ergot alkaloids and triptans.
• Cisplatin and bleomycin, and interferon.
• Amphetamines and ADHD medications (a recent systematic review links these to RP), plus decongestants like pseudoephedrine.
• Combined oral contraceptives and clonidine.
• Cocaine, nicotine and smoking.
• (Harrison's 22E lists beta blockers, cisplatin and bleomycin.)

• Cryoglobulinemia, cold agglutinins, polycythemia, and paraproteinemia.
• Hypothyroidism, which is also a late complication of RP in some patients.
• Hepatitis B or C, which are relevant to cryoglobulinemia.

• Atherosclerosis, Buerger disease (thromboangiitis obliterans; heavy smoker, claudication, ulcers), and embolic disease.
• Neck and shoulder pain, or arm claudication.

• New onset after age 40 with weight loss and constitutional symptoms.
• Paraneoplastic RP, which is often asymmetric.

5. Other points in the history

• Onset and course: age at first episode, change in frequency or severity over time, and which season is worst.
• Impact on daily life: can the patient work, grip or dress? How many attacks per day or week?
• Past history: thyroid disease, migraine (associated with RP), autoimmune disease, previous frostbite, hand trauma or surgery.
• Family history: RP or autoimmune/connective tissue disease. Primary RP supports a positive family history of RP.
• Social history: smoking, alcohol, caffeine, recreational drugs, occupation and hobbies.
• Women: pregnancy plans, and nipple pain with breastfeeding. Nipple vasospasm is a recognized form of RP.
• Treatments tried: warming strategies, calcium channel blockers, PDE-5 inhibitors and topical nitrates. Sildenafil and other PDE-5 inhibitors reduce attack frequency and help ulcer healing (Andrews').

6. Quick bedside mnemonic

7. Next steps after the history

• Look for sclerodactyly, digital pitting, ulcers, telangiectasias and calcinosis.
• Check peripheral pulses and do Allen's test.
• Check blood pressure in both arms.
• Use nailfold capillaroscopy (a dermatoscope or ophthalmoscope at the nailfold).
• Order ANA, with extractable nuclear antigen and anticentromere or Scl-70 antibodies if the ANA is positive. Add a CBC, ESR/CRP, TSH and urinalysis.

Relevant history in Raynaud's phenomenon (focused version)

1. Confirm the attack

• Color change: white, then blue, then red on rewarming.
• Sites: fingers and toes. Also ask about the nose, ears and nipples.
• Triggers: cold, emotional stress, vibration.
• Duration and frequency: how long each attack lasts and how many occur per day or week.
• Symptoms: numbness, pain, tingling.
• Distribution: symmetric or asymmetric, and whether one digit is affected repeatedly.

2. Primary or secondary?

• Age at onset: young (under 30) suggests primary. Onset after 40 suggests secondary.
• Severity and course: mild and infrequent suggests primary. Frequent, prolonged, painful or worsening attacks suggest secondary.
• Family history: of RP or autoimmune disease.
• Digital ulcers, pitting scars, necrosis or gangrene: these point to secondary RP.

3. Symptoms of an underlying disease

• Scleroderma: skin tightening, puffy fingers, calcinosis, telangiectasia, heartburn, dysphagia, dyspnea, dry cough.
• SLE: photosensitive rash, oral ulcers, joint pain, pleuritic pain, hair loss.
• Sjogren's: dry eyes and dry mouth.
• Myositis: proximal muscle weakness, rash.
• Rheumatoid arthritis: joint swelling and morning stiffness.
• Vasculitis, cryoglobulinemia or blood disorders: fever, weight loss, purpura, hepatitis history.
• Hypothyroidism.
• Arterial or compressive causes: claudication, neck or shoulder pain, thoracic outlet syndrome, carpal tunnel syndrome.
• Malignancy: weight loss and constitutional symptoms, especially with late or asymmetric onset.

4. Drug, occupational and social history

• Drugs: beta blockers, ergots, triptans, cisplatin, bleomycin, interferon, ADHD stimulants, decongestants, oral contraceptives, clonidine.
• Occupation: vibrating tools, vinyl chloride, repetitive hand trauma, cold exposure at work.
• Smoking and substances: tobacco, cocaine, amphetamines.
• Past history: previous frostbite or hand injury, migraine.

5. Complications and impact

• Ulcers, infection or non-healing wounds.
• Persistent color change between attacks.
• Effect on work and daily activities.
• What treatments or warming measures have been tried.