Raynaud's Phenomenon Primary and Secondary

Related Subjects: |Relapsing Polychondritis |Reactive Arthritis |Raynaud's Phenomenon |Dermatomyositis |Polyarteritis nodosa |Osteoporosis |Rheumatoid Arthritis |Systemic Sclerosis (Scleroderma) |Rheumatology Autoantibodies |Overlap Syndrome

🧊 Clues for secondary Raynaud's: age of onset >40, male sex, digital ulcerations, asymmetric attacks, ischaemic changes proximal to digits, or abnormal nail fold capillaroscopy. ✅ Prognosis is excellent in primary Raynaud’s; prognosis in secondary depends on the underlying condition.

📘 About

• Vasospasm of distal arteries → fingers & toes.
• Named after French physician Maurice Raynaud (1862).
• Prevalence: 5–20% of women, 4–14% of men.

🔎 Classification

• Primary: Usually young women/teenage girls; benign course.
• Secondary: Due to another condition; prognosis depends on cause.
  • Connective tissue: Scleroderma, SLE, RA, dermatomyositis/polymyositis.
  • Occupational: Vibrating tools.
  • Vascular: Thoracic outlet obstruction, Buerger’s disease, atheroma.
  • Haematological: Thrombocytosis, cold agglutinin disease, PRV, monoclonal gammopathies.
  • Drug-induced: Beta-blockers.
  • Endocrine: Hypothyroidism.
  • Other: PVC exposure, cryoglobulinaemia.

⚖️ Primary vs Secondary Raynaud’s Phenomenon

Raynaud’s can be classified into primary (idiopathic) and secondary (associated with systemic disease). The table below highlights the key distinguishing features:

🧬 Aetiology

• Overactive neural vasoconstriction.
• ↑ Vasoconstrictors, ↓ vasodilators (e.g. nitric oxide).
• Raised plasma viscosity.

📑 Diagnostic Criteria – Primary Raynaud’s

• Attacks triggered by cold or stress.
• Symmetric, bilateral involvement.
• No necrosis or underlying disease.
• Normal nailfold capillaroscopy.
• Normal inflammatory markers and autoantibodies.

🎨 Clinical Features – Classic Triphasic Colour Change

• 1️⃣ White/pallor (ischaemia).
• 2️⃣ Blue/cyanosis (deoxygenation).
• 3️⃣ Red/erythema (reperfusion, often painful).
• Severe secondary disease → ulcers, gangrene.
• Ask about rashes, arthralgia, sicca symptoms → secondary causes.

🔬 Investigations

• Bloods: FBC, U&E, LFT, TFT, ESR/CRP, ANA, dsDNA, RF, glucose.
• CXR: Cervical rib? Subclavian compression?
• Nailfold capillaroscopy:
  • Early: Few haemorrhages, few giant capillaries.
  • Active: Loss of capillaries, frequent giant capillaries, disorganized loops.
  • Late: Dropout, avascular zones, bushy/ramified loops.
  Associated with systemic sclerosis, dermatomyositis, overlap CTDs.

🧣 Management – General

• Keep warm (gloves, core temperature maintenance).
• Stop smoking, avoid vasoconstrictors (decongestants, β-blockers).
• Exercise, stress reduction.
• Refer rheumatology if secondary suspected.

💊 Pharmacological

• First-line: Calcium channel blockers (nifedipine, amlodipine).
• Other options: Sildenafil, topical nitrates, bosentan, prazosin, fluoxetine.
• Advanced/IV: Iloprost infusions (prostacyclin analogue).
• Refractory: Lumbar sympathectomy for lower limb RP.

📚 References

• NICE CKS: Raynaud’s Phenomenon
• Review of Raynaud’s Disease (PMC)

Cases — Raynaud’s Phenomenon

• Case 1 — Primary Raynaud’s (benign) ❄️: A 22-year-old student presents with episodic finger colour changes (white → blue → red) triggered by cold weather. She is otherwise well, with normal nailfold capillaroscopy and negative ANA. Diagnosis: primary Raynaud’s. Managed conservatively with cold avoidance and reassurance.
• Case 2 — Secondary to systemic sclerosis 🧤: A 35-year-old woman reports severe painful digital ischaemia, fingertip ulcerations, and skin tightening. ANA positive, anti-centromere antibody detected. Nailfold capillaries abnormal. Diagnosis: secondary Raynaud’s due to limited cutaneous systemic sclerosis. Managed with nifedipine and rheumatology follow-up.
• Case 3 — Occupational cause ⚒️: A 46-year-old man working with vibrating tools develops episodic blanching of fingers with numbness and tingling. Exam: calluses, reduced sensation. No autoimmune features. Diagnosis: secondary Raynaud’s (hand–arm vibration syndrome). Managed with occupational health input and protective equipment.
• Case 4 — Connective tissue disease overlap 🧪: A 28-year-old woman presents with Raynaud’s alongside arthralgia, photosensitive rash, and oral ulcers. Serology: ANA positive, anti-dsDNA positive. Diagnosis: secondary Raynaud’s in systemic lupus erythematosus (SLE). Managed with vasodilators plus systemic lupus therapy.

Teaching Point 🩺: Raynaud’s is characterised by episodic vasospasm of digital arteries. Primary is common in young women, benign, and not associated with tissue injury. Secondary suggests underlying disease (e.g. systemic sclerosis, SLE, vasculitis, occupational vibration) and carries risk of ulcers and gangrene.