Makindo Medical Notes

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

Clues that may raise concern for secondary Raynaud's include age of onset greater than age 40, male gender, digital ulcerations, asymmetric attacks, ischaemic signs proximal to the fingers and toes, and abnormal nail fold capillaroscopy. The prognosis for primary Raynaud phenomenon is excellent, whereas the prognosis for secondary Raynaud phenomenon depends on the underlying condition

About

• Spasm of distal arteries to the fingers and toes
• French physician, Maurice Raynaud, who first described it. 1862
• Raynaud's may be present in 5-20% of women and in 4-14% of men.

Classification

• Primary : usually affects young women and teenage girls. Primary Raynaud's is generally considered a benign condition
• Secondary: usually to another often connective tissue disease. Prognosis is related to the underlying cause
  • Connective tissue disorders: Scleroderma, Systemic sclerosis, SLE, RA, dermatomyositis or polymyositis.
  • Occupational: Using vibrating tools.
  • Vascular Obstructions: Thoracic outlet obstruction, Buergers disease, atheroma.
  • Haem: Thrombocytosis, cold agglutinin disease, PRV, monoclonal gammopathies.
  • Drugs: Beta-blockers.
  • Hypothyroidism
  • Others : polyvinyl chloride exposure, and cryoglobulinemia.

Primary vs Secondary

Aetiology

• Imbalance of neural vasoconstriction response
• Excess vasoconstrictors
• Deficit of vasodilators e.g. Nitric oxide
• High plasma viscosity

Diagnostic criteria for primary Raynaud phenomenon

• Attacks triggered by exposure to cold and/or stress
• Symmetric bilateral involvement
• Absence of necrosis
• Absence of a detectable underlying cause
• Normal capillaroscopy findings
• Normal laboratory findings for inflammation
• Absence of antinuclear factors

Clinical: Triphasic colour change of the digits

• 1. Initial white or pallor (ischaemic phase)
• 2. Stasis and blue or cyanosis (deoxygenation phase)
• 3. Red or erythema (reperfusion phase). Often painful.
• Severe causes can have progressive digital gangrene and severe ischaemic changes
• Ask about rashes, joint pain, dry eyes, dry mouth suggesting secondary disease

Investigations

• FBC, U&E, LFTS, TFTS, ESR/CRP
• ANA, dsDNA, RF, ACP, Glucose
• CXR: Cervical rib may be compressing subclavian artery.
• Nailfold capillaroscopy is an inexpensive, quick, and non-invasive exam technique that can help differentiate primary from secondary Raynaud's. Lubricant is placed on the nail folds and the nailbed capillaries can be viewed using an ophthalmoscope set at 10 to 40 dioptres. The fourth and fifth digits have the greatest translucency of the skin but all fingers should be visualized. Early abnormal findings will include few capillary haemorrhages and few enlarged capillaries. A more active pattern may show moderate loss of capillaries, frequent giant capillaries, and disorganized vascular architecture. A late pattern may show drop-out with severe loss of capillaries with extensive avascular areas and ramified or bushy capillaries. These abnormalities are found in patients with underlying connective tissue diseases such as systemic sclerosis, dermatomyositis, or undifferentiated connective tissue disease.

Management

• Keep warm - gloves - central body core warmth and not just peripheries
• Stop smoking and avoid sympathomimetics (cold remedies) and beta-blockers
• Regular exercise and manage stress
• Rheumatology referral if secondary disease suspected

Pharmacological

• Calcium channel blockers - nifedipine 10 to 20 mg orally every 6 hours or amlodipine 2.5 to 10 mg/day can reduce attacks
• Sildenafil 20 mg orally up to three times daily may help
• Nitrates would probably be second line but there are many new medications being assessed
• Endothelin-1 inhibitor e.g. bosentan 62.5 to 125 mg orally twice daily
• Ketanserin, a selective antagonist of the S2-serotonergic receptor
• Angiotensin converting enzyme [ACE] inhibitor or ARB2 - reduces angiotensin II
• Infusion of CGRP has been evaluated as a possible therapy
• Prazosin has been reported to improve RP
• Fluoxetine - SSRI
• Infusions of Prostacyclin (PGI2) e.g. iloprost 1 ng/kg/minute for 6 hours intravenously daily)
• Lumbar Sympathectomy for lower limb symptoms

References

• Managing Raynaud's
• A Review of Raynaud's Disease