Raynaud's Syndrome and Phenomenon

Question 1

What are the associated underlying diseases for Raynaud’s phenomenon?
- Almost all autoimmune rheumatology conditions

Answer 1

1. Systemic sclerosis
2. SLE
3. RA
4. Rheumatoid arthritis
5. Dermatomyositis, polymyositis
6. Myeloproliferative diseases
7. Hepatitis B and C
8. Thoracic outlet syndrome (cervical rib)
9. Atherosclerosis
10. Carpal tunnel syndrome
11. Hypothyroidism
12. Drugs - beta blockers, ciclosporinW

Question 2

What is the pathophysiology of Raynauds?

Answer 2

Reversible ischaemia of peripheral arterioles due to abnormal exaggerated response to cold or stress

Triphasic response:
1. White - pallor due to vasospasm
2. Blue - stasis of blood, cyanosis
3. Red - rapid reflow of blood and hyperaemia

Factors causing Raynauds:
1. Increased blood viscocity
2. Abnormal vasculature
3. Vasoconstriction

Question 3

What is the difference between Raynaud’s disease and Raynaud’s phenomenon?

How to differentiate between Primary Raynaud’s and Secondary Raynaud’s?

Answer 3

Raynaud’s disease - primary Raynaud’s - idiopathic
Raynaud’s phenomenon - secondary - associated with autoimmune disease

Question 4

What are the drugs that are associated with secondary Raynaud’s?
How about toxins?

Answer 4

Drugs
1. Bromocriptine
2. Beta blockers
3. OCP
4. Ciclosporin
5. Clonidine
6. Chemotherapy - bleomycin, cisplatin
7. INF-alpha

Toxins
1. Vinyl chloride
2. Arsenic
3. LeadW

Question 5

What investigations would you like to request for Raynaud’s phenomenon?

Answer 5

1. FBC - haematology, autoimmune disorder
2. RP - renal impairment
3. LFT - chronic liver disease, Hep B, Hep C
4. CK - polymyositis, dermatomyositis
5. Coagulation panel - APLS
6. Fasting glucose - DM
7. TFT - hypothyroidism
8. ESR - autoimmune
9. ANA, dsDNA, C3, C4 - SLE, cryoglobulinaemia
10. Cryoglobulins
11. RF, ACCP - RA
12. ENA panel - autoimmune
13. Cold agglutinins - mycoplasma
14. Serum protein electrophoresis - paraproteinaemia
15. CXR - cervical rib

Question 6

How would you manage Raynaud’s phenomenon?

Answer 6

Non-pharmacological
1. Patient education and counseling
2. Identify and remove exposure
- Avoid vasoconstricting drugs: pseudoephedrine
- Remove exposure
3. Hand warmers
4. Smoking cessation

Pharmacological
A. Vasodilators
1. CCB - amlodipine
2. ACEi / ARB
3. Nitrates
4. Prostacyclin analogues
5. Calcitonin gene related peptide

B.
1. Antiplatelet or anticoagulation
2. Statin
3. Fluoxetine
4. Bonsentan

Refractory
1. Digital sympathectomy
2. Hyperbaric oxygen