1. Scleroderma Raynaud’s phenomenon Iraj Salehi-Abari MD., Internist
Rheumatologist

2. Raynaud phenomenon (RP):
Salehi I.
Raynaud phenomenon (RP):
Definition: Sequential color changes in the digits due to arterial vasoconstriction, precipitated by cold, stress, a decrease in temperature and vibration.
Epidemiology:
3-5% of general population
More frequent in women
Scleroderma

3. Raynaud phenomenon (RP):
Salehi I.
Raynaud phenomenon (RP):
Triphasic color changes:
Pallority = ‘’White’’ 2 p.
Cyanosis = ‘’Blue’’ 1 p.
Redness = ‘’Red’’ p.
Scleroderma

4. Active Raynaud’s phenomenon
Salehi I.
Active Raynaud’s phenomenon
Scleroderma

5. Raynaud phenomenon (RP):
Salehi I.
Raynaud phenomenon (RP):
Diagnosis is only made by History
Definite RP: repeated episodes of biphasic color changes upon exposure to cold
Possible RP: Uniphasic color changes + numbness or paresthesia
No RP: No color changes upon exposure to cold
Scleroderma

6. Raynaud phenomenon (RP):
Salehi I.
Raynaud phenomenon (RP):
Primary (idiopathic, isolated):
Without a definable underling cause
An exaggeration of normal vasoconstriction to cold exposure
Secondary (pathologic):
With an underling disease or cause
Raynaud syndrome
Scleroderma

7. Secondary Raynaud ph: Systemic Sclerosis SLE, other CTD
Salehi I.
Secondary Raynaud ph:
Systemic Sclerosis
SLE, other CTD
Occlusive vascular disease
Drugs/toxins
Hematologic abnormalities
Use of vibrating tools & vascular trauma
Frostbite
Scleroderma

8. Secondary Raynaud ph: Other Connective tissue diseases: MCTD
Salehi I.
Secondary Raynaud ph:
Other Connective tissue diseases:
MCTD
Overlap syndrome
PM/DM RA
Sjogres’s syndrome
UCTD
Vasculitis
Scleroderma

9. Secondary Raynaud ph: Occlusive vascular disease: Hypothyroidism
Salehi I.
Secondary Raynaud ph:
Occlusive vascular disease:
Arteriosclerosis
Atheroemboli
Thromboangiitis obliterans
Hypothyroidism
Scleroderma

10. Drugs/toxins cause RP Amphetamines Beta blockers, Clonidine
Interferon-alpha
Bleomycin, Cisplatin, Vinblastine
Cocaine, Nicotine
Cyclosporine
Ergot, Methysergide
Vinyl chloride
Salehi I.
Scleroderma

11. Secondary Raynaud ph: Hematologic disease: Cold agglutinin disease
Salehi I.
Secondary Raynaud ph:
Hematologic disease:
Cold agglutinin disease
Cryofibrinogenemia
Cryoglobulinemia
Paraproteinemia
Polycythemia
Scleroderma

12. Primary Raynaud phenomenon:
Salehi I.
Primary Raynaud phenomenon:
Age of onset: years
More common in female
Occur in multiple family members
Spontaneous remission may occur
May be aggravated by: HTN, CVD, atheroslerosis, and diabetes mellitus
Scleroderma

13. Diagnostic criteria of Primary RP:
Salehi I.
Diagnostic criteria of Primary RP:
Symmetric episodic attacks
No evidence of peripheral vascular disease
No tissue gangrene, tissue injury, or digital pitting
Negative nailfold capillary examination
Negative ANA and normal ESR
Scleroderma

14. Clinical clues to suggest secondary RP:
Salehi I.
Clinical clues to suggest secondary RP:
Later age of onset (>30-40 years)
Male gender
Painful RP
RP with digital ulceration and gangrene
Asymmetric attacks
RP associated with other signs or symptoms
Abnormal lab. data: CBC, ESR, auto-antibodies,…
Scleroderma

15. Salehi I.
Scleroderma

16. Nailfold capillary microscopy:
Salehi I.
Nailfold capillary microscopy:
Enlarged capillary loops or
Distorted capillary loops and
Relative paucity of loops
Suggest an underlying CTD
Enlargement associated with loss of capillaries
More suggestion of SSc
Scleroderma

17. ‘’Slow’’ phase nailfold capillaries in lcSSc: Capillary dilatation
Salehi I.
‘’Slow’’ phase nailfold capillaries in lcSSc: Capillary dilatation
Scleroderma

18. Salehi I.
‘’Active’’ phase nailfold capillaries in dcSSc: Capillary dilatation and avascular areas
Scleroderma

19. Approach to Raynaud phenomenon:
Salehi I.
Approach to Raynaud phenomenon:
Step I: History and physical examination by GP
Step II: Nailfold capillary microscopy by GP
If they are normal, then Primary RP is made by a GP, and no need for laboratory tests. But if there are any abnormality or nailfold capillary microscopy cannot perform
Step III: Hx., Ph. Exam. and Nailfold capillary microscopy by Rheumatologist
Step IV: Laboratory tests
Scleroderma