Scleroderma Raynaud’s phenomenon Iraj Salehi-Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir

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

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

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

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

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

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

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

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

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

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

Primary Raynaud phenomenon: Salehi I. Primary Raynaud phenomenon: Age of onset: 15-30 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

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

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

Salehi I. Scleroderma

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

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

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

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