RAYNAUD’S PHENOMENON

Dr. Ajay Panwar japi(may 2010) PG 3rd year M3 Unit

Raynaud’s phenomenon (RP) refers to reversible spasm of peripheral arterioles in response to cold or stress.

RP is usually seen in distal digits but may also involve nose,ears and tongue.

Definition

Phase 1 – Pallor due to vasoconstriction of precapillary muscular arterioles

Phase 2 – Cyanosis due to venous pooling and deoxygenation of venous blood.

Phase 3 – Erythema because of reactive hyperemia. It is associated with throbbing pain.

Triphasic Response

Raynaud’s disease is the occurrence of the vasospasm alone, with no association with another illness (also known as Primary Raynaud’s).

Raynaud’s phenomenon is secondary to other conditions, most commonly an autoimmune disease (also known as secondary Raynaud’s).

The prevalence of primary Raynaud’s varies among different populations; from 4.9-20.1% in women to 3.8-13.5% in men.

The frequency of secondary Raynaud’s depends on the underlying disorder.

Raynaud’s phenomenon and Raynaud’s disease

Primary Raynaud’s Secondary Raynaud’s

Exaggerated physiological response to cold or stress

Secondary to serious underlying disease

No arterial structural abnormality,normal nail fold capillaries

Abnormal nail fold capillaries

Symmetrical attacks Asymmetrical intense painful attacks

Tissue ulceration,necrosis or gangrene is absent Ischemic skin lesions viz ulcers and pulp pitting present

Median age of onset is 14 years. Age at onset is >30 yrs

Clinical features of Connectivetissue diseases (CTD) are absent.

Clinical features of Connectivetissue diseases (CTD) are present.

ANA and other autoantibodies are absent ANA and other autoantibodies are present

Increased frequency of migraineand Prinzmetal Angina

May not be present

ESR normal,CRP negative ESR raised,CRP positive

Autoimmune diseases: Systemic sclerosis (90%) (both diffuse (dc SSc) and limited (Lc SSc) ,

CREST syndrome. Mixed connective tissue disease (MCTD) (85%) Sjogren syndrome (33%) Systemic lupus erythematosus (SLE) (10-44%) Polymyositis (PM) (29%) and dermatomyositis (DM) (25%) Rheumatoid arthritis (RA) (10-15%) Cryoglobulinemia, cryofibrinogemia (10%) Antiphospholipid syndrome (APLA) Primary biliary cirrhosis Primary pulmonary hypertension

Etiology of Raynaud’s phenomenon

Drugs:• Ergotamines• Interferon α and ß• Oestrogens• Nicotine• B blockers• Cocaine• Clonidine• Sympathomimetics• Cyclosporine A• Cytotoxic drugs: bleomycin, cisplatin,

vincristine,vinblastine,carboplatin• Bromocriptine• Sulfasalazine

Enviornmental agents and chemicals: Frost bite Vibration – Rock drillers,Rock grinders Silica Toluene Polyvinyl chloride(PVC)

Other systemic diseases: Hypothyroidism Cancer

Digits should be examined if either primary or secondary Raynaud’s is suspected for:

- Sclerodactyly, calcinosis, arthritis or digital ulcers.- Nailfold capillaries under magnification from a dissecting

microscope or ophthalmoscope (vide infra) Any signs or symptoms of other syndromes associated with

secondary Raynaud’s phenomenon-- Bone pain may suggest a paraneoplastic syndrome associated

with a hyperviscosity syndrome.- The presence of nephritis, malar erythema, and arthritis suggests systemic lupus erythematosus

Physical Examination

Persistent cyanosis or necrotic distal tissue suggests an underlying disorder, like scleroderma or permanent ischemia.

Livedo reticularis suggests an autoimmune disorder or coagulation abnormality (Antiphospholipid syndrome).

Carpal tunnel syndrome has been associated with Raynaud’s phenomenon.

• Complete blood counts, ESR• Urinalysis• General blood chemical analyses• Antinuclear antibody (immunofluorescent assay –IFA)• Disease specific autoantibodies

If all above tests are normal,following tests should be done:

Laboratory investigations

• Thyroid function tests• Serum protein electrophoresis• Anti-topoisomerase antibodies and anticentromereantibodies• Serum viscosity• Serum creatine kinase• Rheumatoid factor• Hepatitis panel• Cold agglutinins• Antiphospholipid antibodies studies• Cryoglobulins• X-ray hands – Subcutaneous calcification is present in CREST syndrome

Digital perfusion: Measured by digital plethysmography, digital blood pressure, laser Doppler and flowmetry are mainly used for research purpose.

Nail-fold vasculature by capillaroscopy- Normal capillary loops are evenly arranged hairpin like thin,

uniform and symmetric vessels. In SSc these vessels are irregular, dilated, elongated and

tortuous. These are bushy, engorged or corkscrew on appearance.

Specific Tests

Normal nail fold capillaries

Dilated capillaries in systemic sclerosis

Doppler arterial ultrasound: Digital plethysmography or angiography:

Patients with asymmetric attacks, absent pulses, single digitinvolvement, and asymmetry of blood pressure or evidenceof critical ischemia should undergo to rule out large arterydisease such as:• Atherosclerosis• Thromboangiitis obliterans• Vasculitis• Thromboembolic disease• Thoracic outlet syndrome

General measures: Avoidance of exposure to cold,vibrating tools and stress. Avoid specified drugs. Stop smoking. Calcium channel blockers-nifedipine,amlodipine Losartan SSRI-Fluoxetine Statins Antioxidants Treatment of infection

Treatment

Prostaglandin-Iloprost,Epoprostenol

Nitroglycerine patch

Phosphodiesterase inhibitors-cilostazol,Sildenafil,Tadalafil

Endothelin receptor inhibitors-Bosentan

Sympathectomy

For critical ischemia

Nifedipine Aspirin Chemical sympathectomy Iloprost or Epoprostenol Low molecular weight heparin Localized digital sympathectomy Plasmapharesis

Acute Ischemic crisis

Prognosis of primary Raynaud’s is usually very good, with no mortality and little morbidity.

Prognosis of secondary Raynaud’s is related to the underlying disease.

Prognosis for the involved digit in these patients is related to the severity of the ischemia and the effectiveness of maneuvers to restore blood flow.

Prognosis

Thanks for patience