CREST syndrome
Overview
CREST stands for Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia.
Symptoms
The list of signs and symptoms mentioned in various sources for CREST syndrome includes the 5 symptoms listed below: * Calcinosis * Raynaud's phenomenon * Esophageal dysfunction * Sclerodactyly * Telangiectasia
Causes
* Primary (idiopathic) disease o Secondary disease associated with underlying systemic conditions –Scleroderma or CREST –Systemic lupus erythematosus –Rheumatoid arthritis –Mixed connective tissue disease –Sjögren's syndrome * Arteriosclerosis obliterans * Thromboangiitis obliterans –Associated with male smokers * Arterial embolism –Acute onset –Pulseless * Cryoglobulinemia –Hepatitis C * Cold agglutinins –Mycoplasma infection * Macroglobulinemia –Multiple myeloma * Polycythemia vera * Vasculitis (e.g., Wegener's granulomatosis) * Hepatitis B * Hypothyroidism * Thoracic outlet syndrome (brachial plexus) o Carpal tunnel syndrome o Drugs: ?-blockers, methysergide, bleomycin, vinblastine, clonidine, cyclosporine, ergot preparations o Trauma –Often associated with vibratory tool workers, pianists, typists, or meat cutters * Hypothenar hammer syndrome * Reflex sympathetic dystrophy * Multiple sclerosis * Syringomyelia * Poliomyelitis * Neoplasms * Vinyl chloride poisoning * Arteriovenous fistula
Diagnosis
Routine diagnostic studies include a CBC, sedimentation rate, urinalysis, chemistry panel, serum protein electrophoresis, ANA titer, chest x-ray, and EKG. If macroglobulinemia is suspected, a Sia water test and serum immunoelectrophoresis may be done. If cold agglutinins are suspected, a test for cold agglutinins may be done. A sickle cell preparation may be necessary if the patient is black. Collagen diseases may be further evaluated by skin and muscle biopsy and esophageal manometry. Raynaud's phenomena may be demonstrated by immersing the hands in water at a temperature of 10° to 15°C. Whole body exposure to cold is an even better way of demonstrating the actual Raynaud's phenomena. The finding of nail-fold capillary-loop dilation and drop out may also help diagnose Raynaud's phenomena. If scleroderma is suspected, an antisclerodermal antibody titer is done. Doppler studies and arteriography will rule out subclavian artery occlusions. A rheumatology or neurology consultation may be helpful.
Treatment
* Treat any underlying disorders * Stop offending or exacerbating medications * Quit smoking * Protect hands from cold or trauma with gloves * Avoid known triggers (e.g., cold, emotional stress, vibrating tools) * Vasodilator drugs (e.g., long-acting oral nitrates, low-dose sustained-release nifedipine) * Surgical treatment may include sympathectomy