ABC of Rheumatology: RAYNAUD'S PHENOMENON, SCLERODERMA, AND OVERLAP SYNDROMES
Authors: D. A Isenberg, C. Black
Journal: BMJ (1995)

Raynaud's phenomenonWell defined blanching of skin characteristic of Raynaud's phenomenon.Prevalence of Raynaud's phenomenon in autoimmune rheumatic diseases Rheumatoid arthritis <5% Systemic lupus erythematosus 20-30%Sjogrens syndrome 20-30% Myositis 25% Scleroderma >95%Several rheumatological conditions are linked to impaired peripheral circulation.These abnormalities may take various forms, including chilblains, acrocyanosis, and Raynaud's phenomenon.This last condition, described by the French clinician Maurice Raynaud in 1862, occurs in up to 5% of an otherwise healthy population, but may be a link between certain autoimmune rheumatic diseases.Raynaud's disease refers to the development of trophic changes as a result of microcirculatory damage and prolonged local ischaemia.Fortunately, gangrene is relatively rare, and, because patients are often young, recovery may be remarkable. Disturbances of peripheral circulatory systemChilblains-Painful, burning or itching erythematous lesions of hands or feet (rarely ulceration) precipitated by damp and cold (especially if inadequate clothing worn) Acrocyanosis-Persistent cold, blue, and rather sweaty appearance,.usually of hands Raynaud's phenomenon-Episodic, clearly demarcated two or three phase colour change-white (ischaemia), then often blue (stasis), then red (reactive hyperaemia)-of fingers and sometimes toes (rarely nose, tongue, or ears) in response to cold or, less often, emotion * Occupation-working outdoors, using vibrating tools, exposure to chemicals such as vinyl chloride * Examination of peripheral and central vascular system for proximal vascular occlusion * Drugs-such as , blockers, ergotamines, oral contraceptives, bleomycin * Symptoms of other connective tissue disorders: Arthralgia or arthritis Alopecia Skin rashes Cerebral symptoms Photosensitivity Dry eyes or mouth Mouth ulcers Muscle weakness Respiratory or cardiac problems * Full blood count and erythrocyte sedimentation rate * Total immunoglobulin and electrophoresis strip * Urine analysis * Renal and liver function tests * Nail fold capillaroscopy * Test for antinuclear antibody * Chestxrays * Hand xrays* Nifedipine (Adalat Retard 20 mg daily) * Diltiazem hydrochloride 60 mg thrice daily * Nicardipine hydrochloride 20 mg thrice daily * Felodipine 5-10 mg daily * Isradipine 1 mg twice daily, increasing to 2 mg twice daily after four weeks if necessary * Concentrated fish oils (Maxepa 5 capsules twice daily) * Gamolenic acid (Epogam 4-6 capsules twice daily) * Captopril 6-25 mg daily, increasing to 18.75 mg or 25 mg daily if necessary * High prevalence of anticentromere antibodies (70-80%) * Dilated capillary loops of nail folds, usually without capillary dropout Diffuse cutaneous scleroderma * Start of skin changes (puffy or hidebound) within one year of start of Raynaud's phenomenon * Skin of trunk and extremities affected * Presence of tendon friction rubs * Substantial proportion of patients have early onset of interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial disease * Dilated capillaries of nail folds and capillary dropout * Antibodies to scleroderma-70 (topoisomerase-1) in 30% of patients "Scleroderma sine scleroderma" * Raynaud's phenomenon may or may not be present * Skin not affected * Presents with pulmonary fibrosis, scleroderma renal crisis, cardiac disease, and gastrointestinal disease * Antinuclear antibodies (scleroderma-70, anticentromere, and antinucleolar antibodies) may be present