• Common (vascular) systemic complex occurring in 3% of females; 1% of males
• Associated with a variety of connective tissue (CT) diseases, mostly Scleroderma (see below)
• Three phases: symptoms occur in response to environmental stimuli (cold, stress, vibration, caffeine, nicotine)
• (1) Blanching (white): reversible vasospasm mostly in digits Þ pain and numbness due to ß blood supply
• (2) Cyanotic (blue): venous side of circulation relaxes first, resulting in pooling of desaturated blood in digit.
• (3) Rubor (red): reactive vasodilatation from amine released by ischemic tissue.
• If first phase is prolonged Þ ulcerations on fingertips (may even lose part of digits). This is unusual, but occurs.
• Classification of Raynauds: (Raynauds Phenomenon = The Symptom Complex)
• Primary Raynauds Phenomenon (aka Raynauds Disease) = Isolated Raynauds with no clinical consequences
• usually in young women in response to cold.
• Secondary Raynauds Phenomenon: associated with a CT disease: Scleroderma (52%), SLE (9%), Sjogren Syndrome (8%), overlap syndrome (7%), Rheumatoid Arthritis (2%), dermatomyositis, others.
• Evaluation of Raynauds: Needs to be thorough because possibility of secondary RP exists: Complete history, Physical Exam and Studies: CBS with ESB (complete blood count with sedimentation rate), chemistries, urinalysis, Chest X-ray (looking for pericarditis cardiac silhouette, or subclinical lung component), Rheumatoid Factor, Antinuclear Factor (HEP- 2-substrate); Nailfold Capillaroscopy (low power lens used to look at blood vessel proximal to cuticle which may show evidence of a variety of CT diseases that could rule out primary Reynaud's)
• Treatment:is symptomatic: avoid precipitating circumstances(cold, vibration, caffeine, nicotine, pseudoephrine)
• Therapy with Agents that reduce sympathetic tone
• Therapy with agent that have direct effect on vascular tone (Nifedipine, Ca⁺⁺ channel blockers)
• Follow for signs of development of CT disease

Scleroderma – means hard skin

• Classification:
• Localized: isolated areas of skin that are involved with a sclerotic process.
• Morphea (patches) or linear (linear streaks of involvement)
• Systemic: Progressive Systemic Sclerosis
• prognosis depends on subclassification
• Diffuse: widespread skin involvement at onset with rapid progression and visceral involvement
• Limited: localized skin involvement often confined to fingers forearms and face
• Limited Systemic Scleroderma – formerly CREST Syndrome: Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and telangiectasia
• not a benign form of scleroderma; - uncommon disease 1/100,000; F:M = 3:1
• this is a microvasculopathy that can regress then repeat which ultimately leads to fibrosis of skin and viscera
• Organ System Involvement (prognosis depends on the extent of this)
• Vascular System: over 90% of patients manifest RP. Vasculature of any organ can be involved, skin presents
• Early in course, is reversible. Changes in endothelial and intimal cells are less reversible
• Later, irreversible obliterative fibrosis can develop.
• Visceral vasculature involvement is particularly ominous
• Skin: > 95% of patients have clinically apparent cutaneous changes.
• Early Edematous phase: puffy digits with taut skin; not inflammatory or painful
• Late changes: Thickening of dermis and subcutis with adherence to underlying tissue; "hidebound skin": may involve all of the skin except the back and buttocks
• Sclerodactyly: cutaneous changes limited to the digits (none proximal to wrist as in diffuse scleroderma)
• Telangiectasia: vascular malformations. Papillary dilatation not unique to scleroderma.
• Musculoskeletal System: mild proximal weakness (non-inflam myopathy) possible; Inflammatory myopathy is rare.
• Arthralgias are common; inflammatory synovitis is rare; mechanical compromise due to CT contractures.
• Resorption of distal digital bone (likely due to chronic intermittent ischemia).
• GI Tract: dysphasia due to esophageal fibrosis, small bowel hypomotility due to fibrosis(can lead to malabsorption)
• Psuedo-diverticulum (rare but diagnostic) sacular out pouching of both inner and outer smooth muscle layers
• Kidney: renal crisis: RP of renal vasculature Þ hypoperfusion, high rennin Þ accelerated hypertension
• Results in Acute renal failure and Microangiopathic hemolysis – destruction of RBC going thru narrow b.v.
• Managing hypertension dramatically improves this otherwise fatal condition (benefit of early treatment)
• Heart: insignificant Pericarditis occurs, generally without ß ß cardiac output
• Coronary arteries not involved but intramyocardial arteries are Þ patchy myocardial fibrosis Þ ß contractility
• tachyarrhythmia’s tend to develop (irregularities of the heart beat due to involvement of conduction system)
• Pulmonary System:
• secondary pulmonary hypertension due to scleroderma of pulmonary vasculature (Limited form)
• fibrosis of pulmonary parenchyma (tends to occur in patients with diffuse disease).
• Nervous System: Entrapment neuropathies due to adjacent fibrosis: trigeminal neuralgia, carpal tunnel; no CNS involvement

Pathogenic Factors – Vascular-endothelial injury Þ fibrosis; Immuno dysfunction-B and T cells; abnormal fibroblast physiology

• no cure; individualize therapy
• NSAID, corticosteroid.
• More Direct Treatment: Ca⁺⁺blockers,: nifedipine, D-penicillamine for skin/lung problems, cyclophosphamide (highly toxic)