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Found in 95% of pts with SSc Digital arteries of fingers and toes
• Also tongue, nose, ears, nipples Appears suddenly as attacks
• Cold temperatures or stress Color changes
• PallorCyanosisRedness
CONDITIONS ASSOCIATED WITH RAYNAUD'S PHENOMENONImmune mediated
Systemic sclerosis (90%)Mixed connective tissue disease (85%)Sjögren's syndrome (33%)Systemic lupus erythematosus (10–45%)Polymyositis/dermatomyositis (20%)Rheumatoid arthritis (10–15%)Cryoglobulinemia and cryofibrinogenemia (10%)Arteritis (e.g. giant cell arteritis, Takayasu's arteritis)Antiphospholipid syndrome
Occupation related Vibration exposure
Cold injury (e.g. frozen-food packers)Polyvinyl chloride exposureAmmunition workers (outside work)
Obstructive vascular disease Atherosclerosis
Thromboangiitis obliterans (50%)Diabetic microangiopathyThoracic outlet syndrome (e.g. cervical rib)
Drug induced β-blockers (particularly non-selective)
Antimigraine compoundsSulfasalazineCytotoxic drugsCocaine abuse
Metabolic disorders Hypothyroidism
Carcinoid syndrome Infections Chronic viral liver diseases (hepatitis B and C)
CytomegalovirusParvovirus B19
Miscellaneous Neoplasm
Complex regional pain syndrome type 1PolycythemiaArteriovenous fistulaFibromyalgia syndrome
% values are percentage of patients with disease who have Raynaud's phenomenon
Vasoconstriction, cyanosis, hyperemia• Neurogenic
A and B adrenergic receptors have increase sensitivity or density A2C receptor felt to be major pathway for cold induced
vasoconstriction SNS has role – vibration of one hand, stress, CGRP Infusions of adrenergic agonist do not produce pathology
• Endothelium Endothelium dependant vasoconstriction abnormal in 2” Raynauds Asymmetric DiMethylArginine (ADMA) inhibits NO synthase Level increased in 2” Raynauds Endothelin I, angiotensin II, vWF levels high in 2” Raynauds
Blood Cells• Platelets aggregate, Tx A2, TGF beta• RBCs stiff• WBCs produce ROS
• Inflammatory/immune TNF, lymphotoxin, IC abnormalities seen
Definite• Repeated episodes• Biphasic color changes on cold exposure
Possible• Uniphasic change plus numbness or paresthesia
Primary• 4-15% of the
general population• Usually mild and not
associated with structural vascular changes or ischemic tissue damage
• Onset typically occurs between 15-25 years
Secondary• Age of onset usually
after 30 yrs old• More severe
symptoms• Ischemic injury• Enlarged capillary
loops and loss of normal capillaries in the nail folds
FEATURES SUGGESTIVE OF PROGRESSION OF RAYNAUD'S PHENOMENON TO CTD
ClinicalOlder age at onset (>35 years)Vasospasm all year roundAsymmetric attacksSclerodactylyDigital ulcerationFinger pulp pitting scars
LaboratoryIncreased inflammatory markersDetection of autoantibodiesIncreased Von Willebrand factor antigen
Nailfold microscopy Abnormal vessels
H&P• Concern for CTD?• Aggravating drugs?• Occupational or Hx suggesting a cause?
50% vibration workers 60% pts over 60 ASVD
• Nailfold Capillaries Labs
• CBC, CMP, TSH, ESR, CRP• Apropriate serologies
BetaBlockers Vasoconstrictors
• Clonidine, sympathomimetics, narcotics, ergotomines, serotonin agonists
Chemotherapy agents• Bleomycin, cisplatin/carboplatin,
vinblastine/vincristine Other
• Cyclosporin, interferons, estrogens Exposures
• Nicotine, cocaine, polyvinyl chloride
Raynaud’s phenomenon• Avoid cold exposure, layer clothing• Stop smoking• Vasodilator therapy
Nifedipine>Diltiazem>prazosin Topical nitroglycerin paste
• Other agents• Ischemic lesions
IV PGE1 or PGI2 Sympathetic blocks, amputation Bosentan, sildenafil, SSRIs,
Cold avoidance Warm clothing, gloves Stop smoking Withdraw medications Avoid vibration Avoid stress Rewarm with warm water or body
heat
Nifedipine, amlodipine, ditiazem, others
XR nifedipine preferred• 30-180 mg/day
XR amlodipine second choice• 5-20mg/day
Verapamil ineffective
Prazosin• Effective in cochrane review, may lose effect
PDE5 inhibitors• Small studies, sildenafil, tadalafil, verdenafil
Fluoxetine• Crossover, conflicting case reports
Losartan• One blinded study, reduced attacks, ACE data conflicting
Topical nitrates• Effective, many side effects (HA,dizzy)
Statin, NAC, Botox, gingko biloba Bosentan
• Approved in UK, 2 controlled trials, fewer NEW ulcers only
16 patients with disease resistant to vasodilators• 78% with systemic sclerosis• 11% with MCTD• 11% with no CTD• 33% had digital ulcers
50 mg sildenafil vs. placebo BID for 4 weeks
Frequency of attacks 35 vs. 52 (p=0.0064) Duration of attack 581 vs. 1046 minutes
(p=0.0038) Nailfold capillary blood flow velocity
quadrupled (p=0.0004)
Fries R et al. Circulation 2005;112:2980
Iloprost has the most trials• 6-10ng/kg/min x 72 hrs or 0.5-2ng/kg/min x 6hr x 5 days
Prostacyclin (PGI2) also effective• 7.5 – 10 ng/kg/min x 5 hr x 3 days
Epoprostanol not significant• 0.5 to 2 ng/kg/min for 1-3 days
Alprostadil (PGE1) ineffective Oral forms ineffective
Medical emergencyhospitalization Rest in warm environment Pain control Titrate CCB to full tolerated dose Local lidocaine or bupivicaine for rapid
chemical sympathectomy and pain relief (local block)
Heparin/LMWH for 24-72 hours Parenteral prostaglandin Surgical sympathectomy
Temp – lidocaine/bupivocaine (no epi!!)• Digital or wrist local block• If shows temporary effect, then surgery may
help Surgical digital
• Local, less invasive Surgical cervical
• Open or endoscopic
Exposure to cold above freezing On cold exposed skin
• Fingers, toes, feet, nose, knees, elbows Results in edematous, inflammatory
lesions Painful or pruritic Seen more in women, children,
elderly Most idiopathic and self limited
Associated with SLE• A less common form of Chronic Cutaneous
SLE• Increased mucin, +DIF on biopsy• Up to 20% may develop SLE
Especially if have DLE
TREX1 mutation• Familial chillblain and SLE• DNAse, blocks type 1 interferon response
Tissue damage from freezing Direct cell death and inflamation Ice crystals lyse cells Inflammatory reaction
• Thromboxane A2, Prostaglandin F2, bradykinin, histamine
• Tissue ischemia and necrosis
First Degree• Skin pallor and anesthesia
Second Degree• Blisters within 24 h, erythema
Third Degree• Hemorrhagic blisters - eschar
Fourth Degree• Involves muscle/bone – complete necrosis
Superficial/Deep
EXPOSURE!!! Prior cold injury Alcohol Tobacco Exhaustion Malnutrition Peripheral vascular disease Diabetes Mental illness
Ears, nose, cheeks, fingers, toes Cold, numb, clumsy Pallor, insensate Hard or waxy
Move to warm area Do NOT rewarm if may refreeze Avoid trauma, rubbing, harsh
rewarming Warm with warm water or body heat Transport for treatment Avoid walking on frostbitten feet if
possible
Rewarm 40-42C 104-108F water Anesthesia (opioids) Bulky dressing, elevation Infection control TPA if <24h from injury
• 2-4mg bolus, 0.5-1mgh infusion • Angiogram q8-12 H• Continue to perfusion or 48H
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