PERIPHERAL VASCULAR DISEASE: A VASCULAR SURGEON’S POINT OF VIEW
Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by...
Transcript of Project: Document Title: Collagen Vascular Disease ... · – Disease process characterized by...
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Project: Ghana Emergency Medicine Collaborative Document Title: Collagen Vascular Disease: Considerations for Emergent Management Author(s): Joseph Hartmann, D.O., 2012 License: Unless otherwise noted, this material is made available under the terms of the Creative Commons Attribution Share Alike-3.0 License: http://creativecommons.org/licenses/by-sa/3.0/
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Collagen Vascular Disease -Considerations for Emergent
Management
Joseph H. Hartmann, D.O.
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Emergent Management ???
• Chronic conditions with long-term management decisions made by others
but • Because of their chronicity they present with
- flares of general disease process - end-organ involvement
• Occasionally determine the initial diagnosis
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Where are you taking me today?
• Raynaud phenomenon • Reactive arthritis (Reiter Syndrome) • Rheumatoid arthritis • Systemic sclerosis (Scleroderma) • Systemic lupus erythematosus • Polymyalgia rheumatica • Polymyositis / Dermatomyositis • Vasculitides
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Raynaud Phenomenon
• Definition: – Exaggerated vasospasm of digital / precapil-
lary arteries of fingers, toes, ears, nose, knees and nipples
– Initiated by exposure to cold or emotional stress
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Raynaud Phenomenon
• Etiology: – Unknown – May be first precursor of future connective
tissue disorder • Clinical Presentation:
– Triphasic – Typically begins in one finger then symmetric-
ally spreads to other fingers but usually spares the thumb
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Raynaud Phenomenon
• Triphasic progression – White – pallor
• Lack of arterial flow due to vasospasm
– Blue • Cyanosis from blood pooling
– Red • Reactive hyperemia
– Ischemic phases (white-blue) last 15-20 min 8
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National Heart, Lung, and Blood Institute, Wikimedia Commons
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Intermedichbo, Wikimedia Commons
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Niklas D, Wikimedia Commons
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Raynaud Phenomenon
• Relative temperature shifts may be provocative
• General body chill can trigger
• Fear / anxiety can trigger
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Raynaud Phenomenon
• Specific criteria – Symmetric episodic attacks – No evidence of peripheral vascular disease – No tissue gangrene, digital pitting, or tissue
injury – Negative nailfold capillary examination – Negative ANA, normal ESR
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Raynaud Phenomenon
• Nailfold capillary microscopy – – Place drop of immersion oil on periungual
area then examine with ophthalmoscope set at diopter 40 or a dissecting microscope.
– Should see regularly spaced capillary loops – Abnormal findings –
• Enlarged or distorted capillary loops • Relative paucity of loops
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Source Undetermined Source Undetermined
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Raynaud Phenomenon
• Treatment – Avoidance strategies
• Cold, nicotine, sympathomimetics (decongestants, diet pills, herbs containing ephedra)
– Drug therapy • Ca channel blockers, direct vasodilators (NTG,
hydralazine, minoxidil), sympatholytics (methyldopa, reserpine, prazosin), prostaglandins, anticoagulation / antithrombotic tx (aspirin, dipyrid-amole, heparin, LMWH)
• sympathectomy 18
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Raynaud Phenomenon • Severe ischemia
– Warm patient (body and digits) – Analgesics – Antiplatelet tx – aspirin – Vasodilator tx
• Nifedipine extended release 30-60 mg daily • Amlodipine 5-10 mg daily
– Topical NTG – Heparin / LMWH 24-72 hrs – Temporary chemical sympathectomy
• Digital or regional block – lidocaine/bupivicaine
– IV prostaglandin administration 19
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Reactive Arthritis (Reiter Syndrome)
• Definition: – Arthritis following a preceding infection without
intra-articular presence of the pathogen i.e. not a septic joint
• Etiology: – Seronegative (rheumatoid factor negative) – Spondyloarthropathy (very likely HLA-B27 pos) – Follows a GU or GI infection
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Reactive Arthritis
• Clinical Presentation: – Male 15 – 35 y/o – Asymmetric, oligoarthritis (2-4 joints) – Usually involves lower extremeties and sacroiliac
joints – Skin lesions resembling pustular psoriasis on
palms and soles – keratoderma blennorrhagicum – lesions on glans penis – balanitis circinata
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Centers for Disease Control and Prevention, Wikimedia Commons 22
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Source Undetermined Source Undetermined
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Reactive Arthritis
• Classic triad of Reiter syndrome – Nongonococcal urethritis (Chlamydia) – Conjunctivitis / anterior uveitis – Arthritis
• Following GI infection with Shigella, Sal-monella, Campylobacter, Yersinia, Clost-ridium difficile (?)
• Infection precedes arthritis by 2 - 6 wk
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Reactive Arthritis
• Treatment: – NSAIDs
• Naproxen 500 mg TID • Indomethacin 50 mg TID
– Intra-articular glucocorticoids – Expect resolution 3 – 12 months
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Rheumatoid Arthritis
• Definition: – Chronic, symmetric polyarticular synovial joint
disease • Nonarticular & Systemic Manifestations
– HEENT • Episcleritis
– Painless injection of episcleral vessels (self-limiting)
• Scleritis – Dark red / purple discoloration with marked ocular
tenderness – Potential for visual impairment and scleral rupture 26
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Source Undetermined
Source Undetermined 27
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Source Undetermined 28
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Rheumatoid Arthritis
• Cricoarytenoid joint – Dysphonia, hoarseness, stridor – If fix in closed position could require emergent
tracheostomy • Ligamentous destruction of transverse
ligament of C-2 with potential for cord compression
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Rheumatoid Arthritis
• Pulmonary – Pleural effusion * – Interstitial fibrosis – Pulmonary nodules
• Cardiac – Pericarditis * – Pericardial effusion 10% – Myocarditis – CAD – sudden death, MI *
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Rheumatoid Arthritis
• Renal – Focal glomerulonephropathy – Drug toxicity from treatment *
• Vasculitis – Distal infarcts, ulcerations, gangrene
• CNS – Generally spared
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Systemic Sclerosis - Scleroderma
• Definition: – Disease process characterized by progressive
fibrosis, vascular abnormalities and inflamma-tory processes that can be manifested quite locally or diffusely systematically with organ system involvement
• Etiology: – Poorly understood
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Systemic Sclerosis
• Clinical Presentation: – Often initial signs are a thickening, hardening
of the skin, usually fingers, hands and face and Raynaud phenomenon
– Female > Male – African-Americans tend to have worse
prognosis due to greater likelihood of having a more severe diffuse form
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Systemic Sclerosis
• Skin – Sclerodactyly – Telangiectasias – Digital ulcers – Calcinosis – Raynaud phenomenon
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Source Undetermined
In scleroderma, the abnormal build-up of fibrous tissue in the skin can cause the skin to tighten so severely that the fingers curl and lose their mobility.
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Telangiectasia
Dilation of small vessels and capillaries cause flat red marks to appear on the skin
Kerry J, Flickr
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Jmh649, Wikimedia Commons
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Source Undetermined
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Source Undetermined
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Systemic Sclerosis • Pulmonary
– Most common cause of death – Interstitial lung disease
• Alveolitis leading to eventual fibrosis
– Pulmonary vascular disease • Pulmonary hypertension
• Cardiac – Pericarditis w/wo effusion – Myocardial fibrosis
• Resultant ventricular dysfunction with diminished cardiac output
– Dysrhythmias • Fibrosis of conduction system resulting in sudden death
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Systemic Sclerosis • Renal
– Renal crisis • Acute onset of renal failure • Proteinuria and microscopic hematuria • Abrupt onset of hypertensive emergency
• Gastrointestinal – Hypomobility
• Esophageal dysmotility / GERD • Pseudo-obstruction, constipation • Teleangectasias with bleeding • Pneumatosis intestinalis
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Lipothymia, Wikimedia Commons
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Source Undetermined
Source Undetermined
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Systemic Sclerosis • Treatment
– Generally immunosuppressive therapy – Based on specific organ system involved – Emergently
• Pulmonary decompensation • Cardiac – effusion, failure, dysrhythmia • Renal crisis
– ACE inhibitor is first line antihypertensive agent – Captopril – Captopril + Ca channel blocker – Angiotensin receptor blocker for those who can not tolerate
ACE inhibitor
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Systemic Lupus Erythematosus
• Definition: – Chronic autoimmune disease characterized by
presence of autoantibodies with multi-organ system involvement
• Etiology: – Genetic predisposition and nebulous factors
combine to alter immune cell function resulting in production of autoantigens and thereby auto-antibodies with systemic consequences.
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SLE • Clinical Presentation:
– Female > Male 10 : 1 – Typical presentation 21 – 45 yrs of age – African American > Caucasians – Constitutional
• Wt loss, fever, myalgias, arthralgias • Fatigue often the most debilitating
– Skin • Butterfly malar rash – may be fleeting • Oral and nasal ulcerations
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Source Undetermined
Source Undetermined
Source Undetermined
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SLE • Pulmonary
– Pleurisy, effusion, interstitial lung disease, pulmonary hypertension
– “lupus lung” – alveolar hemorrhage • Cardiac
– Pericarditis *, effusion – Increased risk for CAD
• Renal – Lupus nephritis
• Elevated creatinine, proteinuria, hypertension 48
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SLE
• Neurologic – Cognitive defects, cephalgia, seizures,
peripheral neuropathies (stocking / glove), psychosis, stroke (antiphospholipid antibody syndrome)
• Musculoskeletal – Arthritis, atrophy, tendon rupture
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SLE • Treatment:
– Immunosuppression • Glucocorticoids • Methotrexate, cyclophosphamide, azathioprine,
mycophenolate, rituximab – Causes of death
• Early deaths – first few years – Active lupus (cardiac, renal, CNS dz) – Infection due to immunosuppression
• Late deaths – Chronic effects of lupus (ESRD, CAD) – Infection, malignancy
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Polymyalgia Rheumatica
• Definition: – Rheumatic condition frequently associated
with giant cell (temporal) arteritis • Etiology:
– Genetic predisposition – Relatively common – 50% of pts with GCA will develop PMR – 15% of pts with PMR will develop GCA
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Polymyalgia Rheumatica
• Clinical Presentation: – Age 50 or older at onset – Bilateral aching and morning stiffness which
lasts 30 min or more for 1 month or more involving at least 2 of 3 areas
• Neck or torso • Shoulders or proximal arms • Hips or proximal thighs
– ESR = 40 mm/hr or greater
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Polymyalgia rheumatica
Original Image: Twisp, Wikimedia Commons Modified Image Lena Carleton, University of Michigan 53
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Source Undetermined
Giant Cell Arteritis
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Polymyalgia Rheumatica
• treatment: – Prednisone 10 – 20 mg / day
• (compared to 60 mg / day dose for GCA)
– Rapid response is characteristic (often after first dose)
– Relapse is commonly seen requiring increase in prednisone
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Polymyositis / Dermatomyositis
• Definition: – Idiopathic inflammatory myopathies
• Etiology: – Genetic component with presumed environ-
mental triggers – Peak incidence between 40 – 50 yrs of age – Female : Male 2 : 1
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Polymyositis / Dermatomyositis • Clinical Presentation:
– Muscle weakness • Onset is insidious • Gradually worsening over months • Typically symmetric and proximal
– Myalgias / muscle tenderness occurs in 25-50% but is mild compared to PMR or fibromyalgia
– Dermatologic findings in DM • Gottron’s sign
– Erythematous, often scaly exanthem occurring symmetrically over MCP and IP joints and / or over extensor surfaces of elbows and knees resembling psoriasis
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Madhero88, Wikimedia Commons
Madhero88, Wikimedia Commons
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Polymyositis / Dermatomyositis – Heliotrope rash
• Violaceous eruption on upper eyelids, often with swelling
– Shawl sign and V sign • Diffuse flat erythematous lesion over chest and
shoulders (shawl sign) or over anterior neck and chest (V sign)
– Erythroderma • Extensive areas of skin redness (malar, forehead)
– Mechanic’s hands (DM or PM) • Rough, cracking skin at tips and lateral aspects of
fingers with irregular dirty appearing lines 59
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Madhero, Wikimedia Commons
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Madhero88, Wikimedia Commons
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Source Undetermined
Source Undetermined 62
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Polymyositis / Dermatomyositis • Diagnostic testing
– Elevated CK, LDH, aldolase, aminotransferases • Increased incidence of malignancy
associated with dermatomyositis • Treatment:
– Glucocorticoid regimen • initiate with high doses for several months to
establish disease control • Slow taper to lowest effective dose for 9 – 12 months
– Glucocorticoid-sparing regimen • Azathoprine / methotrexate 63
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Vasculitides • Large vessel
– Takayasu arteritis – Giant cell (temporal) arteritis
• Medium vessel – Polyarteritis nodosa – Kawasaki disease
• Small vessel – Churg-Strauss arteritis – Wegener’s granulomatosis – Henoch-Schonlein purpura 64