Anesthetic Implications of Polyautoimmunity in Surgical Patients - PowerPoint Presentation

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Anesthetic Implications of Polyautoimmunity in Surgical Patients

Ella Obrosky, SRNA, University of Pittsburgh Nurse Anesthesia Program

Slide2

Introduction to Autoimmune Disease

ADs are a heterogeneous group of diseases characterized loss of self-tolerance

Significant

burden on health care, economic costs, quality of

life

Approximately 5% of people worldwide develop one or more

ADs,

80

%

are

women

.

AD is the leading

cause of disability in women

.

Problems affecting anesthesia care include diverse and complex disease presentation, polyautoimmunity and insidious development of complications

Slide3

Objectives

Learners will be able to:

Define

Autoimmune Tautology and describe the three lines of evidence for this

theory

Identify

key components of autoimmune pathophysiology/pathogenesis

Identify

common effects and symptoms of autoimmune pathology by body

system

Identify

common autoimmune diseases frequently associated with

poly-autoimmunity

Identify significant perioperative complications in patients with autoimmune disease and polyautoimmunity

Identify anesthetic implications and interventions to reduce the risk of perioperative complications in patients with autoimmune disease.

Slide4

Autoimmune Tautology

ADs share:

Pathophysiologic

mechanisms

Signs

and symptoms

Complications

Autoimmune Tautology

Familial aggregation

Polyautoimmunity

Shared

genetic

factors

Many factors contribute to variable clinical presentations of autoimmunity

Described as a kaleidoscope of autoimmunity

T

arget

cell, affected organ, gender, ancestry, trigger

factors and age

of onset

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Familial Aggregation

Defined as presence of diverse ADs in multiple members of a nuclear family

First

clue that ADs were

related:

More common in twins > siblings & parents > other relatives

Familial

clustering is demonstrated in a multitude of studies over decades of

research

Clustering/aggregation/recurrence is more

common than specific disease

recurrence, known as familial

autoimmune

disease

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Polyautoimmunity

Presence of more than one AD

Three or more: multiple autoimmune syndrome (MAS)

Secondary autoimmune disease is no longer used to describe the phenomenon

Instead, polyautoimmunity appears to be a reflection of diverse manifestations of both disease-specific and non-specific autoantibodies.

One example study of 1083 patients – 34.4% had more than one AD

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Polyautoimmunity Examples

Specific common clusters have names

Type 1 MAS:

MG, thymoma, PM, GCA with myocarditis

Type

2 MAS : Sjogren’s, RA, PBC, AI cirrhosis, SSc, AI thyroid disease

Type 3 MAS : AI thyroid disease, MG and/or thymoma, Sjogren’s, pernicious anemia, ITP, Addison’s, DM1, vitiligo, AI hemolytic anemia, SLE, dermatitis herpetiformis

Schmidt syndrome: Hashimoto’s + AI adrenal insufficiency/Addison’s

Slide8

Commonly Associated ADs

Diabetes: 15% have 2nd AD

Thyroid disease, Addison's, Myasthenia Gravis

AI Thyroid Disease

Myasthenia Gravis

Myasthenia Gravis

RA, Lupus, Pernicious Anemia, Pemphigus, ITP, Hemolytic anemia, Multiple sclerosis, Ulcerative colitis, Sjogren's, Sclerosis, Polymyositis, Thyroid disease

Eaton-Lambert

Thyroid disease, Collagen-Vascular Disease

Polymyalgia Rheumatica

Lupus, Giant cell arteritis in 15%

Polymyositis,

Dermatomyositis

RA, Lupus, Sclerosis

Primary Biliary Cirrhosis

RA, Pernicious anemia, Sjogren's, pyoderma gangrenosum, Antiphospholipid syndrome

Primary

Sclerosing

Cholangitis

IBD (UC>CD), DM, Psoriasis, APS

Rheumatoid Arthritis

Myasthenia Gravis, Pemphigus, Lupus, PM/DM

Systemic Lupus Erythematosus

Pemphigus

Sclerosis

PM/DM, Myasthenia Gravis

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Shared Genetic Factors

Not

all AD’s share the same genetic

susceptibilities

At least 18 common non-major HLA complex loci clusters implicated in AD

Disease-specific

Non-disease specific

Individual genes are not sufficient to explain heritability

Individual variants only add incremental risk 1.1-1.5 fold

Only 10-15% of inherited risk could be explained as of 2010

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Pathogen Triggers

Pathogens: viral, bacterial, fungal, vaccines

ADs are commonly preceded by URI, gastroenteritis

Viruses: EBV, CMV, HSV, adenoviruses

Even oral and intestinal microbiomes are implicated in RA, IBD, reactive arthritis, ankylosing spondylosis, psoriatic arthritis, etc.

Vaccines: bi-directional

Can prevent infections that can trigger AD

Can trigger AD

Example: flu

vaccine and

Guillain-Barré

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Environmental Triggers

Smoking associated

with

trigger or exacerbation of at least 10 different ADs.

Organic solvents,

Occupational dust

Sunlight

Gluten

Stress & hormones

Drugs:

Over 80 medications are known to induce Lupus alone, many others can also induce autoimmune hemolytic anemia.

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Autoimmune Pathogenesis

Presence of genetic susceptibilityExposure to triggering factors Abnormal immune response Self-tolerance is reduced or lostAuto-antibodies: systemic tissue-specificorgan-specific

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Tissue Damage

Direct and indirect damage

Inflammation: cytokines, prostaglandins, reactive oxygen species

Immune complex deposition

Direct immune attack:

Antibodies, B

cells, T cells, macrophages, etc.

Loss of regulation of autoreactive

cells

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Mosaic of Autoimmunity

Simple or stereotypical disease descriptions are no longer useful in

AD

Only 50% of patients with lupus will have a butterfly rash

Many symptoms are common to multiple diseases

Arthralgia, arthritis, alopecia, fatigue, photosensitivity, Raynaud’s phenomenon, vasculitis, coagulopathy, skin rashes

Combination of genetics, triggers, specific autoantibodies involved result in diverse presentations of even single diseases

Change to one component of the immune system can change the presentation of autoimmunity and induce a second disease

Splenectomy, thymectomy, immunosuppressants

Individual phenotypes (presentation) change over time

Severity varies by gender and age of onset

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Disease Classification by Body System

Primary System

Diseases

Vascular

Vasculotides, Raynaud's phenomenon

Collagen-Vascular

Polymyositis/dermatomyositis, Systemic sclerosis (scleroderma), Rheumatoid Arthritis, Systematic Lupus Erythematosus, Sjögren's syndrome

Pulmonary

Goodpasture

syndrome, Idiopathic pulmonary hemosiderosis, Pulmonary alveolar proteinosis,

PNS, CNS

Vitamin B12 deficiency, Guillain-Barré syndrome, Eaton

Lambert

Myasthenic syndrome, Multiple Sclerosis

Neuromuscular

Polymyositis/dermatomyositis, Myasthenia Gravis, Eaton Lambert Myasthenic syndrome

Joints/Rheumatic

Rheumatoid Arthritis, Ankylosing Spondylitis, Psoriatic arthritis, Reactive arthritis (Reiter's), Relapsing polychondritis, Polymyalgia rheumatica,

Systemic

Lupus Erythematosus

Cutaneous

Psoriasis, Sjögren’s syndrome, Bechet’s disease, Bullous pemphigoid, Pemphigus vulgaris, Henoch-Schönlein purpura

Hepatobiliary

Autoimmune hepatitis, Primary

Biliary

Sclerosis, Primary Sclerosing Cholangitis

GI

IBD (ulcerative colitis,

Crohn's

disease), celiac disease (gluten-sensitive enteropathy)

Heme

Pernicious anemia, B12 deficiency, AI hemolytic anemia, HIT type 2, Idiopathic thrombocytopenic purpura (ITP), Antiphospholipid syndrome

Renal

Goodpasture

syndrome, IgA nephropathy, Systematic Lupus Erythematosus, rapidly progressive glomerulonephritis

Endocrine

Diabetes Type 1, Addison’s disease, Hashimoto’s thyroiditis, Graves' disease, Reidel’s thyroiditis

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Vasculotides

Type of Vasculitis

Diseases

Large vessels

Bechet's disease, Cogan's syndrome, Giant cell arteritis (Temporal), Polymyalgia Rheumatica, Takayasu's arteritis

Mostly medium vessels

Buerger's disease (thromboangiitis obliterans), Kawasaki's disease, polyarteritis nodosa, cutaneous vasculitis

Mostly small vessels

Churg-Strauss syndrome, cryoglobulinemia vasculitis, IgA vasculitis, Henoch–Schönlein purpura (HSP), Hypersensitivity vasculitis, microscopic polyangiitis, Wegener's granulomatosis (granulomatosis with

polyangiitis

)

Variable

Medication-induced

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Vascular Complications

Diseases

General vasculitis

Amyloidosis, Ankylosing spondylitis

Dermatomyositis

, Diabetes type 1, Pyoderma gangrenosum, Rheumatoid arthritis, Lupus, Scleroderma, vasculotides

Aortic dissection

Ankylosing spondylitis

Aortitis/dilation

Ankylosing spondylitis, Rheumatoid Arthritis

Pulmonary artery aneurysm

Bechet's Disease

Vasculitis of Vasa Vasorum

Rheumatoid arthritis, Kawasaki disease

GI vasculitis

IBD, microscopic polyangiitis, Dermatomyositis, Rheumatoid arthritis

Raynaud’s phenomenon

vasculotides, PM/DM, Rheumatoid arthritis (uncommon), Sjogren’s (20-30%), Lupus (20-30%),

Scleroderma

(85-95%), Autoimmune hemolytic anemia.

Renal vasculitis

Goodpasture

syndrome, Dermatomyositis

CNS vasculitis

Giant cell arteritis, Takayasu’s arteritis, Polyarteritis nodosa, Wegener's

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Anesthetic Considerations - Vascular

Remember vasculitis in one place most likely means vasculitis any place

.

Preoperative assessment should focus on end-organ complications, known and unknown

May need renal, cardiac, neuro or pulmonary assessment/evaluation if severe disease

Plan

for careful maintenance of baseline

MAP

ART line

– avoid when unnecessary. Assess hand involvement before placing radial or brachial lines

Hypertension

– treat with nicardipine, sodium nitroprusside or nitroglycerine rather than inhaled agents.

Temperature

– some vasculopaths are more prone to clotting and/or vasospasm when they get cold. Keep them

WARM

, especially in the presence of Raynaud’s.

Pathergy

– present especially in Bechet’s, pyoderma gangrenosum, SLE, psoriasis. Will result in new lesions at the puncture site. Consider whether punctures can be avoided. If invasive monitoring is needed consider limiting to single site for central access and monitoring and one large IV.

Vision

– ASK specifically about vision history.

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Cardiac Complications

Diseases

Accelerated atherosclerosis/CAD

DM1, PAN, Psoriasis, RA, SLE, SSc, Takayasu’s, Buerger’s, TTP

Ischemia

(other causes)

AkS,

Cogan's, Autoimmune thyroid disease, Kawasaki's, PAN PM/DM RA, SLE, Takayasu's

Hypertension

CSS,

Goodpasture,

Grave’s, PAN, SLE

Myocardial

fibrosis

Amyloidosis

, AkS, SSc, PM/DM, SSc

Myocarditis/endocarditis

AkS, BD, CSS, IBD, PM/DM, RA, SLE

Valvular

dysfunction

Amyloidosis

, AkS, BD, PM/DM, reactive arthritis, relapsing polychondritis, RA, SLE, Takayasu’s, WG

Pericardial

effusion, lesion, inflammation

Amyloidosis

, BD, Hashimoto’s, IBD, PAN(acute), reactive arthritis, RA, SLE, SSc, WG

LV

dysfunction

SLE, MG, PAN, PM/DM, PBC/PSC, RA, SLE, SSc

RV

dysfunction

SLE, SSc, PBC/PSC, RA, SSc

Cardiomyopathy

amyloidosis, Cogan’s, MG, PAN, RA, SLE, WG

CHF

amyloidosis, CSS, Hashimoto’s, PM/DM, SLE, SSc

Conduction defects,

arrhythmias

amyloidosis, AkS, AI thyroid, Kawasaki’s, MG, PAN, PM/DM, RA, SLE, SSc, Takayasu’s, WG

Coronary

vasculitis

CSS, Cogan’s, PAN, RA, SLE, SSc, Takayasu’s, WG

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Cardiovascular Risk Factors

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Atherosclerosis

Slide23

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ANS Dysfunction

Disease ExamplesAmyloidosisBechet’sDiabetes type 1Guillain-BarreMyasthenia Gravis & ELMSSclerodermaMultiple sclerosisSjogren’sVasculotides

ANS Complications

Vasomotor instability

Autonomic hyperreflexia

Inability to sweat, dry mouth, constipation, urinary retention, gastroparesis

Sphincter incompetence

Erectile dysfunction

Resting tachycardia

Orthostatic intolerance

Silent Ischemia

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Anesthetic Considerations - Cardiovascular

Anesthetic choices should be tailored to the degree of myocardial dysfunction present.

Expect vasomotor instability in patients with ANS dysfunction. Consider assessing in preoperative area.

Basic info:

Thorough assessment of cardiovascular history in ALL AD patients.

ECG for ALL AD patients.

Listen to those valves!

Muffled heart sounds, pericardial friction rub, pulsus paradoxus, unexplained hiccups, hepatojugular reflex – Potential pericardial effusion.

When in doubt refer for cardiac clearance and/or testing

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ANS Assessment

Test

 

Normal Results

Poor Man's Version

HR response to Valsalva maneuver (PNS)

PNS

Normal ratio of longest R-R interval to shortest is >1.21

Does the HR slow down appropriately?

HR response to standing from supine (PNS)

PNS

Normal ratio of longest R-R interval around 30th beat (nadir of brady rate) to R-R interval around 15th beat (peak of tachy rate) is >1.04

Does the HR speed up a bit, then slow down a bit?

HR response to deep breathing (PNS)

PNS

6 deep breaths in one minute. Normal MEAN of difference between peak HR and low HR should be >15 bpm

Does the HR speed up and slow down with slow deep breathing?

BP response to standing (SNS)

SNS

Normal drop is <10 mmHg SBP. >20 drop SBP or >10 drop DBP or HR increase >20 is abnormal.

Hey this one is easy!

BP response to sustained handgrip (SNS)

SNS

Maintain a 30% maximum squeeze for 5 minutes. BP every minute. Initial DBP (low) subtracted from just before release (high) should be >16 mmHg

Hey this one is easy!

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Pulmonary

Complications

Diseases

Pulmonary Hypertension

Autoimmune

hepatitis, APS, BD, RA, SLE, SSc (asymptomatic!), Takayasu’s (50%)

Pulmonary Vasculitis/capillaritis, hemorrhage/hemoptysis

APS, CSS (reactive airway)

Goodpasture,

SLE, WG, other vasculotides

Diffuse interstitial disease/fibrosis

AkS, IBD, PM/DM, RA (rare), SLE, SSc (80%)

Diffuse alveolar disease, deposits, infiltrates, lesions, tumors

Amyloidosis, AkS, CSS PG, RA, SLE, WG

Pleural effusions

AkS, CSS, RA, (very common), SLE

Restrictive pattern

AkS, CSS, PM/DM, RA, SLE, SSc, WG

Obstructive airway/bronchospasm

BD, CSS, WG

Respiratory muscle weakness, +/- aspiration

ELMS, Graves, MG, MS, PM/DM, SLE, SSc

Chronic cough

Sjogren’s, PM/DM, SLE

Dyspnea

DM/PM, SLE

↓DLCO

CSS,

Goodpasture,

PM/DM, RA, SLE, SSc, vasculotides

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AkS: Consider ALL a difficult airwaySLE: Laryngeal inflammation, VC palsy/paralysis in up to 1/3. AFOI if voice changes.RA: Significant risk of AO instability and SC impingementWegener’s: Major airway complications due to upper and lower airway lesions, destruction of bone and tissue, laryngeal and tracheal stenosis, epiglottis destruction

Airway

Complications

Diseases

Oral/laryngeal lesions, fragility, pathergy

Amyloidosis, BD, PV/BP, psoriasis, PG, RA, Sjogren’s, SLE, SSc, Wegener’s

Nasal/sinus changes

CSS, SSc, WG

Tongue

Amyloidosis (large), Hashimoto’s (large), B12 deficiency (atrophic)

Subglottic

narrowing

RA, SLE, WG (major source of M&M)

Cricoarytenoid arthritis

Reactive arthritis, RA, SLE

Loss of TMJ mobility

AkS, PM/DM, RA, SSc (skin related)

Loss of c-spine mobility

AkS, DM1, RA, SSc, Takayasu (may limit CBF on extension)

Dysphagia or CN weakness

Graves, GBS, MG, PM/DM, RA, Sjogren’s, SSc

Esophageal dysmotility, GERD, delayed gastric emptying

DM1, Hashimoto’s, PM/DM, SSc/CREST

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Airway Considerations

Dangerous airways in multiple diseases

Oral lesions = fragility, risk of hemorrhage

Pathergy = risk for postoperative obstruction

Laryngeal involvement and narrowing – Glide or FOI

Neck clearance – RA, AkS

Specifically assess subjective tolerance with extension in Takayasu’s and other vasculitis, RA, AkS, SLE

Specifically ask about voice changes in ALL rheumatic cases

When in doubt bring the difficult airway cart and use FO scope

Emergency subglottic airway can be difficult in rheumatic patients!

Pulmonary assessment is likely sufficient with careful questioning of patient symptoms and auscultation of lung sounds

Delay/cancel elective surgery if patient presents with respiratory symptoms that have not been evaluated

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CNS ComplicationsCNS VasculitisHeadaches, confusion, infarctSeizuresDecreased BF at carotid or vertebral arteries (especially Takayasu’s)Myelopathy, demyelination, SC lesionsPsych disturbances, dementia, cognitive decline (Especially SLE)Hearing disorders (Especially WG)Cortical visual disturbances

Disease Examples

Diabetes type 1

Guillain-Barre

Lupus

Multiple sclerosis

Myasthenia Gravis & ELMS

Pernicious anemia, B12 deficiency

Rheumatoid arthritis

Scleroderma

Sjogren’s

Vasculotides

Slide31

Neurologic Considerations

All patients with vasculitis or potential vasculitis have an increased risk for neurologic symptoms

Anesthesia can unmask insidious development of complications

Assess EYE history and hearing history, even in young patients

Perform at minimum a mini mental status exam, consider more detailed exam to elicit subtle deficits.

Carefully maintain MAP at

baseline

Be cognizant of increased prevalence of dementia and psych disturbances that can both present early, or after prolonged disease

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Pathophysiology of Neuropathy

Slide33

PNS ComplicationsVasculitis of vasa nervorumDemyelination, inflammationCranial nerve dysfunctionPeripheral neuropathy (MOST ADs)ParesthesiaWeakness, myopathy, myalgiaCompression syndromes

Disease Examples

Graves' & Hashimoto's

Diabetes type 1

Guillain-Barre, CIDP

SLE, RA & connective tissue diseases

Multiple sclerosis

Myasthenia Gravis & ELMS

Pernicious anemia, B12 deficiency

Polymyositis, Dermatomyositis

Polymyalgia Rheumatica

Scleroderma

Sjogren’s

Vasculotides

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Neuropathy Considerations

Assess all patients with AD for history of symptoms of neuropathy and paresthesia

Anesthesia, stress, surgery, infection, inflammation, temperature derangements can all unmask subclinical neuropathy

.

Double Crush Phenomenon

- subclinical processes are potentiated or unmasked by a second attack on a nerve pathway from positioning, hypotension, inflammation or medications.

AVOID nitrous oxide

in all patients with demyelinating processes, neuropathy, B12 deficiency.

Assess carefully in postoperative period.

Direct injury or ischemia manifests almost immediately

Inflammatory processes develop over days to weeks

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Neuromuscular Considerations

Specifically assess for weakness or history of symptoms

preoperatively

Discuss possible postoperative ventilation needs

AVOID neuromuscular blockade

when possible.

TOF monitoring in unaffected muscle groups may overdose affected groups.

TOF monitoring affected groups may under-dose unaffected groups.

Titrate carefully for surgical needs only.

AVOID hyperthermia and hypothermia

in patients with known conduction deficits because changes to temperature can produce weakness or even full conduction block.

Be vigilant for neuromuscular weakness on emergence and postoperatively

Residual weakness, sensitivity or disease flare

Assess EOM

strength when patient fully awake

.

Slide36

Joint Manifestations

Spine involvement is common and varies by disease. AkS and RA should always be a red flag for c-spine problems.Atlantoaxial instability and subluxation are present in up to 40% of patients with RA and can remain asymptomatic despite significant spinal cord compression.

Primarily Rheumatic DiseasesAnkylosing spondylitisPsoriatic arthritisRheumatoid arthritisReactive arthritis (Reiter's syndrome)Relapsing polychondritisPolymyalgia rheumatica

Joint

Involvement

Systemic Lupus Erythematosus: most common symptom

Crohn's 10-20%

Ulcerative colitis 2-7%

Polymyositis & Dermatomyositis

Pyoderma Gangrenosum

Sjogren's syndrome

Scleroderma

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Joint Considerations

Preoperative assessment of major joint function imperative for safe positioning and airway maneuvers

Joint

involvement can herald more serious disease

complications

Prolonged steroid use may result in osteoporosis, osteomalacia, pathological fractures.

Severe rheumatic disease may require continuation of NSAIDS or antiplatelet medications during the perioperative period

Slide39

Primary Cutaneous DiseasesBechet's diseaseBullous Pemphigoid & Pemphigus VulgarisDermatomyositisHenoch-Scholein PurpuraPsoriasis & Psoriatic ArthritisLupus: Only 50% will have butterfly rashSclerosis & CREST syndromeSjogren's syndromeWegener's granulomatosis

Secondary Skin InvolvementAmyloidosisAutoimmune hepatitisAutoimmune thyroid diseaseCrohn's diseasePrimary biliary sclerosisReactive arthritisRheumatoid arthritisUlcerative colitisVasculotides

Rashes, plaques, texture, color changes, thickening

Pyoderma gangrenosum, erythema nodosum,

pathergy

common

Slide40

Skin Considerations

Specifically assess preoperatively

History of skin involvement

Oral lesions

Check current rash for signs of untreated infection

Joint limitation

Cricothyroid accessibility

Venous access

Avoid punctures to affected sites and in the presence of pathergy/Koebner

Consider CVL instead of multiple IV’s

Consider TEE or other noninvasive monitoring versus ART line

Slide41

Renal ComplicationsProteinuriaNephrotic syndromeGlomerulonephritisRenal tubular acidosisRenal failureRenal artery stenosisRenal calculiUreteral obstructionNephrotoxicity

Disease ExamplesAutoimmune hepatitisAutoimmune thyroid diseaseAmyloidosisCrohn's disease & Ulcerative colitisDermatomyositisDiabetes type 1Primary biliary cirrhosis & Primary sclerosing cholangitisPsoriasisRheumatoid arthritisSclerodermaSjogren'sVasculotides

Major primary renal involvement in Goodpasture and Wegener’s – 12-50% require dialysis or transplant. Rapidly Progressing Glomerulonephritis (RPGN), IgA nephropathy

Major complication in Lupus – up to 60%, with 10-20% requiring dialysis or transplant

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Renal and Hepatic Considerations

Preoperative labs should be assessed for all AD patients

Correct hypovolemia, electrolyte imbalances as applicable

Avoid decreasing renal and hepatic blood flow

Advocate for your patient in RCM exposure

Consider early and aggressive treatment with fluids, bicarb, n-acetylcysteine.

Choose anesthetic agents accordingly if renal or hepatic disease is present

Slide43

Ophthalmologic ComplicationsConjunctivitisIritis, episcleritis, scleritisUveitisLesions, ulcersOcular vasculitis or inflammationSicca syndrome, keratoconjunctivitis siccaEye painOptic neuritisAltered pupillary light responseDiplopia, EOM weakness, gaze abnormalityPhotophobia, unilateral blindness, blurring, field cuts

Diseases

Ankylosing spondylitis - 40% unilateral uveitis

Autoimmune thyroid disease

Bullous pemphigoid & Pemphigus vulgaris

IBD - 1-10% with uveitis

Lupus

Multiple sclerosis

Myasthenia gravis - 10% have only ocular symptoms

Polymyositis

Reactive arthritis, Rheumatoid arthritis, psoriatic arthritis

Sjogren's

Vasculotides, especially Bechet's & Giant cell arteritis

Vitamin B12 deficiency

Wegener's - lesions & 50-60% have ocular vasculitis

Slide44

Eye Considerations

Preoperative assessment for all AD patientsCurrent or previous visual deficitsLesions, dryness, inflammation or any other physical signsCoach susceptible patients with history of potential for postoperative flare or exacerbationMeticulous eye careConsider goggles in patients with history or potential eye involvementThorough and deliberate postoperative evaluationPotential for treatment of visual changes, deficits, eye inflammation flares if promptly recognizedThrombosis origin: anticoagulation. Inflammatory origin: corticosteroids. Flares: plasmapheresis

Slide45

Hematologic Complications

Diseases

Anemia

Graves (mild), Hashimoto’s (mild), RA (most), Sjogren’s, Lupus,

Scleroderma

Hemolytic anemia

AI hemolytic anemia, Lupus, Drug-induced

Leukopenia

Rheumatoid arthritis, Sjogren’s, Lupus

Megaloblastic anemia

Vitamin B12 deficiency, Crohn's disease, Ulcerative colitis

Thrombocytopenia

Graves', ITP, TTP, Lupus, Vitamin B12 deficiency.

Usually well tolerated >50k

Hypercoagulability

APS

, Bechet's, Primary sclerosing cholangitis, Primary biliary sclerosis, Lupus, TTP, Vitamin B12 deficiency, HIT type 2, Crohn's, Ulcerative colitis

,

vasculotides

Slowed primary hemostasis

ITP, Primary sclerosing cholangitis, Primary biliary sclerosis

Slide46

Coagulopathy

APS

: recurrent arterial and venous thrombosis

APLs may be present in AI hepatitis, SLE

Frequently subclinical until a critical ‘second hit’ such as surgery, pregnancy, trauma

Crisis: CAPS – Catastrophic Antiphospholipid Syndrome

Virchow’s

Triad:

Vascular injury (or inflammation)

Venous stasis (or arterial)

Hypercoagulability

Standard coagulation tests will frequently not reveal hypercoagulability in the face of AD and even may be elevated.

Slide47

Antiphospholipid syndrome

Slide48

Coagulation Considerations

Aggressive VTE prophylaxis is frequently warranted in AD patientsAnesthesia providers can advocate Potential for reduced protamine dosesEarly threshold to use TEGKeep patients WARMAvoid hemoconcentrationMonitor for CAPS in APS especially undergoing CPB

Slide49

Catastrophic Antiphospholipid Syndrome

CAPS is a rare syndrome of intravascular thrombosis and multi-organ failure

P

recipitating

factor: surgery, infection,

medication

APL’s when triggered activate the clotting cascade

Differential: CAPS, hemolytic-uremic syndrome, DIC, HIT

Treatments

Anticoagulation

A

nti-platelet medications

Immunosuppression

Plasmapheresis

IVIG

Slide50

Common Outpatient Medications

Immunosuppressants, with few exceptions, should be continued during the perioperative period.

Corticosteroids should not be interrupted

Stress doses not usually needed, but may be useful in adrenal destruction

Sometimes NSAIDS and antiplatelet medications need to be continued

Anticholinesterase medications should be continued and may require dosing adjustments

Slide51

Preclinical Disease or Suspicion

Prodromal illness is common in autoimmune disease

Abnormalities in laboratory markers of disease before the onset of clinical sign and symptoms

Wide variability in diagnosis and treatment practices early in disease processes, in part due to vague or nonspecific presentations of disease

Other risk factors: known other autoimmune disease, autoimmune disease in nuclear family, Native American ancestry

Consider in the presence of ongoing

fatigue and arthralgia.

Be SUSPICIOUS

Slide52

Preclinical Disease

TIDM

: 2+ autoantibodies 100% predictive

IBD

: +autoantibodies present in 24-31% that later develop UC or CD

WG

: ANCA’s elevate prior to symptoms

APS

: antibodies are present prior to embolic events, sometimes present prior to SLE

RA

: RF and ANCA’s increase prior to development of symptoms

SLE

: ANAs present prior to symptoms, APL’s with + Coombs highly predictive

Sjogren’s

: commonly positive antibodies years prior to development of symptoms

Celiac

: antibodies increase prior to onset of symptoms

Autoimmune thyroid disease

: antibodies often present and highly predictive

Autoimmune biliary disease

: majority with Sjogren’s and antibodies develop symptoms

Autoimmune adrenal disease

: in DM1 patients antibodies precede adrenal destruction

Slide53

!WARNING SIGNS!

Active flare activity

Vasculitis, vasculitis, vasculitis!

Raynaud’s phenomenon, other

abnormal hand

circulation

Rashes, Pathergy or Koebner phenomenon

Vitamin B12 deficiency not otherwise explained

Unexplained neuropathy, especially in adult females without diabetes

Unexplained clotting history, especially in the face of normal or elevated coagulation times

Nuclear family history of AD, especially if multiple family members and/or multiple diseases

Multiple non-specific sequelae of autoimmune diseases

Restless leg syndrome

Narcolepsy

Fibromyalgia

What is that rash?!

Oral or eye lesions

Slide54

Conclusions

AD is more diverse, complex and prevalent than many providers are aware

Complications are also often diverse and complex

Proof of polyautoimmunity is not necessary in order to be suspicious and vigilant

Insidious development of complications is a major source of risk factors for cardiovascular disease and other end-organ involvement

Patients with more complications, more diseases are sicker, even if their symptoms are not severe. Disease activity level is often highly correlated with complications, not just severity of symptoms

The future will undoubtedly provide more research to guide evidence-based practice

Current evidence suggests more aggressive preoperative assessment and perioperative care is warranted to reduce the likelihood of complications related to the stresses of surgery, disease flares, end-organ involvement.

Slide55

Abbreviations

AkS – Anklyosing spondylitisAPS – Antiphospholipid syndromeBD – Bechet’s diseaseCSS – Churg-Strauss syndromeCD – Crohn’s diseaseDM – dermatomyositisDM1 – Diabetes type 1ELMS – Eaton Lambert myasthenic syndromeGCA – Giant cell arteritisGBS – Guillain-Barre syndromeHSP – Henoch–Schönlein purpuraHIT2 – Heparin-induced thrombocytopenia type 2MS – Multiple sclerosis

MG

– Myasthenia gravis

PAN

- Polyarteritis nodosa

PMR – Polymyalgia rheumatica

PM

–

Polymyositis

PBC

– Primary biliary cirrhosis

PSC – Primary sclerosing cholangitis

PG – Pyoderma gangrenosum

RA – Rheumatoid arthritis

SLE – Systemic Lupus Erythematosus

SSc – Systemic sclerosis (scleroderma

)

UC – ulcerative colitis

WG – Wegener’s (Granulomatosis with polyangiits)

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