A patient with Chronic Fatigue Syndrome

Kristin Steffen, MDKristin Steffen, MD

2/22/062/22/06

I have no financial disclosures.

Objectives

Identify case definition for Chronic Fatigue Identify case definition for Chronic Fatigue Syndrome (CFS)Syndrome (CFS)

Recognize similarities between CFS and Recognize similarities between CFS and fibromyalgiafibromyalgia

Describe clinical presentation, prognosis of CFSDescribe clinical presentation, prognosis of CFS Review recommendations for diagnosis Review recommendations for diagnosis Identify 2 treatmentsIdentify 2 treatments Identify useful referencesIdentify useful references Informational presentation only (I am not soliciting Informational presentation only (I am not soliciting

referrals!!)referrals!!)

Case of AF

AF is a 35 yo F diagnosed with CFS in 1992 with AF is a 35 yo F diagnosed with CFS in 1992 with abrupt onset of symptoms following URIabrupt onset of symptoms following URI

Extensive negative work up for alternative cause Extensive negative work up for alternative cause (>14 physicians, every recommended test plus more (>14 physicians, every recommended test plus more than twice as many more)than twice as many more)

Extensive trials of experimental treatments (32+)Extensive trials of experimental treatments (32+) On SSI total disability, appealing employer decision On SSI total disability, appealing employer decision

to revoke employer-based disability benefitto revoke employer-based disability benefit Stable: neither improving nor worsening, currently Stable: neither improving nor worsening, currently

taking no specific pharmacologic therapy taking no specific pharmacologic therapy

1994 CDC Case Definition for CFS I. Clinically evaluated, unexplained, I. Clinically evaluated, unexplained,

persistent, or relapsing fatigue that is of new persistent, or relapsing fatigue that is of new or definite onset; it is not the result of or definite onset; it is not the result of ongoing exertion; is not alleviated by rest; ongoing exertion; is not alleviated by rest; and results in substantial reduction in and results in substantial reduction in previous levels of occupational, previous levels of occupational, educational, social, or personal activitieseducational, social, or personal activities

ANDAND

1994 Case definition of CFS, cont II. Four or more of the following symptoms that II. Four or more of the following symptoms that

persist or recur during six or more months of persist or recur during six or more months of illness that do not predate fatigue:illness that do not predate fatigue: Self-reported impairment in short term memory Self-reported impairment in short term memory

or concentrationor concentration Sore throatSore throat Tender cervical or axillary nodesTender cervical or axillary nodes Muscle painMuscle pain

1994 Case definition of CFS, cont II. Four or more of the following symptoms that II. Four or more of the following symptoms that

persist or recur during six or more months of persist or recur during six or more months of illness that do not predate fatigue:illness that do not predate fatigue: Multijoint pain without redness or swellingMultijoint pain without redness or swelling Muscle painMuscle pain Headaches of a new pattern or severityHeadaches of a new pattern or severity Unrefreshing sleepUnrefreshing sleep Post-exertional malaise lasting >24 hoursPost-exertional malaise lasting >24 hours

Conditions that exclude CFS Dx

Any active medical condition that may explain the Any active medical condition that may explain the presence of chronic fatigue…presence of chronic fatigue…

Any previously diagnosed medical condition Any previously diagnosed medical condition whose resolution has not been documented whose resolution has not been documented beyond reasonable doubt…beyond reasonable doubt…

Any past or current diagnosis of specified Any past or current diagnosis of specified psychiatric disorders…psychiatric disorders…

Alcohol or other substance abuseAlcohol or other substance abuse Severe obesity (BMISevere obesity (BMI>>45)45)

Conditions that do not exclude CFS Dx Any condition defined primarily by symptoms that Any condition defined primarily by symptoms that

cannot be confirmed by diagnostic laboratory cannot be confirmed by diagnostic laboratory tests…tests…

Any condition under specific treatment to alleviate Any condition under specific treatment to alleviate all symptoms related to that condition and for all symptoms related to that condition and for which adequacy of treatment has been which adequacy of treatment has been documented…documented…

Any condition, such as Lyme disease or syphilis, Any condition, such as Lyme disease or syphilis, that was treated with definitive therapy before the that was treated with definitive therapy before the development of chronic symptomatic sequelae.development of chronic symptomatic sequelae.

Conditions that do not exclude CFS Dx Any isolated or unexplained PE finding or Any isolated or unexplained PE finding or

lab or imaging test abnormality that is lab or imaging test abnormality that is insufficient to strongly suggest the insufficient to strongly suggest the existence of an exclusionary condition…existence of an exclusionary condition…

Idiopathic Chronic Fatigue

Clinically evaluated, unexplained chronic Clinically evaluated, unexplained chronic fatigue of greater than 6 months duration fatigue of greater than 6 months duration that does not meet criteria for CFS that does not meet criteria for CFS diagnosisdiagnosis

Similarities between CFS and Fibromyalgia Common in women Common in women Myalgias and fatigue in >90%Myalgias and fatigue in >90% Symptoms in common: cognitive and mood, Symptoms in common: cognitive and mood,

headache, nonrestorative sleepheadache, nonrestorative sleep No known causeNo known cause Clinical diagnosis (no specific diagnostic tests, Clinical diagnosis (no specific diagnostic tests,

except trigger points in fibromyalgia)except trigger points in fibromyalgia) Chronic symptoms, no highly effective treatmentChronic symptoms, no highly effective treatment

Fibromyalgia Diagnostic Criteria

Widespread body pain (left and right, above Widespread body pain (left and right, above and below the waist) and axial skeletal pain and below the waist) and axial skeletal pain (neck, chest wall, mid or low back)(neck, chest wall, mid or low back)

Presence in 11 of 18 tender point sites on Presence in 11 of 18 tender point sites on digital palpation with “an approximate force digital palpation with “an approximate force of 4 kg”of 4 kg”

Cause of CFS

UNKNOWNUNKNOWN Possible precipitants that have been studied Possible precipitants that have been studied

but have not been found to be causative:but have not been found to be causative: InfectionInfection Immune dysfunctionImmune dysfunction Endocrine-metabolic dysfunctionEndocrine-metabolic dysfunction Neurally-mediated hypotensionNeurally-mediated hypotension

Clinical Presentation with CFS

VariableVariable Sudden onset of fatigue after URISudden onset of fatigue after URI After URI, continued overwhelming fatigue After URI, continued overwhelming fatigue

plus add’l sx (e.g. altered sleep, cognition)plus add’l sx (e.g. altered sleep, cognition) Symptoms exacerbated by excessive Symptoms exacerbated by excessive

physical activityphysical activity No prior hx of backache/chronic headacheNo prior hx of backache/chronic headache

Clinical Presentation, cont.

Typically in formerly high functioning individualsTypically in formerly high functioning individuals Once inciting illness resolved, physical exam is Once inciting illness resolved, physical exam is

NORMALNORMAL Feel feverish, but normal tempFeel feverish, but normal temp Achy joints, but no findings on examAchy joints, but no findings on exam Muscle fatigue, but normal biopsiesMuscle fatigue, but normal biopsies Frequent sore lymph nodesFrequent sore lymph nodes Disabled by symptoms, but outwardly healthy Disabled by symptoms, but outwardly healthy

appearingappearing

Clinical Presentation, cont.

Patients may be accused of malingering by family, Patients may be accused of malingering by family, colleaguescolleagues

*Cognitive dysfunction: no specific pattern of *Cognitive dysfunction: no specific pattern of cerebral abnormalities uniquely characterize CFS cerebral abnormalities uniquely characterize CFS patients; most prominent features may be slowed patients; most prominent features may be slowed processing speed, impaired working memory, poor processing speed, impaired working memory, poor learninglearning (can’t “rule in” CFS by neuropsych testing, (can’t “rule in” CFS by neuropsych testing,

can’t “rule out” cognitive dysfunction symptom can’t “rule out” cognitive dysfunction symptom as not legitimate)as not legitimate)

Prognosis

Some recover completely, some recover and are Some recover completely, some recover and are able to work but have flares, some never recoverable to work but have flares, some never recover

Of those who recover, most recover within 5 yearsOf those who recover, most recover within 5 years As illness progresses reports of muscle pain and As illness progresses reports of muscle pain and

forgetfulness increase, depression decreasesforgetfulness increase, depression decreases Poorer prognosis when patient believes the illness Poorer prognosis when patient believes the illness

is due to a (given) physical causeis due to a (given) physical cause

Diagnosis

Clinical, based on typical presentation, case Clinical, based on typical presentation, case definitiondefinition

No diagnostic exam or test findings existNo diagnostic exam or test findings exist Purpose of evaluation is to identify and treat Purpose of evaluation is to identify and treat

any underlying contributing factorsany underlying contributing factors

Diagnosis

Recommended evaluation:Recommended evaluation: History and physical examHistory and physical exam CBC with diff, ESR, chemistries (electrolytes, kidney CBC with diff, ESR, chemistries (electrolytes, kidney

tests, liver tests, glucose, total protein, iron), TSHtests, liver tests, glucose, total protein, iron), TSH Further tests IF clinically indicated to exclude Further tests IF clinically indicated to exclude

alternative diagnoses suspected on the basis of initial alternative diagnoses suspected on the basis of initial testingtesting

Special immunologic testing, brain imaging, etc. are Special immunologic testing, brain imaging, etc. are not recommendednot recommended

No specific cognitive testing recommended No specific cognitive testing recommended

Treatment of CFS

Only two therapies have been shown beneficial in Only two therapies have been shown beneficial in clinical trials:clinical trials: Cognitive behavioral therapyCognitive behavioral therapy Graded exerciseGraded exercise

No known “Cure”No known “Cure” Goal: symptom management and reintegration Goal: symptom management and reintegration

into social and occupational networksinto social and occupational networks

Ineffective Measures

Immune serum globulinImmune serum globulin AcyclovirAcyclovir GalantamineGalantamine CorticosteroidsCorticosteroids AmantadineAmantadine DoxycyclineDoxycycline MagnesiumMagnesium Colonic enemasColonic enemas

Evening primrose oilEvening primrose oil Vitamin B12Vitamin B12 AmpligenAmpligen Essential Fatty acidsEssential Fatty acids Liver extractLiver extract Dialyzable leukocyte Dialyzable leukocyte

extractextract InterferonInterferon Exclusion dietsExclusion diets Removal of dental fillingsRemoval of dental fillings EtcEtc

Symptom Management

Cognitive Behavioral Therapy and Cognitive Behavioral Therapy and education about CFSeducation about CFS

Graded exercise programGraded exercise program Pharmacologic therapy for pain, Pharmacologic therapy for pain,

nonrestorative sleep, fatiguenonrestorative sleep, fatigue NSAIDs, tricyclics, SSRIs, anxiolytics, ?NSAIDs, tricyclics, SSRIs, anxiolytics, ?

stimulantsstimulants

Myths

No evidence that CFS patients lose their No evidence that CFS patients lose their fingerprintsfingerprints

No evidence for nutritional deficiency in No evidence for nutritional deficiency in CFSCFS