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CMT-dos > SLE/Fibromyalgia > Flashcards

SLE/Fibromyalgia Flashcards

1
Q

Autoimmune Disease

Pathophysiology

A

The body’s immune system attacks healthy cells; cannot distinguish between healthy and unhealthy cells; leads to an inflammatory state

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2
Q

Autoimmune Disease

Risk Factors and most common diseases

A

Environmental (smoking, diet, stress, pollution, infectious)
Genetic

Rheumatoid arthritis, Lupus, Celiac Disease, Sjogren’s syndrome, Multiple Sclerosis, Polymyalgia Rheumatica, Ankylosing Spondylitis, Diabetes Mellitus Type 1, Alopecia Areata, Vasculitis, Temporal Arteritis, Chron’s, Scleroderma, Hashimoto’s Thyroiditis

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3
Q

autoimmune disorders

challenginhg to diagnose

A

Symptoms: fatigue, malaise, activity intolerance, pain, low grade fever

Usually requires evaluation by multiple specialists of a long period of time
Goes in and out of stages flare up-active-remission
Not uncommon to have two or more autoimmune problems

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4
Q

Systemic Lupus Erythematosus (SLE)

General/presentation

A

Autoimmune disorder associated with inflammation, + ANA, multiple organ involvement

Presentation: Fever, anorexia, malaise, weight loss, malar rash, arthralgias

Work up: CBC (anemia, thrombocytopenia), CMP, UA (proteinuria), ESR, serum complement (low C3 or C4); ANA +

Diagnosis: meet 4/11 criteria “ RASHNIA4”

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5
Q

SLE

RASHNIA4

* Rash spares nasolabial folds
A

Diagnosis: meet 4/11 criteria “ RASHNIA4”
R: renal disease ( + proteinuria)
A: arthralgias
S: serositis ( pleuritis, pericarditis)
H: hematologic disorder ( anemia, thrombocytopenia)
N: neurologic disorder (seizure, psychosis)
I: immunologic abnormalities
A: antinuclear antibodies (ANA)
4 types of rashes 1- malar “butterfly” 2-discoid (chronic, scarring) 3-photosensitivity 4-oral ulcers

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6
Q

SLE

Pathophysiology

A

Multisystem inflammatory autoimmune disease
More common in females than males by 9:1, more common black women vs white
Peak age of Dx is 9-15 yrs
Susceptible individual has apoptosis event
Due to genetics, has defective clearance
Immune response leads to immune complexes

Autoantibodies against nuclear antigens
Complement activation, levels reduced
Neutrophil extracellular traps (NETs)
Release of interferon-alpha (IFN a)
Tissue and organ damage

Can be drug-induced! ( hydralazine, procainamide, INH, Quinidine)

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7
Q
A

Discoid Lupus Erythematosus

"well demarcated scars"
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8
Q
A

Subacute cutaneous lupus erythematosus

not likely to scar, usually after sun exposure
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9
Q
A
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10
Q

SLE

labs and imaging

A

Physical exam findings as mentioned

Labs:
CBC (anemia, thrombocytopenia)
CMP
UA (proteinuria)
ESR
ANA + ( ANA is highly sensitive but nonspecific- if + check antibodies)

Antibodies: antiphospholipid, anti-smith (Lupus), anti-dsDNA (nephritis), anti-histone (drug-related lupus)
serum complement (low C3 or C4)

Imaging: (?)
Cxray ( rule out effusion/pulm involvement)
Ecg/echocardiogram (rule out cardiac involvement)

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11
Q
A

+ ANA means autoantibodies are present thus your immune system is attacking itself
+ anti-DS DNA and anti-smith are specific to SLE
ANA can be positive in CREST, Sjogren’s, myositis, scleroderma

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12
Q
A

can be used if in Rheum, notice anti Smith and anti ds DNA are most specific

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13
Q

SLE

Tx

pharm and non-pharm

A

Close monitoring
Goal is to improve/maintain organ function and prevent permanent organ injury

Pharmacotherapy : (dose on disease activity)
Hydroxychloroquine (Plaquenil) 1st line
Corticosteroids for acute flare
NSAIDS- treat symptoms not dz
Iv cyclophosphamide for life-threatening cerebritis or nephritis followed by oral mycophenolate mofetil

Non-Pharmacologic:
Avoid sunlight/high sunscreen factor
Counseling support
Healthy diet and exercise

Hydroxychloroquine-annual eye exam
Methotrexate: CBC, CMP q 3 months
Glucocorticoids: FLP, CMP, DEXA annually

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14
Q

SLE complication

Lupus Nephritis
Labs, dx, tx

A

Check UA and urine micro ( + hematuria, casts, and proteinuria)

Will need kidney biopsy

Treatment: IV cyclophosphamide followed by po mycophenolate mofetil

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15
Q

SLE

Monitoring Lupus

A

Pregnancy counseling
Labs: CMC, UA, BMP, ESR ( +/- antibodies) q 6months
Annual eye exam if on HCQ
Screening for hyperlipidemia, diabetes and osteoporosis if on steroids

Current Vaccines

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16
Q

A 24-year-old woman presents to the clinic with a facial rash. She has noticed a rash on her face, triggered by sun exposure. She has also noted hair loss, malaise, and fatigue. She has a history of Hashimoto’s thyroiditis with a resultant hypothyroidism which has been stable on levothyroxine for years. Physical exam is notable for an erythematous rash which spares the nasolabial folds, diffuse thinning of the scalp hair and short hairs on the anterior hairline. Current labs reveal:
Hbg 9.6 TSH 2.0
Leukocytes 2.0
Platelets 220
Creatinine 1.5
ESR 57
ANA 1:160
Urinalysis shows 3+ protein

Which of the following antibodies is the most specific serologic test for this disease?
A. Anticentromere antibodies
B. Anti-histone antibodies
C. Anti-Jo-1 antibodies
D. Anti-Smith antibodies

A

D. Anti-Smith antibodies

17
Q

A 24-year-old woman presents to the clinic with a facial rash. She has noticed a rash on her face, triggered by sun exposure. She has also noted hair loss, malaise, and fatigue. She has a history of Hashimoto’s thyroiditis with a resultant hypothyroidism which has been stable on levothyroxine for years. Physical exam is notable for an erythematous rash which spares the nasolabial folds, diffuse thinning of the scalp hair and short hairs on the anterior hairline. Current labs reveal:
Hbg 9.6 TSH 2.0
Leukocytes 2.0
Platelets 220
Creatinine 1.5
ESR 57
ANA 1:160
Urinalysis shows 3+ protein

What is the treatment for this patient?
A. Methotrexate
B. Hydroxychloroquine
C. Mycophenolate Mofetil
D. NSAIDS

A

B. Hydroxychloroquine

18
Q

A 38-year-old woman presents to the clinic with a facial rash that develops after being in the sun. She describes the rash
As purplish-red, covering her nose and cheekbones. It is non-textured and not itchy. She has chronic fatigue but works full time and has
5 children. She has a history of hypertension and is on hydralazine. Her physical exam and vital signs at this time are unremarkable.
Initial labs include:
White blood cells 2.4
Hemoglobin 11
Platelets 314
ESR 57

Which of the following antibodies is the next best step?
A. Anti-centromere antibodies
B. Anti-histone antibodies
C. Anti-nuclear antibody
D. Anti-double-stranded DNA antibodies
E. Anti-smith antibodies

A

C. Anti-nuclear antibody
get this first then check specific antobodies.

19
Q

fibromyalgia

general

A

Chronic widespread musculoskeletal pain and focal tenderness accompanied with fatigue, and poor sleep
Insidious onset with cyclic nature

Psycho/physio/social effects- significant impairment on quality of life with large economic burden
Pathogenesis is poorly understood- thought to be an inflammatory condition

Research suggests
Disordered central nociceptive signal processing that leads to sensitization expressed as hyperalgesia and allodynia

Amplification of pain in spinal cord via spontaneous nerve activity
Lower pain thresholds
Dysregulation of hypothalamic-pituitary-adrenal axis
Functional brain imaging suggest aberrant processing may be attributed to an imbalance between excitatory and inhibitory neurotransmitters

20
Q

Fibromyalgia

Epidemiology
Triggers

A

Higher prevalence in females, possible genetic predisposition

Research suggests “trigger” for disease- usually emotional, viral, or lyme

Associated with autoimmune disease

Argued by some to be a chronic pain syndrome

Changes in the diagnostic criteria over the past decade, including the elimination of specific tender points, have resulted in more patients with chronic pain meeting the criteria for fibromyalgia

21
Q

Fibromyalgia

Presentation:

A

Chronic, diffuse, bilateral musculoskeletal pain > 3 months; tender points in 11 of 18
Normal physical exam with disproportionate pain

Patients usually report fatigue, activity intolerance, malaise, cognitive dysfunction “fibro-fog”, stiffness in am, headaches, dry mouth, paresthesia, sleep disturbances

22
Q

Fibromyalgia

Work up:

A

CBC, CMP, TSH
clinical diagnosis with multiple specialists
American Pain Society guideline recommends a complete joint examination, manual muscle strength testing, and a neurological exam
Fibromyalgia Rapid Screening Tool ( FiRST)
Consider: PHQ9, GAD 7, MDD, sleep evaluation
*discussion FM/a

Consider work up in patients with chronic pain without a history of tissue injury or inflammation more than three months in duration with fatigue, mood issues, and sleep disturbance
Multiple diagnostic criteria: tender points on physical exam eliminated in 2011 with a symptoms severity scale and self-reported widespread pain index added

23
Q

fibromyalgia

A
24
Q

Fibromyalgia

choose wisely

A

The Choosing Wisely initiative recommends against testing for Lyme disease as a cause of musculoskeletal symptoms without an exposure history and relevant examination findings

Medication adverse side effects rarely cause diffuse pain ( statins, opioids, bisphosphonates, and aromatase inhibitors)

Tests for rheumatoid factor or antinuclear antibody levels are not recommended in patients without features concerning for autoimmune disease due to high false-positive rates

25
Q

fibromyalgia

FM/a Test

A

Cytokine array-based blood test which has the potential to help confirm dx

Patients with fibromyalgia demonstrate higher cytokine production in stimulated immune cells that general population

FM/a test demonstrated 93% sensitivity and 89% specificity in 160 patients with Fm compared to patients in control

Compared with people who have RA of SLE without FM specificity of the FM/a test was 70%

Is approx. $1200, one time test- may help differentiate FM from other conditions

26
Q

Differential Diagnosis of Fibromyalgia

A
27
Q

fibromyalgia

co-morbidities

A

More than ½ patients with FM report depression

Increase likelihood of bipolar disorder, generalized anxiety, and substance abuse

Increase likelihood of restless leg syndrome

Increase reports of temporomandibular pain, irritable bowel syndrome, vulvodynia, chronic fatigue syndrome, interstitial cystitis, endometriosis, chronic tension headaches, migraine headaches, chronic lower back pain

28
Q

Fibromyalgia

TLC Tx

A

Exercise: strongest evidence for aerobic exercise of moderate intensity to improve pain, function, fatigue, and sleep quality

Diet: “clean eating”, anti-inflammatory

Complementary Treatments: acupuncture, chiropractic, biofeedback, hypnosis, meditation, thai chi, yoga, massage therapy

Others: transcutaneous electrical nerve stimulation units, thermal therapies, hyperbaric oxygen, laser and phototherapy, transdermal magnesium, vibroacoustic and rhythmic sensory stimulation, IV ketamine

29
Q

Fibromyalgia

med Treatment

A

FDA-approved Rx for treatment of FM

Duloxetine (Cymbalta)
Nerve pain and antidepressant medication
SNRI: increases serotonin and norepinephrine- responsible for regulating mood and sensitivity to pain

Milnacipran (Savella)
nerve pain and antidepressant medication (SNRI)

Pregabalin (Lyrica)
nerve pain medication (anticonvulsant) work to calm overactive nerve cells that send pain signals throughout the body, while also improving sleep and anxiety

30
Q

fibromyalgia

Treatment guidelines for fibromyalgia have been established by the American Pain Society and EULAR

goal of treatment in fibromyalgia is to reduce symptoms, improve function, and engage the patient’s involvement in self-care

more tx options

A

Nonpharmacologic
Patient education
Exercise ( aerobic, resistance training, yoga, aquatic)
Cognitive behavioral
Complimentary medicine/holistic medicine

Pharmacologic
Antidepressants ( TCAs, SSRIs, SNRIs)
Anticonvulsants
Analgesics
Sedative hypnotics
*cyclobenzaprine (Flexeril) at hs

31
Q
A

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