SLE/Fibromyalgia Flashcards
Autoimmune Disease
Pathophysiology
The body’s immune system attacks healthy cells; cannot distinguish between healthy and unhealthy cells; leads to an inflammatory state
Autoimmune Disease
Risk Factors and most common diseases
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
autoimmune disorders
challenginhg to diagnose
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
Systemic Lupus Erythematosus (SLE)
General/presentation
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”
SLE
RASHNIA4
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
SLE
Pathophysiology
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)
Discoid Lupus Erythematosus
Subacute cutaneous lupus erythematosus
SLE
labs and imaging
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)
+ 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
can be used if in Rheum, notice anti Smith and anti ds DNA are most specific
SLE
Tx
pharm and non-pharm
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
SLE complication
Lupus Nephritis
Labs, dx, tx
Check UA and urine micro ( + hematuria, casts, and proteinuria)
Will need kidney biopsy
Treatment: IV cyclophosphamide followed by po mycophenolate mofetil
SLE
Monitoring Lupus
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
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
D. Anti-Smith antibodies
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
B. Hydroxychloroquine
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
C. Anti-nuclear antibody
get this first then check specific antobodies.
fibromyalgia
general
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
Fibromyalgia
Epidemiology
Triggers
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
Fibromyalgia
Presentation:
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
Fibromyalgia
Work up:
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
fibromyalgia
Fibromyalgia
choose wisely
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
fibromyalgia
FM/a Test
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
Differential Diagnosis of Fibromyalgia
fibromyalgia
co-morbidities
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
Fibromyalgia
TLC Tx
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
Fibromyalgia
med Treatment
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
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
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
