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PPC3 Exam 2 > Lupus > Flashcards

Lupus Flashcards

1
Q

Lupus Epidemiology (6)

A
  1. Incidence: 0.5-0.6 per 100,000
  2. Childhood onset 15-20% of cases; second most common rheumatological disease of childhood
  3. Mean age at onset: 12-13 years old
    a. Rare < 8 years old
    b. The younger you are at onset, the worse the disease
  4. Race: Higher in Hispanic, African American, Asian, and Native American
  5. Sex: F»M
    * 4:1 pre-pubertal, 8:1 post-pubertal
  6. Newborns → neonatal lupus, presents with rash, usually goes away
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2
Q

Lupus Etiologies (7)

A
  1. Summer time – sun triggers it; must wear sunscreen all day every day
  2. Fluorescent bulbs
  3. Stress
  4. Infection
  5. Genetic + family h/o connective diseases
  6. Hormonal: oral contraceptives, pregnancy, teenage years
  7. Drugs (prolonged use of)
    i. Hydralizine
    ii. Sulfonamides
    iii. Anticonvulsants
    iv. INH
    v. And others
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3
Q

Lupus pathophysiology (4)

A
  1. Chronic, autoimmune disease affecting multiple organ systems
  2. Failure of regulatory mechanisms that sustain self tolerance
  3. Characterized by autoantibodies, circulating immune complexes and T lymphocytes
    * Anti-nuclear antibody (ANA)
  4. Any baby that is born with heart block → mother must be tested for lupus
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4
Q

Lupus survival (6)

A
  1. 10 year survival: 85% - 92%
    * 30 years ago: 76%

Common causes of mortality:

  1. Infection (10% - 80%)
  2. CNS disease (10% - 30%)
  3. Renal failure (20% - 60%)
  4. Cardiac/pulmonary
  5. Thromboembolic disease
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5
Q

Most common lupus manifestations (8)

A
  1. Rash
  2. Butterfly macular rash
  3. Generalized photosensitivity
  4. Alopecia
  5. Raynaud’s phenomenon
  6. Arthritis
  7. Renal disease
  8. Sore on roof of mouth (non-painful)
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6
Q

Other lupus manifestations (14)

A
  1. Joint pain/arthritis
  2. Fever
  3. Headache
  4. Malaise
  5. Rash/diffuse facial erythema
  6. Neuro involvement can occur – cog delays
  7. Glomerulonephritis with HTN and nephritic syndrome
  8. Pericarditis
  9. Endocarditis
  10. Lymphadenopathy
  11. Hepatosplenomegaly
  12. CNS changes: seizures, psychosis, personality changes
  13. Anemia
  14. Thrombocytopenia
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7
Q

Consider SLE when (3)

A

a. Female patient with lupus s/s
b. With prolonged fever/illness
c. With evidence of multi system involvement

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8
Q

Lupus labs (9)

A
  1. Elevated ESR
  2. Hemolytic anemia
  3. Low hemoglobin
  4. Positive ANA (in 97%)
  5. Low SLE
  6. CRP
  7. Rf +
  8. Can do false positive of syphilis; always test for lupus if you see positive syphilis
  9. Urinalysis
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9
Q

Lupus: Positive ANA (4)

A
  1. Sensitive
  2. NOT specific (30% of healthy children have +ANA)
  3. Reported as a titer and staining pattern
  4. Pattern of nuclear immunofluorescence suggests type of antibodies present in patients serum
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10
Q

More specific lupus autoantibodies (6)

A

a. Anti-dsDNA*
b. Anti –Smith*
c. Anti-RNP
d. Anti-Ro (SSA)
e. Anti-La (SSB)
f. Antibodies to dsDNA and Sm antigen are virtually diagnostic of SLE

*If double stranded DNA is positive, you most likely have lupus

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11
Q

ACR Lupus Criteria (11)

A

Must have 4 of 11 (*Must exclude drug/toxin induced) – one must be immunologic

  1. Malar rash/Butterfly rash
  2. Discoid rash
  3. Photosensitivity
  4. Oral ulcers
  5. Arthritis
  6. Serositis
  7. Renal disorder: Persistent proteinuria or Cellular casts
  8. Neurologic disorder*
  9. Hematologic disorder*
  10. Positive immunoserology (immunologic)
    a. anti-dsDNA (double-stranded DNA)
    b. anti-Smith
    c. Lupus anticoagulant
    d. CL antibodies
    e. False positive RPR
    f. aPL antibodies (antiphospholipid antibodies) – may be positive
  11. Positive ANA*
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12
Q

A RASH POINTS TO MD

A 
Arthritis
Renal disease
ANA positive
Serositis
Hematologic disorder
Photosensitivity
Oral ulcers
Immunologic disorder
Neurologic symptoms
Malar rash    
Discoid rash
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13
Q

Comprehensive Antiphospholipid Panel (5)

A
  1. Anti-cardiolipin IgG/IgM/IgA
  2. Anti-β2 glycoprotein I IgG/IgM/IgA
  3. Lupus anticoagulant
  4. Associated with increased risk of:
    a. Arterial/venous thrombosis
    b. Miscarriage
    c. Thrombocytopenia/Hemolytic anemia
    d. Migraines
    e. Seizures
    f. Libman Sacks endocarditis
  5. **Present on two or more occasions at least 12 weeks apart
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14
Q

SLE Patients General Points (3)

A
  1. Flares are mimetic – initial presentation in first few months often dictates clinical pattern
  2. C3, C4 and dsDNA measure disease activity
  3. Advances in treatment improve survival yet increase risk of infection and drug- related toxicity
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15
Q

Treatment-Baseline (6)

A
  1. Plaquenil- anitmalarial
  2. NSAIDS
  3. Calcium and Vit D
  4. Sun screen
  5. Treatment is based on organ involvement
    * Binlista works on rashes, arthralgias, not great with kidney disease though
  6. Avoid estrogen containing contraceptives; use IUD
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16
Q

Mainstay of Treatment: Immunosuppressive Oral Therapy (6)

A
  1. Corticosteroids
    * Mainstay of treatment – tell patients to take it before 8am
    * 1 gram once per week infusion – less systemic side effects
  2. Mycophenolate Mofetil (Cellcept)
  3. Azathioprine (Imuran)
  4. Methotrexate – works well with arthritis
  5. Imuran
  6. Benlysta – lowers B-cell count
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17
Q

Mainstay of Treatment: Immunosuppressive Infusion Therapy (2)

A
  1. Cyclophosphamide
  2. Rituximab – B-cell inhibitor
    * Puts you at risk for infections
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18
Q

Steroid Toxicity (10)

A
  1. Immunosuppression
  2. Hypertension
  3. Hyperglycemia
  4. Adrenal Suppression
  5. Cataracts
  6. Dyslipoproteinemia
  7. Tremors
  8. Myopathies
  9. Avascular Necrosis of bone
  10. Osteoporosis/Growth retardation
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19
Q

Lupus Skin Findings (4)

A
  1. Hair loss
  2. Raynaud’s
  3. Rashes
    a. Malar rash
    b. Vasculitic rash
    i. Petechae
    ii. Purpura
  4. Abnormal nailbed capillaries
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20
Q

Lupus HEENT (2)

A
  1. Palatal ulcers (Common)

2. Retinal vasculitis (Uncommon)

21
Q

Lupus Lungs Findings (4)

A
  1. Restrictive Lung Dz
  2. Pulmonary Hemorrhage
  3. Pneumonia
  4. Pleuritis
22
Q

Lupus Cardio Findings (5)

A
  1. Endocarditis
  2. Pericadial effusions
  3. Myocardial infarction
  4. Hyperlipidemia
  5. Hypertension:
    a. Renal issues
    b. Medication related
23
Q

Lupus Nephritis: 6 types with symptoms

A

Type I: Minimal mesangial; presents with mild proteinuria

Type II: Mesangial proliferative; presents with asymptomatic hematuria/proteinuria

Type III: Focal segmental glomerulonephritis (active or chronic); presents with active lupus, moderate proteinuria

Type IV: Diffuse proliferative glomuerlonephritis (active or chronic; global or segmental); presents with nephrotic syndrome, renal insufficiency and HTN

Type V: Membranous; presents with nephrotic syndrome

Type VI: Advanced glomerulosclerosis; presents with renal insufficiency or ESRD

24
Q

Lupus: Joints/Muscle Pain (6)

A

Possible etiologies:

  1. Arthritis
  2. Myositis
  3. Avascular necrosis (secondary to SLE vs. steroids)
  4. Septic Arthritis*

Red flags:

  1. Fever
  2. Refusal to bear weight
25
Q

Lupus arthritis (3)

A
  1. Non-erosive
  2. Often in small joints of hands but can be anywhere
  3. Jaccoud’s arthropathy
    a. Deforming
    b. Due to periarticular fibrosis, ligament laxity and joint subluxation
26
Q

SLE and joint pain work-up (3) and treatment (4)

A

Work-up:

  1. Nothing
  2. Xrays
  3. CBC, ESR, CRP, C3, C4 dsDNA

Treatment notes:

  1. Naprosyn (10-15 mg/kg/day divided BID)
  2. GI prophylaxis*
  3. Rarely require opiods (AVN)
  4. Shouldn’t need morphine
27
Q

Lupus possible neurological manifestations (5)

A
  1. Lupus cerebritis
  2. Cognitive dysfunction
  3. Migraines
  4. Depression
  5. Psychosis
28
Q

SLE and chest pain: possible etiologies (8)

A

Cardiovascular

  1. Pericarditis
  2. tamponade
  3. myocardial infarction

Pulmonary

  1. Pleuritis
  2. Infection
  3. Alveolar hemorrhage
  4. pulmonary embolism
  5. Costochondritis
29
Q

SLE and chest pain: red flags (5)

A
  1. Abnormal vital signs
  2. Muffled heart sounds
  3. Difficulty lying flat/orthopnea
  4. Hemoptysis
  5. History of positive aPL antibodies (or nephrotic syndrome)
30
Q

SLE and chest pain: work-up (7)

A

a. EKG
b. Chest X-ray
c. CT scan, VQ scan (clinical suspicion)
d. CBC, Chem, LFTs (albumin), urine protein,
e. C3, C4, dsDNA
f. Cardiac enzymes

31
Q

SLE and chest pain: treatment notes (2)

A
  1. Serositis: NSAIDS/steroids

2. Alveolar hemorrhage: plasmapheresis

32
Q

SLE and headaches: etiology (7)

A
  1. Lupus cerebritis/vasculitis
  2. Thrombosis: CVA/Venous sinus thrombosis
  3. Hypertensive emergency
  4. Pseudotumoral cerebri
  5. Migraines
  6. Meningitis
  7. Depression
33
Q

SLE and headaches: red flags (4)

A

a. Severe, unremitting
b. Abnormal vital signs
c. Abnormal neurologic exam
d. Papilledema

34
Q

Lupus Cerebritis (10)

A
  1. Headaches
  2. Decreased concentration
  3. Poor school performance
  4. Psychosis
  5. Seizures
  6. Stroke
  7. Chorea
  8. Vertigo
  9. Transverse myelitis
  10. Peripheral neuropathy
35
Q

SLE and Headaches: Work-up (4)

A
  1. CBC, Chem, LFTs, C3, C4, dsDNA, urinalysis
  2. Anti-ribosomal P antibodies
  3. Imagining – MRI/MRA/MRV is ideal
  4. Lumbar puncture – include oligoclonal bands and anti-neuronal antibodies
    * Elevated WBC/protein with cerebritis
36
Q

SLE and headaches: treatment notes (2)

A
  1. If patient well appearing, start by treating as Migraine

2. If no improvement in headache, further work-up required

37
Q

SLE and Fever/Infection (4)

A
  1. Immunosuppression (PO, SC or IV)
    * Ask about infusions (cyclophosphamide, rituximab)
  2. Functionally asplenic
    * S. pneumococcus, N. meningitidis
  3. Always on differential because medications may mask signs: fever, peritoneal signs
  4. Never stop prednisone!
    * May hold other immunosuppressive agents
38
Q

SLE and infection: well-appearing patient (3)

A

a. Likely viral
b. CBC, BCx
c. No change in medications

39
Q

SLE and infection: Mild bacterial infection (4)

A
  1. Uncomplicated UTI, Strep throat, mild pneumonia
  2. Antibiotics
  3. Hold immunosuppression
  4. Continue current prednisone
40
Q

SLE and infection: severe infection/sepsis (6)

A
  1. Antibiotics
  2. Supportive care
  3. Hold immunosuppression
    * Can leave on hydroxychloroquine (plaquenil)
  4. Don’t stop prednisone!
  5. Stress dose steroids
  6. Prevent adrenal crisis
41
Q

Adrenal Crisis (6)

A
  1. Hypotension/shock, especially if disproportionate to underlying illness
  2. Abdominal pain, vomiting, diarrhea

Electrolyte abnormalities

  1. Hyponatremia
  2. Hyperkalemia
  3. Metabolic acidosis
  4. Hypoglycemia
42
Q

Stress dosing of corticosteroids (3)

A
  1. Physiologic maintenance: Prednisone: 0.1 mg/kg/day, ~ 5-10 mg daily
  2. Extreme stress (serious infection, trauma, surgery):
    Prednisone: 0.5 – 1 mg/kg/day
  3. Hydrocortisone 100mg = Prednisone 25 mg
43
Q

SLE and Cytopenias: Etiologies (5)

A

a. Anemia of chronic disease
b. “Idiopathic” thrombocytopenic purpura
c. Hemolytic anemia (Coombs +)
d. TTP/HUS
e. Macrophage activation syndrome

44
Q

SLE and Cytopenias: Red flags (3)

A

a. Abnormal vital signs
b. Evidence of bruising or bleeding
c. Abnormal coagulation tests

45
Q

Macrophage Activation Syndrome (9)

A
  1. Similar to HLH
  2. SoJIA, SLE
  3. Potentially life threatening
  4. Fever, LAD, HSM
  5. Consumptive coagulopathy (~DIC)
  6. Severe cytopenias (*plts)
  7. Extreme hyperferritinemia (>10,000)
  8. High CRP with Low ESR
  9. Overwhelming systemic inflammatory response
46
Q

Macrophage Activation Syndrome: Work-Up (4)

A
  1. CBC, Chem, LFTs, Urinalysis, ESR, CRP
  2. Hemolysis labs: retic, haptoglobin, LDH
  3. Coags: PT/INR, PTT, fibrinogen, D-dimer
  4. MAS: ferritin, triglycerides
47
Q

Macrophage Activation Syndrome: Treatment notes (3)

A
  1. Check baseline labs
  2. TTP: requires plasmapheresis
  3. MAS: high dose steroids and cyclosporine
48
Q

Lupus Take Home Points (3)

A
  1. Always ask about immunosuppression
    * Both oral and infusions
  2. Always consider infection/thromboembolic event in differential
  3. Never stop prednisone
    * Adrenal crisis
49
Q

Lupus: Major Jones Criteria (5)

A

J →joints; polyarthritis

<3 → carditis; heart murmur, friction rub, pericarditis, cardiomegaly, CHF

N → Subcutaneous nodules; an extensor surfaces of hands, feet, scalp, vertebra

E → erythema marginatum

S → Sydenham’s chorea; sudden abnormal movements

PPC3 Exam 2 (11 decks)

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