Lupus Flashcards
Lupus Epidemiology (6)
- Incidence: 0.5-0.6 per 100,000
- Childhood onset 15-20% of cases; second most common rheumatological disease of childhood
- Mean age at onset: 12-13 years old
a. Rare < 8 years old
b. The younger you are at onset, the worse the disease - Race: Higher in Hispanic, African American, Asian, and Native American
- Sex: F»M
* 4:1 pre-pubertal, 8:1 post-pubertal - Newborns → neonatal lupus, presents with rash, usually goes away
Lupus Etiologies (7)
- Summer time – sun triggers it; must wear sunscreen all day every day
- Fluorescent bulbs
- Stress
- Infection
- Genetic + family h/o connective diseases
- Hormonal: oral contraceptives, pregnancy, teenage years
- Drugs (prolonged use of)
i. Hydralizine
ii. Sulfonamides
iii. Anticonvulsants
iv. INH
v. And others
Lupus pathophysiology (4)
- Chronic, autoimmune disease affecting multiple organ systems
- Failure of regulatory mechanisms that sustain self tolerance
- Characterized by autoantibodies, circulating immune complexes and T lymphocytes
* Anti-nuclear antibody (ANA) - Any baby that is born with heart block → mother must be tested for lupus
Lupus survival (6)
- 10 year survival: 85% - 92%
* 30 years ago: 76%
Common causes of mortality:
- Infection (10% - 80%)
- CNS disease (10% - 30%)
- Renal failure (20% - 60%)
- Cardiac/pulmonary
- Thromboembolic disease
Most common lupus manifestations (8)
- Rash
- Butterfly macular rash
- Generalized photosensitivity
- Alopecia
- Raynaud’s phenomenon
- Arthritis
- Renal disease
- Sore on roof of mouth (non-painful)
Other lupus manifestations (14)
- Joint pain/arthritis
- Fever
- Headache
- Malaise
- Rash/diffuse facial erythema
- Neuro involvement can occur – cog delays
- Glomerulonephritis with HTN and nephritic syndrome
- Pericarditis
- Endocarditis
- Lymphadenopathy
- Hepatosplenomegaly
- CNS changes: seizures, psychosis, personality changes
- Anemia
- Thrombocytopenia
Consider SLE when (3)
a. Female patient with lupus s/s
b. With prolonged fever/illness
c. With evidence of multi system involvement
Lupus labs (9)
- Elevated ESR
- Hemolytic anemia
- Low hemoglobin
- Positive ANA (in 97%)
- Low SLE
- CRP
- Rf +
- Can do false positive of syphilis; always test for lupus if you see positive syphilis
- Urinalysis
Lupus: Positive ANA (4)
- Sensitive
- NOT specific (30% of healthy children have +ANA)
- Reported as a titer and staining pattern
- Pattern of nuclear immunofluorescence suggests type of antibodies present in patients serum
More specific lupus autoantibodies (6)
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
ACR Lupus Criteria (11)
Must have 4 of 11 (*Must exclude drug/toxin induced) – one must be immunologic
- Malar rash/Butterfly rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis
- Renal disorder: Persistent proteinuria or Cellular casts
- Neurologic disorder*
- Hematologic disorder*
- 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 - Positive ANA*
A RASH POINTS TO MD
Arthritis Renal disease ANA positive Serositis Hematologic disorder Photosensitivity Oral ulcers Immunologic disorder Neurologic symptoms Malar rash Discoid rash
Comprehensive Antiphospholipid Panel (5)
- Anti-cardiolipin IgG/IgM/IgA
- Anti-β2 glycoprotein I IgG/IgM/IgA
- Lupus anticoagulant
- Associated with increased risk of:
a. Arterial/venous thrombosis
b. Miscarriage
c. Thrombocytopenia/Hemolytic anemia
d. Migraines
e. Seizures
f. Libman Sacks endocarditis - **Present on two or more occasions at least 12 weeks apart
SLE Patients General Points (3)
- Flares are mimetic – initial presentation in first few months often dictates clinical pattern
- C3, C4 and dsDNA measure disease activity
- Advances in treatment improve survival yet increase risk of infection and drug- related toxicity
Treatment-Baseline (6)
- Plaquenil- anitmalarial
- NSAIDS
- Calcium and Vit D
- Sun screen
- Treatment is based on organ involvement
* Binlista works on rashes, arthralgias, not great with kidney disease though - Avoid estrogen containing contraceptives; use IUD
Mainstay of Treatment: Immunosuppressive Oral Therapy (6)
- Corticosteroids
* Mainstay of treatment – tell patients to take it before 8am
* 1 gram once per week infusion – less systemic side effects - Mycophenolate Mofetil (Cellcept)
- Azathioprine (Imuran)
- Methotrexate – works well with arthritis
- Imuran
- Benlysta – lowers B-cell count
Mainstay of Treatment: Immunosuppressive Infusion Therapy (2)
- Cyclophosphamide
- Rituximab – B-cell inhibitor
* Puts you at risk for infections
Steroid Toxicity (10)
- Immunosuppression
- Hypertension
- Hyperglycemia
- Adrenal Suppression
- Cataracts
- Dyslipoproteinemia
- Tremors
- Myopathies
- Avascular Necrosis of bone
- Osteoporosis/Growth retardation
Lupus Skin Findings (4)
- Hair loss
- Raynaud’s
- Rashes
a. Malar rash
b. Vasculitic rash
i. Petechae
ii. Purpura - Abnormal nailbed capillaries
Lupus HEENT (2)
- Palatal ulcers (Common)
2. Retinal vasculitis (Uncommon)
Lupus Lungs Findings (4)
- Restrictive Lung Dz
- Pulmonary Hemorrhage
- Pneumonia
- Pleuritis
Lupus Cardio Findings (5)
- Endocarditis
- Pericadial effusions
- Myocardial infarction
- Hyperlipidemia
- Hypertension:
a. Renal issues
b. Medication related
Lupus Nephritis: 6 types with symptoms
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
Lupus: Joints/Muscle Pain (6)
Possible etiologies:
- Arthritis
- Myositis
- Avascular necrosis (secondary to SLE vs. steroids)
- Septic Arthritis*
Red flags:
- Fever
- Refusal to bear weight
Lupus arthritis (3)
- Non-erosive
- Often in small joints of hands but can be anywhere
- Jaccoud’s arthropathy
a. Deforming
b. Due to periarticular fibrosis, ligament laxity and joint subluxation
SLE and joint pain work-up (3) and treatment (4)
Work-up:
- Nothing
- Xrays
- CBC, ESR, CRP, C3, C4 dsDNA
Treatment notes:
- Naprosyn (10-15 mg/kg/day divided BID)
- GI prophylaxis*
- Rarely require opiods (AVN)
- Shouldn’t need morphine
Lupus possible neurological manifestations (5)
- Lupus cerebritis
- Cognitive dysfunction
- Migraines
- Depression
- Psychosis
SLE and chest pain: possible etiologies (8)
Cardiovascular
- Pericarditis
- tamponade
- myocardial infarction
Pulmonary
- Pleuritis
- Infection
- Alveolar hemorrhage
- pulmonary embolism
- Costochondritis
SLE and chest pain: red flags (5)
- Abnormal vital signs
- Muffled heart sounds
- Difficulty lying flat/orthopnea
- Hemoptysis
- History of positive aPL antibodies (or nephrotic syndrome)
SLE and chest pain: work-up (7)
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
SLE and chest pain: treatment notes (2)
- Serositis: NSAIDS/steroids
2. Alveolar hemorrhage: plasmapheresis
SLE and headaches: etiology (7)
- Lupus cerebritis/vasculitis
- Thrombosis: CVA/Venous sinus thrombosis
- Hypertensive emergency
- Pseudotumoral cerebri
- Migraines
- Meningitis
- Depression
SLE and headaches: red flags (4)
a. Severe, unremitting
b. Abnormal vital signs
c. Abnormal neurologic exam
d. Papilledema
Lupus Cerebritis (10)
- Headaches
- Decreased concentration
- Poor school performance
- Psychosis
- Seizures
- Stroke
- Chorea
- Vertigo
- Transverse myelitis
- Peripheral neuropathy
SLE and Headaches: Work-up (4)
- CBC, Chem, LFTs, C3, C4, dsDNA, urinalysis
- Anti-ribosomal P antibodies
- Imagining – MRI/MRA/MRV is ideal
- Lumbar puncture – include oligoclonal bands and anti-neuronal antibodies
* Elevated WBC/protein with cerebritis
SLE and headaches: treatment notes (2)
- If patient well appearing, start by treating as Migraine
2. If no improvement in headache, further work-up required
SLE and Fever/Infection (4)
- Immunosuppression (PO, SC or IV)
* Ask about infusions (cyclophosphamide, rituximab) - Functionally asplenic
* S. pneumococcus, N. meningitidis - Always on differential because medications may mask signs: fever, peritoneal signs
- Never stop prednisone!
* May hold other immunosuppressive agents
SLE and infection: well-appearing patient (3)
a. Likely viral
b. CBC, BCx
c. No change in medications
SLE and infection: Mild bacterial infection (4)
- Uncomplicated UTI, Strep throat, mild pneumonia
- Antibiotics
- Hold immunosuppression
- Continue current prednisone
SLE and infection: severe infection/sepsis (6)
- Antibiotics
- Supportive care
- Hold immunosuppression
* Can leave on hydroxychloroquine (plaquenil) - Don’t stop prednisone!
- Stress dose steroids
- Prevent adrenal crisis
Adrenal Crisis (6)
- Hypotension/shock, especially if disproportionate to underlying illness
- Abdominal pain, vomiting, diarrhea
Electrolyte abnormalities
- Hyponatremia
- Hyperkalemia
- Metabolic acidosis
- Hypoglycemia
Stress dosing of corticosteroids (3)
- Physiologic maintenance: Prednisone: 0.1 mg/kg/day, ~ 5-10 mg daily
- Extreme stress (serious infection, trauma, surgery):
Prednisone: 0.5 – 1 mg/kg/day - Hydrocortisone 100mg = Prednisone 25 mg
SLE and Cytopenias: Etiologies (5)
a. Anemia of chronic disease
b. “Idiopathic” thrombocytopenic purpura
c. Hemolytic anemia (Coombs +)
d. TTP/HUS
e. Macrophage activation syndrome
SLE and Cytopenias: Red flags (3)
a. Abnormal vital signs
b. Evidence of bruising or bleeding
c. Abnormal coagulation tests
Macrophage Activation Syndrome (9)
- Similar to HLH
- SoJIA, SLE
- Potentially life threatening
- Fever, LAD, HSM
- Consumptive coagulopathy (~DIC)
- Severe cytopenias (*plts)
- Extreme hyperferritinemia (>10,000)
- High CRP with Low ESR
- Overwhelming systemic inflammatory response
Macrophage Activation Syndrome: Work-Up (4)
- CBC, Chem, LFTs, Urinalysis, ESR, CRP
- Hemolysis labs: retic, haptoglobin, LDH
- Coags: PT/INR, PTT, fibrinogen, D-dimer
- MAS: ferritin, triglycerides
Macrophage Activation Syndrome: Treatment notes (3)
- Check baseline labs
- TTP: requires plasmapheresis
- MAS: high dose steroids and cyclosporine
Lupus Take Home Points (3)
- Always ask about immunosuppression
* Both oral and infusions - Always consider infection/thromboembolic event in differential
- Never stop prednisone
* Adrenal crisis
Lupus: Major Jones Criteria (5)
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
