Lupus

Question 1

Epidemiology

At least 1.5 million Americans and
* 5 million people worldwide have a form of lupus

Gender:
* 9 in 10 people with lupus are women
* More common in ___ of
childbearing age

Age:
* Peak incidence between ages of 15-45
years old

Ethnicity:
* More prevalent in Asian, African, Arab,
Hispanic/Latins and American Indians
background

Answer 1

women

Question 2

Exact etiology of SLE is unknown but many pre-disposing factors

Answer 2

Question 3

Drug-induced Lupus Erythematosus (DILE)

• Overreaction to certain medications
• Symptoms occur __ - __ months of
  drug initiation
• Resolution occurs within ___ of drug discontinuation
• Around 10% of SLE cases are drug-
  induced
• There are at least 46 medications that
  can cause DILE

Answer 3

• 3-6
• weeks

Question 4

MPMMPHATIQ

Answer 4

• Methimazole
• Propylthiouracil
• Methyldopa
• Minocycline
• Procainamide
• Hydralazine (alone, and in BiDil)
• Anti-TNF agents (ex. Infliximab and
  etanercept)
• Terbinafine
• Isoniazid
• Quinidine

Question 5

Answer 5

D) minocycline

Question 6

Signs/Symptoms

• Fatigue
• Depression
• Photosensitivity
• Joint Pain
• Nausea/Vomiting
• Fever
• Weight Loss
• Malar “_______” rash

Answer 6

butterfly

Question 7

Answer 7

• butterfly rash, discoid rash, raynaud phenomenon
• lupus retinopathy
• lupus nephritis

Question 8

Answer 8

a) butterfly
c) sun exposure

Question 9

Key labs

all labs positive in lupus
- ANA - not ___
- anti-dsDNA - ___ specificity, correlates with disease activity, important marker of lupus ___
- anti-SM - ___ specificity
- Antiphospholipid Antibody - Increase clotting factors

Answer 9

• specific
• high, nephritis
• high

Question 10

Answer 10

• rash
• mouth ulcers
• ANA +
• anti-dsDNA +

Question 11

Pharmacologic Treatments

Answer 11

• HCQ
• NSAIDs
• glucocorticoids
• immunosuppressants
• biologics

Question 12

HCQ

MOA: Antimalarial —> Inhibit overactive immune cells
* Place in therapy: Recommended for ALL patients with SLE
* Benefits: Reduce flares and help manage pain
* Dosing: 200-400 mg PO daily
* Max: 400 mg daily

Question 13

HCQ SE

Side effects:
- Ophthalmologic: Retinal Toxicity ( ____ )
- CNS: Neuropsychiatric Effects (anxiety,
depression, insomnia, suicidal ideation,
etc)
- CV: QT prolongation, cardiomyopathy
- Neuromuscular: Myopathy
- Dermatologic: Hypersensitivity reaction
- Other: Hypoglycemia, Hemolytic anemia
( ____ )

Answer 13

• bullseye mucopathy
• G6PD

Question 14

G6PD Deficiency

• ___ disorder that causes RBCs to prematurely break down

Answer 14

X-linked

Question 15

HCQ - monitoring

Baseline:
* CBC
* LFTs
* SCr
* EKG

Periodic:
* ___ exam
* 3 months after starting therapy,
then annually

Answer 15

• eye

Question 16

NSAIDs

Place in therapy: Considered first line for mild symptoms

Dosing:
* Ibuprofen: 400-600 mg PO Q6-8H
* Naproxen: 500 mg PO BID

Side effects
- GI: Bleeding, gastritis, perforation
- CV: Increased BP, worsening HF, cardiovascular events
- Renal: Increased SCr, renal toxicity
- Hepatic: Hepatoxicity

Monitoring Parameters:
* Baseline:
* CBC
* LFTs
* SCr
* BP

S/sx of ___and bleeding

Answer 16

• fluid retention

Question 17

Glucocorticoids

MOA: Inhibit B and T cell responses

Benefits: Anti-inflammatory and helpful
during flares
- Variable dosage forms - PO, topical, IV
- Place in therapy: ___ treatment, if not responsive to NSAIDs/ antimalarial

Answer 17

• adjunctive

Question 18

GC

PO/IV Side Effects
* Ophthalmologic: Glaucoma
* CV: ___ BP
* Bone: Increased risk of ___
* GI: GI bleed, gastritis
* CNS: Psychosis/sleep disturbances
* Other: Weight gain, increased ___ ,
increased risk of infection risk &
___ syndrome

Topical SE
- skin atrophy
- rosacea
- telangiectasis

Answer 18

• increased
• osteoporosis
• BG
• Cushing

Question 19

Glucocorticoids (GC)

Monitoring parameters:

Baseline:
* BP
* BMP
* FLP
* Bone mineral density

Routine:
* ___ - Every 6 months
* ___- Every 6 months
* ___ - Annually

Answer 19

• BMP
• FLP
• Bone mineral density

Question 20

Immunosuppressants (IS)

MOA: Suppression of immune function
from attacking healthy cells

Place in therapy:
- Adjunct to ___ therapy to lower
the dose
- Insufficient response to ___

Medications:
* Methotrexate (MTX)
* Azathioprine (AZA)
* Cyclophosphamide (CYC)
* Mycophenolate mofetil (MMF)

Answer 20

• steroid
• HCQ

Question 21

Dosages and Side Effects

Answer 21

• weekly
• BID

Question 22

Dosages and Side Effects

cyclophosphamide (CYC)
IV: ___ mg/m2 BSA q month x 6 months

Answer 22

• 0.5

Question 23

AZA: monitor ___

Answer 23

TPMT

Question 24

Biologics

MOA: Monoclonal antibodies that block __ -cell mediated immunity

Place in therapy:
* Inadequate response to ___
and ___
* Severe disease

Medications:
* ___ (Benlysta®)
* ___ (Tiruxan ®)
* ___ (Saphnelo®)

Answer 24

• B-cell
• HCQ, immunosuppressants
• belimumab
• rituximab
• anifrolumab

Question 25

# biologics Pearls: - No live vaccines ___ days before starting therapy OR ___ therapy - Do not use more than 1 biologic at the same time

Answer 25

- 30 - during

Question 26

# biologic BEL SE - ___ - infusion reactions anifrolumab - ___ rituximab SE - Infusion reactions - ___ reactivation - premedication ~ 30 min prior

Answer 26

Question 27

# Additional therapies ___ Inhibitors (CNI)

Answer 27

Calcineurin

Question 28

# Cutaneous Lupus - 10% of lupus cases and rarely life-threatening - Presents on the skin with rash and lesions First Line: Topical agents: - GC: Clobetasol, betamethasone, triamcinolone, hydrocortisone - CNI: Tacrolimus, pimecrolimus not topical - ___ - Systemic GC Refractory: * High-dose GC * ___ or ___

Answer 28

- HCQ - MTX, MMF

Question 29

# Lupus Nephritis LN is a serious complication of SLE which can affect 60% of patients within 10 years of diagnosis Therapy: Mild/moderate nephritis (Class 1 or 2 LN): - GC+/- another immunosuppressant ( ___ , ___ , or ___ ) Severe nephritis (Class 3 or 4 LN): - ___ (preferred) or CYC +/- GC Triple therapy: * ___ + ___ or CYC +/- ___ * ___ + ___ +/- GC (triple therapy)

Answer 29

- AZA, MMF, or CNI - MF - MF - belimumab, MMF, GC - CNI, MMF

Question 30

# LN flow chart

Answer 30

Question 31

# MSC

Answer 31

- diuretics - NSAIDs (unless nephritis)

Question 32

# Pregnancy & SLE Higher maternal and fetal risk compared with healthy women * Miscarriage * Fetal growth retardation * Maternal mortality * Preeclampsia The best prognosis is when the patient achieves remission for ≥ ___ months before pregnancy * Pregnancy can cause flares Contraception * Avoid ___ -containing contraception * Screen for ___ syndrome

Answer 32

- 6 - estrogen - antiphospholipid

Question 33

# Antiphospholipid antibody An autoimmune disorder characterized by ___ that can cause blood clots and miscarriages

Answer 33

- antiphospholipid syndrome

Question 34

# summary - SLE primarily occurs in women of childbearing age - SLE is a multi-system autoimmune disease that can involve any organ and present in many ways - Many drugs can induce lupus-like symptoms - Backbone of lupus therapy: ___ +/- ___ +/- ___ for pain and inflammation - Monitor for drug side effects/toxicities

Answer 34

- HCQ, GC, NSAIDs