Therapeutics Exam 4 Lupus

Question 1

What are the 3 buckets of lupus etiology?

Answer 1

Genetics
Hormonal (estrogen)
Environmental

*exact etiology is unknown

Question 2

What drugs cause Drug-Induced Lupus Erythematous?

Answer 2

Methimazole
Propylthiouracil
Methyldopa
Minocycline
Procainamide
Hydralazine
Anti-TNF agents
Terbinafine
Isoniazid
Quinidine

(My Pretty Malar Marking Probably Has A TransIent Quality)

Question 3

What are the symptoms of lupus?

Answer 3

Fatigue
Depression
Photosensitivity*
Joint Pain
N/V
Fever
Weight Loss
Malar “Butterfly” Rash*

Question 4

What is a classic skin manifestation of lupus?
A. butterfly-shaped rash on the face
B. blisters on the hand
C. scaling on the scalp
D. darkening around the eyes

Answer 4

A. butterfly-shaped rash on the face

Question 5

Which of the following is a common trigger for lupus flare-ups?
A. regular exercise
B. high sodium intake
C. sun exposure
D. high cholesterol

Answer 5

C. sun exposure

Question 6

What are the 2 diagnostic tools for lupus?

Answer 6

SLICC (Systemic Lupus International Collaborating Clinics)

EULAR/ACR (European League Against Rheumatism/ American College of Rheumatology 2019)

Question 7

Using the SLICC guidelines, what is required for a patient to be diagnosed with lupus?

Answer 7

They meet >/= 4 total features with 1 from each group

OR

Biopsy-proven Lupus nephritis WITH systemic lupus
(+ anti-dsDNA antibodies or + ANA)

Question 8

Using the EULAR/ACR guidelines, what is required for a patient to be diagnosed with lupus?

Answer 8

*Only count the highest-weighted criterion score within a single domain

Lupus when: Patient’s score is >/= 10 AND at least 1 clinical criterion is fulfilled

Question 9

What are the 4 laboratory tests done to determine a diagnosis of lupus?

Answer 9

Anti-Nuclear Antibody (ANA)

Anti-Double-Stranded DNA (anti-dsDNA)

Anti-Smith Antibody (Anti-SM)

Antiphospholipid Antibody

**all positive in lupus and negative when healthy

Question 10

What is the reference range of Anti-Nuclear Antibody (ANA)?

Answer 10

> 1:40 = positive

*note that this is not specific

Question 11

What does the Anti-Double-Stranded DNA lab value tell us?

Answer 11

-High specificity

-Correlates with disease activity

-Important marker in lupus nephritis

Question 12

What does the Anti-Smith Antibody (Anti-SM) lab value tell us?

Answer 12

-High specificity

-Binds to SM proteins that are attached to DNA

-Found in smaller % of patients

Question 13

What does the Antiphospholipid Antibody lab value tell us?

Answer 13

There are increased clotting factors and a higher clot risk when this is positive

Question 14

What are the 5 drugs/classes that a patient with lupus will be on?

Answer 14

Hydroxychloroquine
NSAIDs
Glucocorticoids
Immunosuppressants
Biologics

Question 15

What is the moa of hydroxychloroquine?

Answer 15

Antimalarial
-inhibits overactive immune cells

-reduces flares and helps manage pain

Question 16

Who should receive hydroxychloroquine?

Answer 16

All patients with lupus

1st-line

Question 17

What is the dosing of hydroxychloroquine?

Answer 17

200-400mg po daily

MAX: 400 mg daily

Question 18

What are the side effects of hydroxychloroquine?

Answer 18

Retinal toxicity (bulls eye maculopathy)

Psychiatric events

QTc prolongation + cardiomyopathy

Hypersensitivity

Hypoglycemia

**Hemolytic anemia (G6PD deficiency)

Question 19

How does a G6PD deficiency effect patients receiving hydroxychloroquine?

Answer 19

This is an x-linked disorder that causes RBCs to break down prematurely

-Increases risk of anemia with hydroxychloroquine

-Do not give this drug to pts with this mutation

Question 20

What is the role of NSAIDs in lupus?

Answer 20

1st-line for mild symptoms

-Antipyretic
-Anti-inflammatory
-Analgesic

Question 21

What is the dosing of NSAIDs used for lupus?

Answer 21

Ibuprofen: 400-600 mg po q6-8h

Naproxen: 500 mg po bid

Question 22

What is the role of glucocorticoids in lupus patients?

Answer 22

Inhibit B and T cell responses

-Anti-inflammatory, helpful during flares

*Adjunct treatment, if not responsive to NSAIDs/hydroxychloroquine

Question 23

What are the glucocorticoids that we can use for lupus?

Answer 23

Prednisone
Methylprednisolone
Topical

Question 24

What is the dosing of the glucocorticoids?

Answer 24

Mild/Mod Prednisone: 5-30 mg/day po

Severe Prednisone: 1mg/kg/day po

IV methylprednisolone: 500-1000 mg IV daily x 3-6 days, then po prednisone

Question 25

What are the topical glucocorticoids that can be used?

Answer 25

Low-potency (face): Fluocinolone valerate + Hydrocortisone butyrate Moderate (trunk + extremities): Triamcinolone acetonide + Betamethasone valerate High potency (scalp sores + palms): Clobetasol

Question 26

What is the role of immunosuppressants in lupus therapy?

Answer 26

Adjunct to steroid therapy to lower steroid dosing Insufficient response to hydroxychloroquine

Question 27

What are the immunosuppressants used for lupus?

Answer 27

Methotrexate Azathioprine Cyclophosphamide Mycophenolate mofetil

Question 28

What is the dosing of methotrexate?

Answer 28

5-15mg WEEKLY

Question 29

What are the side effects of methotrexate?

Answer 29

Bone marrow suppression Infection **this is the only immunosuppressant that does not cause malignancy

Question 30

What is the dosing of mycophenolate?

Answer 30

1-1.5mg BID

Question 31

What are the side effects if mycophenolate?

Answer 31

Bone marrow suppression Infection malignancy AIS

Question 32

What is the dosing of cyclophosphamide

Answer 32

1-1.5 mg/kg once daily IV: 0.5 mg/m2 BSA q month x 6 months **this is the only drug in this class with IV dosing

Question 33

What is the dosing of Azathioprine?

Answer 33

50 mg daily

Question 34

What are the side effects of cyclophosphamide and azathioprine?

Answer 34

Bone marrow suppression Infection Malignancy

Question 35

What deficiency needs to be monitored with azathioprine?

Answer 35

TPMT deficiency

Question 36

How does TPMT deficiency affect azathioprine?

Answer 36

Azathioprine is a prodrug metabolized to 6-MP 6-MP can go down two pathways, one where it becomes active metabolites and one where it becomes inactive TPMT is responsible for producing inactive metabolites from 6-MP When a patient is TPMT deficient, all of the 6-MP is activated making the activity of the drug be way too high -toxic bone marrow suppression ***CANNOT GIVE AZATHIOPRINE TO THESE PATIENTS***

Question 37

What is the role of biologics in lupus therapy?

Answer 37

Used when inadequate response to hydroxychloroquine and immunosuppressants

Question 38

What are the biologics used in lupus?

Answer 38

Belimumab Rituximab Anifrolumab

Question 39

What are the important points to remember with biologics?

Answer 39

No live vaccines 30 days before starting therapy or during therapy Do not use more than 1 biologic at a time

Question 40

What is the dosing of Belimumab?

Answer 40

10mg/kg every 2 weeks -give 3 doses

Question 41

What is the dosing of Anifrolumab?

Answer 41

300 mg every 4 weeks

Question 42

When giving rituximab, what is an important point to remember?

Answer 42

Patients must be pre-medicated for infusion reactions 30 minutes before the IV infusion

Question 43

What must patients be screened for before starting rituximab?

Answer 43

Hepatitis B

Question 44

For cutaneous lupus what are our 1st line treatment options?

Answer 44

Topical Glucocorticoid Agents: Clobetasol Betamethasone Triamcinolone Hydrocortisone

Question 45

What is the treatment for lupus nephritis?

Answer 45

Mild/Mod: Glucocorticoid +/- Immunosuppressant Severe: *Mycophenolate mofetil* is the preferred immunosuppressant Consider triple therapy

Question 46

What drug should not be given to patients with nephritis?

Answer 46

NSAIDs

Question 47

Pregnant patients with lupus have the best prognosis when they achieve remission for how many months before pregnancy?

Answer 47

>/= 6 months

Question 48

What drugs for lupus can be used in pregnancy?

Answer 48

*Hydroxychloroquine* -1st choice NSAIDs (until wk 20) Glucocorticoids -lowest dose for shortest time

Question 49

What is antiphospholipid syndrome?

Answer 49

An autoimmune disorder than can cause blood clots and miscarriages

Question 50

For patients with the antiphospholipid antibody, what do we give as prophylaxis?

Answer 50

No prior fetal loss: Aspirin 81mg Recurrent fetal loss: Aspirin 81mg +/- LMWH

Question 51

For patients with antiphospholipid antibody, what do we give as therapy for acute thrombotic events/ hx of thrombosis?

Answer 51

LMWH