Lupus

Question 1

What is Systemic LUPUS Erythematosus?

Answer 1

• Autoimmune Disease: Body attacks self
• Causes widespread inflammation and tissue damage that affects almost any organ

Question 2

What is the Epidemiology of Lupus?

Answer 2

• 16,000 new cases/year
• Females >males
• Age 15-45

Question 3

What is the Pathophysiology of Lupus?

Answer 3

• Genes & Environment
• Leading to Abnormal Immune Response
• Making Autoantibodies Immune Complexes
• Resulting Inflammation & Damage

Question 4

what are some of the Risk Factors for Lupus?

Answer 4

• Environmental: Infections, UV, Dust, Soil, Allergens, Smoking
• Genetic: 3+ on X-Chromosome –> why its female
• Hormonal

Question 5

What is the way that Drug-INduced Lupus Erythematosus [DILE] happens?

Answer 5

• Its the overreaction to meds
• Symptoms within 3-6 months
• Resolution within weeks

Question 6

What are some of the Medications that cause DILE in Lupus?

Need to know!!

Answer 6

• Metimazole, Propylthiouracil
• Methyldopa
• Minocycline
• Procainamide
• Hydralazine
• Anti-TNF Agents
• Terbinafine
• Isoniazid
• Quinidine

Question 7

What are some of the symptoms of Lupus?

Answer 7

• Fatigue, Depression, Weight loss of gain, Muscle pain, “Butterfly” Rash, Photosensitivity, Joint Pain

Question 8

What are some of the areas that Lupus could affect?

Answer 8

• Brain: Psychosis, Seizures
• Lungs: Pneumonitis
• Vessels: Vasulitis
• Kidneys: LUPUS NEPHRITIS
• Skin: Erythema, Butterfly Rash
• Heart
• Spleen
• Joints

Question 9

What are some other important information about Lupus?

Mortality? QoL? Cost?

Answer 9

• Mortality: 10-15% will die early
• QoL: Limited functioning; 89% cant work
• Cost: Very expensive $33,223

Question 10

What is Lupus known as?

Answer 10

• “Great Imitator”
• Mimics other diseases

Question 11

Based on the 2012 SLICC Criteria, what is important to know about Diagonsis of Lupus?

Dont need to know everything just breif info

Answer 11

• Clinical & Immunologic Criteria
• Must meet 4 of 17 criteria [>1 clinical & >1 immunologic]; OR biopsy

Question 12

What is the most common Immunologic Criteria that we look at for Lupus Diagnosis?

Answer 12

• ANA: 98% prevalence

Question 13

What are the goals of therapy for Lupus?

Answer 13

• Induction: Control Disease
• Maintenacne: Maintain Remission; Prevent Flares
• Adjunctive: Reduce drugs adverse effects

Decreased Flares; Increase Remission

Question 14

What are some of the Pharmacologic Treatments for Lupus?

Answer 14

• Hydroxycholorquine
• Glucocorticoids
• Immunsuppressants
• Biologics

Question 15

What is important to know about Hydrocholoquine in Lupus?

MOA? Place?

Answer 15

• MOA: Antimalarial –> Reduces Autoantibodies
• Place: ALL patients with Lupus

Question 16

What are some of the Side Effects of Hydrocholoquine in Lupus?

Answer 16

• Cardiomyopathy, Hemolytic Anemia [G6PD Deficiency], Hypoglycemia, QTc Prolongation, Rentinal Toxicity

Eye Exam ANNAULLY

Question 17

Breif describe what happens within G6PD Deficiency in Lupus?

Answer 17

• G6PD makes glutathione that fights free radicals
• SO; Decreased G6PD = Increased Free Radicals = Lysed RBC

Question 18

What is the importance of Steroids in Lupus?

MOA? Place?

Answer 18

• MOA: Decreased WBC Activity
• Place: Mild-Mod = PO Steroids; Severe = IV Steroids

Question 19

What are some of the steriods that are used in Lupus?

Answer 19

• IV Methyprednisolone
• PO Prednisone

Question 20

What is the Maintenacne goal for steriods in Lupus?

KNOW FOR EXAM

Answer 20

• < 7.5 mg/day [prednisone equivalent] and stop ASAP

Question 21

What are some of the Side Effects of Steroids in Lupus?

Important to know

Answer 21

• Cardio, Insomina, Cushing Syndrome, N/V/D, Ulcers, Hyperglycemia, Infections, Osteoporosis, Glaucoma

Question 22

What is the MOA of Immunosuppressants in Lupus?

Answer 22

• MOA: Stops immune system from attacking healthy tissues

Question 23

What is the Place of Therapy for Immunosuppresants in Lupus?

Answer 23

• Insufficient response in HCQ & are Steroid Sparing [helps decreased steroid use]
• Mild Disease = MTX
• Mod Disease = AZA, CNI, MMF
• Severe Disease = CYC [a lot of side effects]

Question 24

What are some of the Side Effects for the Immunosuppresants in Lupus?

For MTX, MMF, AZA, CYC

Answer 24

• Bone Marrow Suppression, Infections, Malignancy

Question 25

What are two important things to understand about Methotrexate & Azathioprine in Lupus?

Answer 25

- MTX: **dosed WEEKLY** - AZA: Genetic Test --> **TPMT/NUDT15 Deficiency**

Question 26

Breif describe the TPMT deficiency in Lupus?

Answer 26

- AZA --> 6-MP and TPMT makes it **INACTIVE** - SO; **Decreased TPMT will Increase 6-MP [AZA] toxicities = Bone Marrow Suppression**

Question 27

What is the MOA for the Biologics in Lupus

Answer 27

- MOA: **Monoclonal Antibodies blocks B-cells**

Question 28

What is the Place of Therapy for the Biologics in Lupus?

Answer 28

- Inadeqate response to HCQ + S - Mod Disease = **belimumab** - Severe Disease = **rituximab**

Question 29

What are some of the Side Effects for the Biologics in Lupus?

Answer 29

- Hypersensitivity and/or Infusion Reactions - Premedicate | **Rituximab has Hep B reactivation and PML**

Question 30

What are some of the medications that are used for Premedication in Bioloigcs in Lupus?

Answer 30

- Acetaminophen [NSIADs], Histamines, Methylprednislone

Question 31

What are some of the treatments for Cutaneuos Lupus?

Answer 31

- First Line: **Topical** [clobetasol, betamethasone, triamcinolone, hydrocortisone], **HCQ, Steriods**

Question 32

What are some of the treatments for Lupus Nephritis?

Answer 32

- **Induction**: MMF or CYC + Steriods - **Maintenance**: MMF or AZA [not in TPMT deficiency] - **Severe**: RTX

Question 33

What is Anti-Phospholipid Syndrome in Lupus? | MOA?

Answer 33

- Autoimmune disorder with **antiphospholipid antibodies** that causes blood clots and miscarrage

Question 34

What are some of the Prophylactic Treatments that are used in Anti-phospholipid syndrome in Lupus?

Answer 34

- Primary Prophylaxis: **Aspirin 81mg** - Secondary Prophylaxis: **Warfarin +/- Aspirin 81mg** - During Pregnancy: **Enoxaparin**

Question 35

What is important to know about Pregnancy in Lupus?

Answer 35

- Higher maternal and fetal risk [increased **Miscarrage & flares**] - Should start to try to get pregnant **6 months** after remission - Avoid: **Cyclophosphamide, Mycophenolate, Methotrexate** --> wati **3-6 months** before trying