Exam 4 lecture 7 Flashcards by Matti Daniel

Define lupus

Chronic autoimmune disease with diverse clinical presentation.
Immune system attacks healthy tissue and organs throughout the body.
Control of this disease remains a challenge

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Predisposing factors to lupus

Genetics- 1st degree relatives; 20 x more likely to develop SLE

Hormonal- Estrogen production may modulate the incidence and severity of SLE

Env’t- CIgarette smoking, meds, UV light, air pollution, ciruses, stress, pesticides

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What is Drug induced lupus erythematosus (DILE)? WHen do symptoms occur? When does resolution occur?

Overreaction to certain meds
symptoms occur 3-6 months of drug initiation
Resolution occurs within weeks of drug dx

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What us a mnemonic to memorize drugs that cause DILE

My Pretty Malar Marking Probably Has A TransIent Quality

Every capital letter is a drug (including the I in transiet)

Methimazole
Propylthiouracil
Methyldopa
Minocycline
Procainamide
Hydralazine
Anti-TNF agents (infliximab and etanercept)
Terbinafine
Isoniazid
Quinidine

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s/s of lupus (SLE)

Fatigue
Depression
Photosensitivity
Joint pain
N/V
Fever
Weight loss
Malar “butterfly” Rash

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What are some mucocutaneous s/s of SLE?
Opthalmologic?
Renal?

mucocutaneous- butterfly rash, discoid rash, raynaud phenomenon

Opthalmologic- Lupus retinopathy

Renal- Lupus nephritis

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What is Raynaud Phenomenon

Exaggerated vascular response to cold temperature or emotional stress

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What are the two diagnostic tools that will be used for lupus

SLICC
EULAR

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How does SLICC criteria work

Must meet > 4 total features with 1 from each group

Biopsy proven lupus nephritis WITH systemic lupus

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criteria for EULAR

Patients score is > or = 10 AND atleast 1 clinical criterion is fulfilled

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What are some key labs for SLE? WHat is a normal range? WHat is its specificity?

Anti-nuclear antibody (ANA)- reference range (<1:40)= negative. Positive in lupus patients but not specific
Anti double stranded DNA (Anti-dsDNA)- Negative, High specificity (correlates with disease activity and is an important marker in lupus nephritis)
anti- smith antibody (Anti-SM)- negative- High specificity for diagnosis
Antiphospholipid antibody- negative, increases clotting factors

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What are 5 drug classes that patients will be on when they have lupus?

Hydroxychloriquin (HCQ)
NSAIDs
Glucocorticoids
Immunosuppressnats (IS)
Biologics

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MOA of HCQ? Place in therapy? Benefit of HCQ? Dosing (exam)

MOA- anti malaria-> inhibit overactive immune cells

Place in therapy- Recommended for ALL pts with SLE

Benefits- reduces flares and help manage pain

Dosing- 200-400mg PO daily

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Side effects of HCQ

-opthalmic: retinal toxicity (bulls eye maculopathy)
-Hemolytic anemia if you have G6PD deficiency
- CNS (depression, anxiety)
- CV- QT prolongation
- HS rxn

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What type of disorder is G6PD deficiency

X-linked disorder that causes RBCs to prematurely break down (happens more in males)

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What drug do we not give if patient has G6PD deficiency

HCQ

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Monitoring parameters for HCQ

CBC
LFTs
SCr
EKG

Periodic eye exam 3 onths after eye exams and annually there after

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MOA of NSAIDs? Benefits? Place in therapy?

MOA- inhibits COX 1 and 2 to decrease the formation of prostaglandin precursor

Benefits- Antipyretic, anti-inflammatory and analgesic

Place in therapy- 1st line for mild symptoms

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Dosing of Ibuprofen and naproxen

Ibuprofen- 400-600 mg PO Q 6-8 H
Naproxen- 500 mg PO BID

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Side effects of NSAIDs

GI bleeding, gastritis or perforation
CV- increased BP,worsening HF, CV events
Renal- increased Scr, renal toxicity
Hepatic- Hepatotoxicity

Monitoring parameters for NSAIDs

CBC
LFTs
SCr
BP
S/sx of fluid retention and bleeding

Glucocorticoid MOA? Benefits? Place in therapy?

MOA: Inhibits B and T cell responses
Benefits: Anti inflammatory and helpful during flares
Place- adjunct treatment if not responsive to NSAIDs, HCQ

Dosing of oral glucocorticoid (prednisone)

MIld-moderate disease: Prednisone 5-20 mg/day
Severe: 1 mg/kg/day

IV dosing of glucocorticoid

Methylprednisolone: 500-1000 mg IV x 3-6 days then PO prednisone

For topical glucocorticoids, name low potency, moderate potency and high potency glucocorticoids and where they are used

low potency: Flucinolone and hydrocortisone butyrte (face) Moderate: Triamcinalone and Betamethasone (Trunk and extremities) High potency: CLobetasol (scalp sores and palms)

PO/IV glucocorticoids side effects

Opthalmic: glaucoma CV: increased BP Bone: Increased risk of osteoporosis GI: GI bleed CNS: Psychosis/sleep disturbances Cushings syndrome weight gain

Topical glucocorticoids side effets

Skin atrophy, rosacea, telengiectasis

Monitoring for glucocorticoids

Baseline: BP, BMP, FLP, BMD ROutine BMP q 6 mo FLP 6 mo BMD: annually

Immunosuppressants MOA? Place in therapy? Meds?

MOA- suppression of immune system from attacking healthy cells Place in therapy: Adjunct to steroid therapy to lower the dose or insufficient response to HCQ meds: Methotrexate Mycophenolate cyclophosphamide azathroprine

Methotrexate dosing? side effects? Mycophenolate dosing? Side effects?

Methotrexate: 5-15 mg once weekly. May cuse BMS, infection Mycophenolate: 1-1.5 g BID, BMS, infection, malignancy, AIS

Cyclophosphamide dosing and side effects? Azathioprine Dosing and side effects?

Cyclophosphamide: 1 - 1.5 mg/kg once daily IV: 0.5 mg/m2 BSA q month c=x 6 months side effects: BMS, infection, malignancy Azathioprine: 50 mg daily BMS, infection, malignancy Monitor for TPMT deficiency in azathioprine BMS= bone marrow suppression

Biologics MOA? Place in therapy? Medications?

MOA: Monoclonal antibodies that block B cell mediated immunity Place in therapy: - inadequate response to antimalarial and immunosuppressants - severe disease Medication -Belimumab - rituxiamb - anifrolumab

Pearls with biologics with lupusq

No live vaccines 30 days before starting therapy OR during therapy Do not use more than 1 biologic at the same time

Belimumab dosing? Side effects?

10 mg/kg every 2 weeks x 3 doses HS and infusion rxn

Anifrolumab dosing and side effects

300 mg every 4 wks, HS rxn

Rituximab dosing? Side effects?

1g on days 0 and 15 or 375 mg.m2 once weekly for 4 doses Side effects- Infusion rxn, Hep B reactivation, ML premedicate 30 mins prior to administration

What is an additional biologic that can be used in lupus

Calcineurin inhibitors (CNI) - tacrolimus - vocolosporin (oral lupus med)

non pharm adjustments for lupus

Balance of rest and exercise Smoking cessation Limit sun exposure and use fo sun screen

How common and dangerous is cutaneous lupus? First line treatmemnt? Refractory treatment?

10% of lupus cses, rarely life threatening. Presents with rash and lesions 1st line: 1)TOpica; agents - GC: clobetasol, betamethasone, hydrocortisone,triamcinalone CNI: Tacrolimus, pimecrolimus 2)HCQ 3) systemic GC Refractory - high dose GC MTX MMF (mycophenolate)

how common and dangerous is lupus nephritis? Therapy for class I/II lupus nephritis?

LN is a serious complication of SLE which can affect 6-% of patients within 10 yrs of diagnosis Therapy Mild/moderate nephritis - GC+/- another immunosuppressive AZA, MMF or CNI Severe nephritis MMF (preferred) or CYC +/- GC Triple therapy: - belimumab + MMF or CYC +/- GC CNI + MMF +/- GC

Therapy for class III or IV lupus nephritis

-Glucocorticoid + -CNI + MPAA or -MPAA (Most preferred) (can have induction and maintenance) or - cyclophosphamide (2nd line after MPAA) or -Belimumab + MPAA or reduced dose cyclophosphamide

Drugs for FLuid retention Pain and inflammation?

Diuretic NSAIDs (recommend tylenol first)

Compare healthy pregnancy vs lupus pregnancy? When is the best prognosis COntraception?

Lupus pregnancies at more risk for - miscarriages - fetal growth retardation etc Best prognosis is when the patient achieves remission for > 6 months COntraception - Avoid estrogen containing contraception - Screen for antiphosholipif syndrome

Which drugs are safe to use in pregancy? Which should we discontinue

HCQ ( safe throughout pregnancy) NSAIDs ( discontinue at 20 weeks or later) GC- safe throughout MMF, CYC, MTX- contraindicated AZA- risk vs benefit with proveider Biologics- do not recommend

What is antiphospholipid syndorme

An auto immune disorder characterized by antiphospholipid syndrome that can cause blood clots and miscarriages

Prophylaxis for antiphospholipid antibody

- no prior fetral loss- apsirin 81 mg - Recurrent fetal loss- aspirin 81 mg + LMWH If acute thrombotic event/hx of thrombosis- LMWH

Can we use warfarin in pregnant woman

NEVER