LUPUS Flashcards

1
Q

What factors lead to an increase risk of lupus in female pts

A

Early age at menarche, OCP use and postmenopausal hormone use increase risk

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2
Q

Environmental triggers for Lupus

A

Tobacco (increases anti-dsDNA)

Ultraviolet light (exacerbates SLE, not a trigger for new onset)

Epstein Barr Virus infection (molecular mimicry)

Drugs (e.g. procainamide, hydralazine, isoniazid, some antibiotics like minocycline)

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3
Q

What are the 11 criteria for Lupus

A
  1. Malar rash
  2. Discoid rash
  3. Photosensitivity
  4. Oral ulcers (painless)
  5. Arthritis
  6. Serositis
  7. Renal Disease
    (> 0.5 g/d proteinuria, or
    Cellular casts)
  8. Neurologic Disease
    - Seizures
    - Psychosis (without other cause)
  9. Hematologic Disorders
    - Hemolytic anemia, or
    - Leukopenia (< 4000/uL), or
    - Lymphopenia (< 1500/uL), or
    - Thrombocytopenia (< 100,000/uL)
  10. Immunologic abnormalities.
    - Anti-DNA, or
    - Anti-Sm, or
    - Antiphospholipid Antibodies
  11. Positive ANA (95-100%)!
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4
Q

DDx Malar rash from rosacea

A

Rosacea

  • Predominance of telangiectasias and pustules that may sting / burn
  • Heat and alcohol intake worsen the erythema
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5
Q

DDx Seborrheic Dermatitis from malar rash

A

Seborrheic dermatitis

  • Scaly erythematous plaques that occur on the eyebrows, scalp, behind the ears and ear canal
  • Commonly found within the nasolabial folds
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6
Q

What is discoid lupus

A

Chronic cutaneous lupus erythematosus (CCLE)
-Discoid Lupus lesions typically expand with peripheral erythema and hyperpigmentation

Leaves hallmark atrophic central scarring, telangiectasia, and hypopigmentation

Most commonly found on face, scalp, and neck

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7
Q

If discoid lupus occurs in the mouth, how does it present

A

Raised erythematous patches with adherent keratotic scaling and follicular plugging

PAINFUL!

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8
Q

Define ACLE

A

Acute Cutaneous Lupus Erythematosus (ACLE)
-Generalized
Photosensitive maculopapular rash, primarily hands

-Localized
Malar rash of the face

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9
Q

Define SCLE

A

Subacute Cutaneous Lupus Erythematosus (SCLE)

  • Most photosensitive lupus rash
  • Effects torso and limbs, spares face
  • More often drug induced
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10
Q

Define CCLE

A

Chronic Cutaneous Lupus Erythematosus CCLE

  • Discoid lupus
  • Painful
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11
Q

What is the most common presenting feature of Lupus

A

Arthritis
-Occurs in 95% of SLE patients

-Transient, symmetric, affects hands, wrists and knees, seldom deforming, non-erosive, less severe than RA

Most common presenting feature of SLE

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12
Q

What is the hallmark lab for renal lupus nephritis

A

Hallmark clinical finding is hematuria and/or proteinuria

Screening SLE patients at 3 month intervals is prudent.
24H urine protein; spot urine protein/creatinine ratio

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13
Q

What are the Occular manifestations of lupus

A

SLE retinopathy

Immune complex mediated
Clinical findings: Cotton-Wool spots, retinal hemorrhages, hard exudates

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14
Q

How does lupus effect the cardiovascular

A

Raynauds

+/- pericarditits

Valvular heart disease :Mitral and aortic valves thicken, with or w/o nonbacterial vegetations. (Libman-Sacks Endocardititis)

Coronary artery disease
-SLE is associated with accelerated atherosclerosis and is itself a risk factor for CAD.

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15
Q

What is the most common pulmonary manifestation of lupus

A

Pleurtitis

And Pleural effusions in up to 50% of pts

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16
Q

What should be done on any Lupus pt with CNS involvement

A

Lumbar Puncture should be conducted on any SLE patient with suspected CNS involvement (rule out infection)

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17
Q

How can lupus show up on MRI

A

T2 hyperintense focal lesions in the periventricular and subcortical white matter that can appear identical to the lesions seen in multiple sclerosis

MRI of the spinal cord is important to confirm the diagnosis of myelopathy
-widening of the cord from edema

18
Q

Most common HeaM finding in a pt with lupus

A

Leukopenia

19
Q

A lupus pt with Increased Reticulocytes Count, unconjugated Bilirubin, LDH
Reduced Haptoglobin
Positive Coombs test

Think

A

Hemolytic anemia

20
Q

What is the most common anemia in a pt with lupus

A

Anemia of chronic disease

21
Q

Lab findings in Lupus

A

CBC: Anemia (AIHA/MAHA), leukopenia, thrombocytopenia

Chemistry—hyperkalemia from renal tubular acidosis or poor renal function

LFT – generally from medications

CK elevated in associated myositis (more common in mixed connective tissue dZ)

UA = hematuria, proteinuria

24hr urine protein (preferred over spot urine proteinuria tests)

Acute phase reactants: ESR correlates with SLE activity, but is nonspecific (anemia/renal disease can elevate). CRP does not correlate with SLE.

Anti-dsDNA antibodies are highly specific for SLE
Anti-SM highly specific for SLE

22
Q

If a pt has Antiphospholipid abs and lupus

What is the elevated risk for this pt

A

Lupus anticoagulant, Anti-Cardiolipin AB

-Risk factor for venous and arterial thrombosis and miscarriage

23
Q

Anti dsDNA is associated with the kidneys.. .how

A

Glomerulonephritis

24
Q

Anti-Sm is associated with

A

AntiU1RNP Abs

25
Q

What screenings should be done for a pt with lupus

A

High CAD risk factor modification (HTN/HLD)
Vaccines (Influenza/PPV 23)
Bone health (Cal/Vit. D)
annual cervical cancer screening.

26
Q

Non Rx approach to Lupus Tx

A

Photoprotection (75% SLE pts are photosensitive)

Tobacco cessation

Patient education

Exercise

Healthy diet

Vaccination

27
Q

What medication should be in every Lupus Tx regiment

A

Hydroxychloroquine (HCQ) or Chloroquine (CQ) should be part of every lupus pt’s treatment regimen

28
Q

If a pt is on hydroxychlorquine, what is the most feared ADE

A

Most feared side effect of HCQ is irreversible retinopathy

Patients should receive ophthalmology screenings at initiation of therapy and then annually after the 5th year of therapy

29
Q

What is the treatment for a pt with lupus and persisitently active disease despite antimalarial therapy

A

Immunosuppressive Rx

Azathioprine : first immunosuppressive agent added to HCQ for non-renal manifestations
-Safe in pregnancy

MMF: Effective for renal manifestations. Teratogenic

Methotrexate: best when inflammatory arthritis is predominant

Leflunomide: used for mostly cutaneous & musculoskeletal features

Cyclophosphamide: reserved for more severe manifestations of lupus

IVIG: used for hematologic manifestations
-Also effective for systemic infections when immunosuppressives are being avoided

30
Q

What is the mainstay and 1st line therapy for lupus manifestations

A

Steroids

Mainstay and first-line therapy for most lupus manifestations

  • Attempt to limit to short-term use only
  • Low dose therapy (5-20mg of prednisone daily)

Reserve higher doses (e.g. 40-60mg daily) for severe disease manifestations

Steroid tapers usually 3 months in length

Very high doses for CNS lupus or severe lupus nephritis

31
Q

What is the only FDA approved Rx for lupus tx

A

Belimumab (Benlysta)
-Only SLE medication approved by the FDA

Consider in patients with non-renal SLE who have ongoing dz activity

32
Q

What is the approach to cutaneous manifestations of lupus

A

Topical steroids use low potency first

33
Q

What are the common manifestations of Lupus flares

A

Photosensitive rash, polyarthritis, serositis, and fatigue

And low complement

34
Q

What are the medications that cause drug induced lupus

A

Classically associated with hydralazine, isoniazid, procainamide, minocycline, interferon alpha, anti-TNF agents
-Hydrochlorothiazide is associated with Subacute Cutaneous Lupus

35
Q

A pt presents with polyarthritis, myalgia, fever, and serositis
And Anti-histone antibodies

Think what type of lupus

A

Drug induced

Common Rx: hydralazine, isoniazid, procainamide, minocycline, interferon alpha, anti-TNF agents

Tx; stop the Rx

36
Q

Should lupus pts take COCs?

A

Oral contraceptives traditionally “forbidden” due to concerns about worsening SLE activity although risk probably overstated
(2005 OC-SELENA trial)

37
Q

What is the PATH acronym for Lupus and pregnancy

A

Proteinuria
Antiphospholipid syndrome
Thrombocytopenia
Hypertension

The presence of any of these four risk factors during the first trimester of pregnancy is associated with at 30–40% risk of pregnancy loss

38
Q

What are the safe contraceptives to give to Lupus pts

A

Levonorgestrel IUD safe and effective for most pts with SLE

Oral estrogen-progestin may be used in pts with stable low disease activity and documented NEGATIVE antiphospholipid antibodies

39
Q

How do we use heparin and asprin in pregnant lupus pts

A

Heparin and aspirin for pregnant patients who also have Antiphospholipid Syndrome (APS)

40
Q

Define Antiphospholipid Antibody Syndrome

A

Hypercoagulability -> arterial or venous thrombosis
DVT, PE, stroke, renal injury

Recurrent pregnancy losses

Autoantibodies to phospholipid

  • Anticardiolipin antibodies (IgG/IgM)
  • ß2-glycoprotein I

Lupus anticoagulant activity (prolonged aPTT)

Can occur independently or in association with SLE

41
Q

If a pt develops a DVT or has a pregnancy lost with Lupus

What should be suspected

A

Antiphospholipid Antibody Syndrome

42
Q

What is the treatment for Antiphospholipid Antibody Syndrome

A

Anticoagulation
-No primary prevention recommended

Secondary prevention for venous/arterial thromboembolism

  • Heparin ->Warfarin (INR goal 2-3)
  • Direct anticoagulant (Rivaraxoban, etc.): Less effective
  • Lifelong anticoagulation

For individuals who are pregnant or become pregnant: LMWH preferred over warfarin (teratogenic)