Subacute Cutaneous Lupus Erythematosus (SCLE) - Most photosensitive lupus rash - Effects torso and limbs, spares face - More often drug induced

LUPUS Flashcards by Demetré Harris

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

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

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Environmental triggers for Lupus

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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What are the 11 criteria for Lupus

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

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DDx Malar rash from rosacea

Rosacea

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

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DDx Seborrheic Dermatitis from malar rash

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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What is discoid lupus

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

Raised erythematous patches with adherent keratotic scaling and follicular plugging

PAINFUL!

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Define ACLE

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

-Localized
Malar rash of the face

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Define SCLE

Subacute Cutaneous Lupus Erythematosus (SCLE)

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

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Define CCLE

Chronic Cutaneous Lupus Erythematosus CCLE

Discoid lupus
Painful

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What is the most common presenting feature of Lupus

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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What is the hallmark lab for renal lupus nephritis

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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What are the Occular manifestations of lupus

SLE retinopathy

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

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How does lupus effect the cardiovascular

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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What is the most common pulmonary manifestation of lupus

Pleurtitis

And Pleural effusions in up to 50% of pts

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What should be done on any Lupus pt with CNS involvement

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

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How can lupus show up on MRI

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

Most common HeaM finding in a pt with lupus

Leukopenia

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

Think

Hemolytic anemia

What is the most common anemia in a pt with lupus

Anemia of chronic disease

Lab findings in Lupus

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

If a pt has Antiphospholipid abs and lupus

What is the elevated risk for this pt

Lupus anticoagulant, Anti-Cardiolipin AB

-Risk factor for venous and arterial thrombosis and miscarriage

Anti dsDNA is associated with the kidneys.. .how

Glomerulonephritis

Anti-Sm is associated with

AntiU1RNP Abs

What screenings should be done for a pt with lupus

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

Non Rx approach to Lupus Tx

Photoprotection (75% SLE pts are photosensitive) Tobacco cessation Patient education Exercise Healthy diet Vaccination

What medication should be in every Lupus Tx regiment

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

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

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

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

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

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

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

What is the only FDA approved Rx for lupus tx

Belimumab (Benlysta) -Only SLE medication approved by the FDA Consider in patients with non-renal SLE who have ongoing dz activity

What is the approach to cutaneous manifestations of lupus

Topical steroids use low potency first

What are the common manifestations of Lupus flares

Photosensitive rash, polyarthritis, serositis, and fatigue And low complement

What are the medications that cause drug induced lupus

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

A pt presents with polyarthritis, myalgia, fever, and serositis And Anti-histone antibodies Think what type of lupus

Drug induced Common Rx: hydralazine, isoniazid, procainamide, minocycline, interferon alpha, anti-TNF agents Tx; stop the Rx

Should lupus pts take COCs?

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

What is the PATH acronym for Lupus and pregnancy

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

What are the safe contraceptives to give to Lupus pts

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

How do we use heparin and asprin in pregnant lupus pts

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

Define Antiphospholipid Antibody Syndrome

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

If a pt develops a DVT or has a pregnancy lost with Lupus What should be suspected

Antiphospholipid Antibody Syndrome

What is the treatment for Antiphospholipid Antibody Syndrome

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)