LUPUS Flashcards
What factors lead to an increase risk of lupus in female pts
Early age at menarche, OCP use and postmenopausal hormone use increase risk
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)
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%)!
DDx Malar rash from rosacea
Rosacea
- Predominance of telangiectasias and pustules that may sting / burn
- Heat and alcohol intake worsen the erythema
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
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
If discoid lupus occurs in the mouth, how does it present
Raised erythematous patches with adherent keratotic scaling and follicular plugging
PAINFUL!
Define ACLE
Acute Cutaneous Lupus Erythematosus (ACLE)
-Generalized
Photosensitive maculopapular rash, primarily hands
-Localized
Malar rash of the face
Define SCLE
Subacute Cutaneous Lupus Erythematosus (SCLE)
- Most photosensitive lupus rash
- Effects torso and limbs, spares face
- More often drug induced
Define CCLE
Chronic Cutaneous Lupus Erythematosus CCLE
- Discoid lupus
- Painful
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
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
What are the Occular manifestations of lupus
SLE retinopathy
Immune complex mediated
Clinical findings: Cotton-Wool spots, retinal hemorrhages, hard exudates
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.
What is the most common pulmonary manifestation of lupus
Pleurtitis
And Pleural effusions in up to 50% of pts
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)
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)
