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SLE (Clinical Features (need 4/11 for dx) (Neurologic Disorder (Seizure,…
SLE
Clinical Features (need 4/11 for dx)
Skin
Malar Rash (50%)
Sun sensitive. Spares nasolabial folds.
Discoid Rash (25%)
Discrete, erythematous, slightly infiltrates plaques w/ well-formed adherent scale. Ears, scalp.
Photosensitivity (60-100%)
Latency hrs-wks. SPF 30 always. Protective clothing. Avoid sun.
Livedo Reticularis (30%)
See burnt orange tab.
Oral
Ulcers (12-45%)
Usually painless. Can be nasal. No specific Rx.
Lungs/Heart
Serositis
Inflammation of serous membrane (lungs, heart, rarely abd cavity)
Pleurisy (pleuritic pain, friction rub)
Pleural effusion (CXR)
Pericarditis (EKG, rub)
Pericardial effusion (echo or other imaging)
Libman-Sacks Endocarditis (LSE):
nonbacterial, verrucous thrombi on mitral or aortic valve.
Renal Disease
Lupus Nephritis (50-75%)
Typically onsets w/in first ~4 yrs of disease
Criteria:
Persistent proteinuria >0.5g/day. Cellular casts.
Untreated, 50% progress to ESRD
Treatment
I/II (Minimal mesangial, mesangial proliferative: good, no treatment
III/IV (Focal proliferative, diffuse proliferative):
HTN, proteinuria, active urine sediment, +dsDNA, low C3/C4, rising Cr.
Severe, aggressively treat.
V (Membranous): heavy proteinuria, bland sediment. Intermediate, treat.
VI (Advanced sclerosing): ESRD
Neurologic Disorder
Seizure
Psychosis
Peripheral neuropathy
Stroke
Always r/o other causes:
2/3 attributable to something else
Hematologic disorder
Leukopenia (<4000)
Lymphopenia (<1500)
Thrombocytopenia (<100,000)
Hemolytic anemia
Non-erosive arthritis (90%)
Inflammatory symptoms: swelling, tenderness, >30 min AM stiffness
Most often involves small joints of hand
Jaccoud's Arthropathy
: tendon inflammation, laxity, reversible w/ hand placed on table (ulnar dev. of RA cannot do this)
No evidence of erosions, cysts on films
Labs/Immunologic Criteria (need 1/6 for dx)
(+) ANA
(
sensitive
, not diagnostic in pt w/o symps)
ANA titer (higher = more significant)
Anti-dsDNA
(
specific
, poor prognosis - renal disease)
Anti-Smith
(
specific
, not prognostic)
Anti-phospholipid Abs. (anticardiolipin, lupus anticoag, B2 glycoprotein)
Complements (decr. C3, C4, CH50 b/o immune complex formation)
Epidemiology
Female:Male 9:1
Onset: teens-40s
Higher prevalence in people of color than whites (AAs 3-6x risk, Hispanic/NA 2-3x, Asian 2x)
Autoimmune diseases (e.g. thyroid) in family, but not necessarily specifically SLE
Treatment
Anti-Inflammatory
NSAIDs
ASA
Low dose to decr. risk of CAD
Hydroxychloroquin
e
Reduces flares, improves morb/mort., decr. organ damage, reduces thrombosis, improves lipids, enhances other drugs' effectiveness
SE: Retinal tox.
Steroids
Topical
Systemic (pred)
Immunosuppression
MTX
TNF-a antagonists
Cyclophosphamide (severe disease)
AZA
Subacute Cutaneous Lupus
Erythematous papules or small plaques w/ slight scaling
May simulate psoriasis or polymorphous light eruption
Neonatal lupus erythematosus
: resolves in 4-6 wks. Non-scarring. Check for cardiac abnormalities (heart block?)
Livedo Reticularis
Associated w/ antiphospholipid syndrome.
Seen in ~30% of SLE pts
Mottled rash w/ lace-like, purple-ish discoloration
Raynaud's
Cold-induced vasospasm
50% of SLE
White (vasospasm) -> Blue (ischemia) -> Red (re-perfusion)
Prognosis
VARIABLE
Most risk from early organ disease (esp renal), infection throughout (esp w/ immunosuppression), and later on, CAD.
Pregnancy
Better if 3-6 mo. quiet
STOP teratogenic meds: Cellcept, Cytoxan, MTX.
Check labs
SSA (Ro), SSB (La): fetal heart block 2%
Antiphospholipid Abs: Cause early reccurent fetal loss, late term loss.
Lupus anticoag.
Cardiolipin
Beta-2 glycoprotein
Drug-Induced Lupus
Symptoms
Rash
Arthritis
Pleuropericarditis
Cytopenias
Fevers
Labs
(+) ANA
Anti-Histone Ab
dsDNA (less common)
NOT SEEN: SSA, SSB, Smith
Treatment = D/C drug
Common offenders
Hydralazine, TNF-inhibs, procainamide, methyldopa, minocycline.
Isoniazid, nitrofurantoin, penicillin, sulfonamides, tetracycline