Clinical Features (need 4/11 for dx)
Malar Rash (50%)
Sun sensitive. Spares nasolabial folds.
Discoid Rash (25%)
Discrete, erythematous, slightly infiltrates plaques w/ well-formed adherent scale. Ears, scalp.
Latency hrs-wks. SPF 30 always. Protective clothing. Avoid sun.
Livedo Reticularis (30%)
See burnt orange tab.
Usually painless. Can be nasal. No specific Rx.
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.
Lupus Nephritis (50-75%)
Typically onsets w/in first ~4 yrs of disease
Persistent proteinuria >0.5g/day. Cellular casts.
Untreated, 50% progress to ESRD
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
Always r/o other causes:
2/3 attributable to something else
Non-erosive arthritis (90%)
Inflammatory symptoms: swelling, tenderness, >30 min AM stiffness
Most often involves small joints of hand
: 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)
, not diagnostic in pt w/o symps)
ANA titer (higher = more significant)
, poor prognosis - renal disease)
, not prognostic)
Anti-phospholipid Abs. (anticardiolipin, lupus anticoag, B2 glycoprotein)
Complements (decr. C3, C4, CH50 b/o immune complex formation)
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
Cyclophosphamide (severe disease)
Low dose to decr. risk of CAD
Reduces flares, improves morb/mort., decr. organ damage, reduces thrombosis, improves lipids, enhances other drugs' effectiveness
SE: Retinal tox.
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?)
Associated w/ antiphospholipid syndrome.
Seen in ~30% of SLE pts
Mottled rash w/ lace-like, purple-ish discoloration
50% of SLE
White (vasospasm) -> Blue (ischemia) -> Red (re-perfusion)
Better if 3-6 mo. quiet
STOP teratogenic meds: Cellcept, Cytoxan, MTX.
SSA (Ro), SSB (La): fetal heart block 2%
Antiphospholipid Abs: Cause early reccurent fetal loss, late term loss.
Most risk from early organ disease (esp renal), infection throughout (esp w/ immunosuppression), and later on, CAD.
dsDNA (less common)
NOT SEEN: SSA, SSB, Smith
Treatment = D/C drug
Hydralazine, TNF-inhibs, procainamide, methyldopa, minocycline.
Isoniazid, nitrofurantoin, penicillin, sulfonamides, tetracycline