Systemic Lupus Erythematous¶
Eva Niklinska
Background¶
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Characterized by multisystem inflammation, activation of complement, autoantibody formation
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Presentation:
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Constitutional: fatigue, fevers, weight loss
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Neurologic: cerebritis, myelitis, mononeuritis multiplex, psychosis
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Skin: malar rash (spares nasolabial folds), discoid lesions (scarring), photosensitivity
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Hematologic: leukopenia, anemia, thrombocytopenia
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Pulmonary: pleuritis, pleural effusion, ILD
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Cardiac: pericarditis/myocarditis, increased risk for CAD, Libman-Sacks endocarditis
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Renal: nephritic/nephrotic syndrome
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MSK: arthralgias, myalgias, arthritis (nonerosive, often symmetric)
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Ophtho: keratoconjunctivitis sicca 2/2 concurrent Sjogren’s
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Evaluation¶
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Labs to check upon admission: dsDNA (don’t re-send ANA/ENA panel if known ddx), C3/C4, ESR, CRP, CBC w/ diff, CMP, UPC
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2019 EULAR/ACR classification criteria for diagnosis:
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ANA (≥ 1:80) + additive scoring (classify as SLE if score is 10+)
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Do not count criteria if there is a more likely explanation for it
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Occurrence of a criterion on one occasion is sufficient. Criteria do not need to occur simultaneously. At least one criterion must be clinical
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Ddx: RA, MCTD, SS, Sjogren’s, Vasculitis (Behcet’s), PM/DM, Fibromyalgia
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* Note + ANA is now entry criteria [ANA only needs to be + once]
| Clinical Criteria | Weight | Laboratory Criteria | Weight |
|---|---|---|---|
Constitutional Fever |
2 | Antiphospholipid antibodies (Lupus AC, CL, β2GP1) |
2 |
Hematologic Leukopenia Thrombocytopenia Autoimmune hemolysis |
3 4 4 |
Complement proteins Low C3 OR C4 Low C3 AND C4 SLE-specific antibodies |
3 4 |
Neuropsychiatric Delirium Psychosis Seizure |
2 3 4 |
Anti-dsDNA OR Anti-Smith |
6 |
Mucocutaneous Non-scarring alopecia Oral ulcers Subacute cutaneous OR discoid lupus Acute cutaneous lupus |
2 2 4 6 |
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Serosal Pleural or pericardial effusion Acute pericarditis |
5 6 |
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Musculoskeletal Joint involvement (2+ joints) |
6 | ||
Renal Proteinuria (>0.5g/24h) Renal Bx Class II or V lupus nephritis Renal Bx Class III or IV lupus nephritis |
4 8 10 |
Management¶
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Hydroxychloroquine mainstay of treatment, 200-400 mg/day; dose 5 mg/kg/day for long term to decrease risk of retinal toxicity
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Safe in pregnancy; decreases VTE/HLD risk
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Annual ophtho exam for retinal toxicity
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NOT immunosuppressive
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Glucocorticoids for flares: Usually started with hydroxychloroquine and tapered once hydroxychloroquine has taken effect
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Other immunosuppressive medications (MTX, MMF, AZA, RTX) used with rheumatology consultation: MMF, cyclophosphamide or other advanced therapies used in SLE with renal involvement or severe end organ involvement