Systemic Lupus Erythematosus

Autoimmune disease affects multisystem 1.5 million cases of lupus Prevalence of 17 to 48 per 100,000 population African Americans > Whites Chronic follows a relapsing and remitting course. More than 90% of cases of SLE occur in women, frequently starting at childbearing age Women > Men - 9:1 ratio Can occur in childhood or later in life Onset usually between ages of 15 and 45 years,

Clinaical Manifestations A person has SLE if 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation. (Specificity 95%, sensitivity 75%). It is important to remember that a patient may have SLE and not have 4 criteria.

Criteria 1. Butterfly rash 2. Discoid lupus 3. Photosensitivity 4. Oral ulcers 5. Arthritis 6. Serositis 7. Neurologic: seizure, psychosis 8. Hematologic cytopenias such as leukopenia, lymphopenia, anemia, or thrombocytopenia) 9. Renal: acute or chronic renal failure, acute nephritic disease) 10. Immunologic: anti-DNA, anti-phospholipid 11. Anti-nuclear antibody (ANA)

Types of cutaneous lupus About 80% of patients with SLE have skin involvement (cutaneous LE) and it is the first sign of SLE  Acute Subacute Chronic (discoid) Lupus profundus

Malar Rash Most common type is the acute rash Central face malar rash, flat or raised erythematous butterfly lesion involving both cheeks and nasal bridge resolves without scarring It is photosensitive

Discoid Lupus Erythematosus (DLE): Plugged hair follicles and adherent scale. Atrophy or thinning of the top layer of skin. Hardness of the skin. Telangiectasias. Erythema. Scarring & permanent hair loss can develop

Subacute cutaneous lupus erythematosus (SCLE) typically manifests in 1 of 2 forms: annular/polycyclic psoriasiform/papulosquamos Lesions typically occur in a photosensitive distribution. Many patients notice that sun exposure results in an exacerbation of their disease, and some report worsening each spring and summer.

Investigations Blood disorders - Leukopenia (< 4 × 10 3cells/µL on >1 occasion) - lymphopenia (< 1500 cells/µL on >1 occasion) - thrombocytopenia (< 100 × 10 3 cells/µL in the absence of offending medications) - hemolytic anemia Renal involvement – Based on presence of proteinuria (>0.5 g/day or 3+ positive on dipstick testing) or cellular casts (including red blood cells [RBCs], hemoglobin, granular, tubular, or mixed) or based on the opinion of a rheumatologist or nephrologist 

Antinuclear antibodies (ANAs)indirect immunoflourescent - Higher titers generally more specific (>1:160); must be in the absence of medications associated with drug-induced lupus Immunologic phenomena Anti dsDNA anti-Smith (Sm) antibodies antiphospholipid antibodies (anticardiolipin immunoglobulin G [IgG] or immunoglobulin M [IgM] or lupus anticoagulant) Anti (Ro, La antibodies） (immuno-blot) LBT lupus band test (direct immuno-fluorescence ) Biopsy

Treatment Topical steroids (potent ones or intralesional injections in case of discoid lupus) Sunscreens are mandatory in all cases Antimalarial agents: Hydroxychloroquin 200 mg twice daily. Systemic steroids, only for systemic involvement. Immunosuppressives

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