LE

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

Malar Rash

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 Ч 10 3cells/µL on >1 occasion)- lymphopenia (< 1500 cells/µL on >1 occasion)- thrombocytopenia (< 100 Ч 10 3 cells/µL in the absence of offending medications)- hemolytic anemiaRenal 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 lupusImmunologic phenomenaAnti dsDNA anti-Smith (Sm) antibodiesantiphospholipid 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