This lecture was conducted during the Nephrology Unit Grand Ground by a Sub-intern under Nephrology Division, Department of Medicine in King Saud University. Nephrology Division is NOT responsible for the content of the presentation for it is intended for learning and /or education purpose only.

Systemic Lupus Ereythematosus By : Abrar Y. Faden 5 th year medical student

Out Line Background & Overview Clinical manifestation Work ups Diagnosis Treatment & Management

Definition It’s a chronic inflammatory autoimmune disease. changeable manifestations along with relapsing & remitting course.

Pathophysiology Characterized by a multisystem microvascular inflammation with the generation of aoutoantibodies. Defect in the apoptosis / necrosis process. Either mediated by immune complexes or direct effect.

Although a specific cause is unknown, many factors can associate in the development of SLE including : racial, hormonal, genetic & environmental factors. Many immune disturbances, innate & acquired accures in SLE.

Environmental and exposure-related causes Silica dust and cigarette smocking Administration of estrogen to postmenopausal women that low vitamin D levels increase autoantibody production & was also linked to B-cell hyperactivity Photosensitivity is clearly a precipitant of skin disease. Breastfeeding

Classic presentation Has a triad of: Fever, Joint Pain & Rash. But may present with any of many symptoms: Constitutional, musculoskeletal, dermatology, renal, neuropsychiatric, cardiac, pulmonary or hematologic, gastrointestinal.

Diagnostic Criteria (SOAP BRAIN MD) Serositis: pleurisy, pericarditis Oral ulcer: palatal or nasopharyngeal Artheritis: nonerosive, >2 joints, tenderness, swelling, or effusion Photosensitivity Blood disorder: leukopenia< 4000/mm3, lymphopenia <1500/mm3, thrombucytopenia < 100,000/mm3, hemolytic anemia

Renal involvement: protienuria >0.5 g/d or >3+ if quantitation not performed Antinuclear antibodies (ANAs): >1:160 abnormal Immunological phenomena: dsDNA, APAB Neurological disorders: seizures or psychosis Malar rash Discoid rash Diagnostic Criteria (SOAP BRAIN MD) Renal involvement: protienuria >0.5 g/d or >3+ if quantitation not performed Antinuclear antibodies (ANAs): >1:160 abnormal Immunological phenomena: dsDNA, APAB Neurological disorders: seizures or psychosis Malar rash Discoid rash

Diagnosis A pt classified as having SLE if he has a biopsy- proven lupus nephritis with ANA or anti-dsDNA, or satisfies 4 of the diagnostic criteria. It must be based on the proper group of clinical & imaging findings & laboratory evidences.

Work up Screening laboratory studies to diagnose possible SLE should include the following: Complete blood count (CBC) with differential Serum creatinine Urinalysis ANA ; sensitivity 95%; not diagnostic without clinical features

More work ups Autoantibody tests used in the diagnosis of SLE: Anti-dsDNA - High specificity; sensitivity only 70%; level variable based on disease activity Anti-Sm - Most specific antibody for SLE; only 30-40% sensitivity ESR & CRP C3 & C4 levels Liver function test Creatinine kinase

More studies Renal & Skin biopsies Chest & joint Xrays MRI ECG Lumbar puncture

SLE Nephritis

Treatment Depends on the disease manefistations. Antimalarial, immunosuppressant, steroids & NSAID. Fever, rash, musculuskeletal and serositis >>hydroxychloroquine, NSAIDS & low-to-moderate– dose steroids Methotrexate Azathioprine & mycophenolate High dose steroids & cyclophosphamide

Management Depends on disease severity Regular follow up + laboratory testing

thanQ