Systemic Lupus Erythematosus (SLE)

dr. Isbandiyah, SpPD

Introduction

Lupus is an autoimmune disease characterized by acute and chronic inflammation of various tissues of the body Unknown cause Can affect :

Skin Joints Kidneys Lungs Nervous system Serous membranes Other organs of the body

Types of Lupus

Systemic Lupus: This type of lupus is the most common, it affects major organs, and can be fatal.
Discoid Lupus: This type of Lupus affects only the skin. It is not fatal, but can cause severe scarring, and may develop into Systemic Lupus if not treated. Drug-induced Lupus: This form of Lupus is Systemic Lupus caused by certain medications. When the medicine is stopped, the disease goes away.

Who Gets SLE?

80-90% are women People of African, Native American, Hispanic, and Asian descent are more likely to get Lupus. Prevalence of SLE in US is 15 to 50 per 100,000.

Pathophysiology

ARA Criteria for Diagnosis of Systemic Lupus Erythematosus

Criteria
Malar rash Discoid rash
Photosensitivity Oral ulcers Arthritis Serositis Renal disorder Neurologic disorder Hematologic disorder Immunologic disorders Antinuclear antibody

Definition
fixed erythema, flat or raised over the malar eminences, tending to spare the nasolabial fold erythematous raised patches with adherent keratotic scalling and follicular plugging, athropic scarring may occur in older lesions skin rash is a result of unusual reaction to sunlight, by patient history or physician observation oral or nasopharyngeal ulceration, usually painless, observed by physician non erosive arthritis involving 2 or more peripheral joints characterized by tenderness, swelling or effusion pleuritis history of pleuritic pain or rub heard by physician or evidence of pleural effusion or pericarditis decumented by ECG, rub or evidence of pericardial effusion persistent proteinuria (> 0.5 gr/day or > 3+ if quantitation not perfomed) or cellular casts (may be red cell, hemoglobin, granular, tubular or mixed) seizures or psychosis in the absence of offending drugs or known metabolic derangements (uremia, ketoacidosis, or electrolyte imbalance) hemolytic anemia - with reticulocytosis or leucopenia (< 4000/mm3) or lymphopenia (< 1500/mm3) or thombocytopenia (< 100.000/mm3) positive antiphospholipid antibody or Anti DNA (antibody to native DNA in abnormal titer) or Anti Sm (presence of antibody to Sm nuclear antigen) an abnormal titer of antinuclear antibody by immuno fluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drugs induced lupus syndrome

Lupus criteria

M- Malar rash D- Discoid rash S- Serositis O- Oral ulcers A- Arthritis P- Photosensivity

B- Blood abnormalities R- Renal A- ANA antibodies I - Immune abnormalities N- Neurologic

Lupus symptoms

Arthritis

Musculoskeletal

Myositis Tenosynovitis Ischemic necrosis of bone

Cutaneous

80 percent Mallar rash : Ig and complement at the dermal-epidermal junction,

Cutaneous

Discoid rash

Cutaneous

Oral ulcers

Alopecia Vasculitis rash Bullous lessions Photosensivity

Cutaneous

Subacute cutaneous lupus 50 % of affected patients have SLE 10 % patients with SLE have this type of skin lesion

Renal

Renal involvement is common Abnormal urinalysis

Proteinuria Hematuria Pyuria

Renal

Tubulointerstitial nephritis Vascular disease Renal disease infrequently associated with drug-induced lupus

Renal involvement WHO Classification

Class I - Minimal mesangial lupus nephritis Class II - Mesangial proliferative lupus nephritis Class III - Focal lupus nephritis Class IV - Diffuse lupus nephritis Class V - Membranous lupus nephritis Class VI -Advanced sclerosing lupus nephritis

Cardiac

Pericarditis Myocarditis Endocarditis Atherosclerosis MI

Pulmonary involvement

Pleuritis Pulmonary infiltrates Interstitial inflammation Alveolar hemorrhage

Neurologic

Cognitive disorders Headache Mood disorder Stroke/TIA

Seizure Neuropathy Psychosis Aseptic meningitis Myelopathy

Hematologic

Anemia Hemolysis Leukopenia - lymphopenia Thrombocytopenia

Gastrointestinal

Peritonitis Vasculitis Pancreatitis Dysphagia

Treatment

NSAIDs Antimalarials (Hydroxychloroquine) Corticosteroids Immunosuppressive agents (Cyclophoshamide, Methotrexate, Azathioprene, Mycophenolate)

Antimalarials (Hydroxychloroquine)

Skin manifestations Musculoskeletal complaints Reduce the risk of disease flares Prevent major damage to the kidneys and central nervous system Not the drugs of choice for renal and CNS. The most common adverse reactions are related to the GI, skin, and CNS Ophthalmologic examination

Treatment
Dermatitis Minimize exposure to UV Topical glucocorticoids Antimalarial Retinoic acid Methotrexate and Azathioprene Topical tacrolimus/ Dapsone/Thalidomide Arthritis NSAIDS Antimalarial Corticosteroids IL-1 receptor antagonist, anakinara Methotrexate

Treatment
Systemic glucocorticoids + immunosuppressive agents (Cyclophosphamide) Significant organ involvementRenal Significant CNS Alveolar hemorrhage Systemic vasculitis

Thank You