SYSTEMIC LUPUS ERTHEMATOSUS (SLE)

Soumya Nath Maiti

SLE is the classical example of systemic auto immune or collagen diseases

Classification 1. Systemic form – Acute and chronic

inflammatory lesions widely scattered in the body

2. Discoid form – Chronic and localized skin lesions involving the bridge of nose and adjacent cheeks

Etiology

1) Antinuclear antibodies (ANA) are the antibodies against common nuclear antigen that includes DNA as well as RNA

2) Antibodies to double stranded DNA most specific for SLE

3) Anti smith Antibodies act against smith antigens which is part of ribonucleoproteins

4) Other non specific antibodies – Anti RNP, Anti Histone, Antiphospholipid

The source of these autoantibodies as well hyper gammaglobulinaemia seen in SLE is the polyclonal activation of B cells brought about by following derangements

1. Immunologic factors–B cell or T cell defect

2. Genetic factors-Class II HLA gene defect

3. Other factors-Drugs, Viral infections, Hormones

Pathogenesis

The auto antibodies formed causes tissue injury.

Two types of immunologic tissue injury can occur in SLE

Type II hypersensitivity is characterized by formation of auto antibodies against blood cells Type III hypersensitivity is characterized by antigen antibody complex

Stages

Stage I – Immune insufficiency Stage II – Hyperactivation of T and B cells Stage III – Invasion of tissues by T and B cells Stage IV – Active disease Stage V – Organ destruction

Clinical features

Arthritis and arthalgia --- Joint manifestations occur in 90 % of subjects Large joints are affected Jaccoud’s arthopathy may affect the hand in upto 50 % cases

Skin lesions— These occur in 65% of cases. The classic lesion is the erytheamatous photosensitive butterfly rash affecting the cheeks and nose. Frontal baldness Discoid lupus Ulcers in mouth and pharynx

Cardiovascular lesions Develop in 25 – 40% cases Cardiac lesions include pericarditis, pericardial effusion, myocarditis etc Libman sacks endocarditis is occasionally seen Vasculitis leads to Raynaud’s phenomenon, necrotic ulcers of the finger pulp

Respiratory system 30 % cases Common lesions are dry

pleurisy, pleural effusion , fibrosing alveolitis and lupus pneumonitis

Kidney 60 % cases Renal involvement includes

Nephrotic syndrome , haemturia, Acute nephritic syndrome

Nervous system Affected in 40 % cases Neurological manifestation

usually is a late manifestation Lesions are caused due to

vasculitis Includes stroke, psychosis,

convulsions, cranial nerve palsies, peripheral neuropathy.

Muscular system Myalgia, Muscle wasting,

Eye Occulr involvement in SLE

may arise from embolic lesions of libman sacks endocarditis or vascular occlusions resulting from the hypercoagubility associated with lupus anticoagulants

Retinal vascular disease including retinal arterial or venous occlusion and Retinal haemorroage or retinal oedema may be seen

SLE may also cause optic neuropathy with reversible or irreversible visual loss.

 Choroidopathy is uncommon as a clinical manifestation but may be apparent fluorescein angiography

Occlusive vasculopathy has decreased in incidence due to improved management .

Management

There is no cure for SLE. The goal of treatment is to control symptoms.

Mild disease may be treated with: ---- Nonsteroidal anti-inflammatory medications

(NSAIDs) treat arthritis and pleurisy Corticosteroid creams to treat skin rashes An antimalarial drug (hydroxychloroquine) and

low-dose corticosteroids for skin and arthritis symptoms

Intravenous Immunoglobulins

Severe or life-threatening symptoms (such as hemolytic anemia, extensive heart or lung involvement, kidney disease, or central nervous system involvement) often require more aggressive treatment. Treatment for more severe lupus may include :-

High-dose corticosteroids or medications to decrease the immune system response

Cytotoxic drugs

Thank you