Introduction
Systemic lupus erythematosus (SLE) is a multisystemic auto immune disorder with a broad spectrum of manifestations.
In systemic lupus erythematosus condition, the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs
- It affects all organs and systems.
- It presents as a chronic disease with a waxing and waning course
- It presents with significant morbidity and possible mortality
- It affects mainly African Americans and Hispanic
- Affects mostly females of child bearing age [16-55yrs]
- Early damage due to the disease; late disease due to infections and atherosclerosis.
Causes & pathogenesis of systemic lupus erythematosus
- No definite aetiology, but genetic and environment factors implicated
- Female sex
- Genetic factors – commoner in
monozygotic twins - Epigenetic factors
- Environmental – ultraviolet rays,
- Drugs; e.g. procainamide, hydralazine, methyldopa
- Viral (e.g.Epstein-Barr virus (EBV);
- Oral drugs such as oral contraceptives
Symptoms and clinical features of systemic lupus erythematosus
The following are the common symptoms or clinical features of SLE:
- systemic lupus erythematosus affects all organs and systems – skin, joints, heart, lungs, kidneys, brain,
- It is diagnosis mostly by American College of Rheumatology (ACR) Criteria and lately, Systemic Lupus international collaborating clinics [SLICC] criteria
- It usually presents with fever, polyarthralgia, fatigue, loss of weight, Hair loss, skin rashes, mouth or pharyngeal ulcers.
- Specific organ involvement:
- Lung – pleurisy, pleural effusion, pulmonary fibrosis
- Heart – Pericarditis, pericardial effusion, ischemic heart disease
- Kidneys – Proteinuria, acute and chronic renal failure, nephrotic syndrome
- CNS – meningitis, encephalitis, seizure, psychosis
- Blood – Hemolytic anaemia, leucopenia, thrombocytopenÃa
- Pregnancy – Antiphospholipid syndrome recurrent pregnancy losses, intra-uterine growth retardation
Differential Diagnosis
- Rheumatoid Arthritis
- Scleroderma, inflammatory myopathies
- Fibromyalgia
- Benign Hypermobility Syndrome
Investigations
- Haematocrit, white blood cell count,
platelet count - ESR, CRP
- Urine Analysis and microscopy – for casts, RBCs, protein
- Electrolytes and urea
- Chest X-ray
- ECG
- Serology: Anti-nuclear Antibody (ANA); Extractable Nuclear Antigen (ENA); Double stranded DNA
- Kidney Biopsy – as indicated
Management of systemic lupus erythematosus
Corticosteroids
- Prednisolone 1-2mg/kg orally daily
- Anti-malarial – Hydroxychloroquine 200mg-400mg daily
- Azathioprine – 2mg-3mg/kg body
- Mycophenolate 1gm to 3gm daily
- Cyclosporine
- Cyclophosphamide
- Biologic DMARDS – Rituximab,
Non-pharmacologic
Non Pharmacological approach used in the management of SLE include the following:
- Avoid sunlight
- Avoid physical and emotional stress
- Physical Exercise
- Use sunscreen