Systemic Lupus Erythematosus treatment guideline

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

  1. It affects all organs and systems.
  2. It presents as a chronic disease with a waxing and waning course
  3. It presents with significant morbidity and possible mortality
  4. It affects mainly African Americans and Hispanic
  5. Affects mostly females of child bearing age [16-55yrs]
  6. Early damage due to the disease; late disease due to infections and atherosclerosis.

Causes & pathogenesis of systemic lupus erythematosus

  1. No definite aetiology, but genetic and environment factors implicated
  2. Female sex
  3. Genetic factors – commoner in
    monozygotic twins
  4. Epigenetic factors
  5. Environmental – ultraviolet rays,
  6. Drugs; e.g. procainamide, hydralazine, methyldopa
  7. Viral (e.g.Epstein-Barr virus (EBV);
  8. 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:

  1. systemic lupus erythematosus affects all organs and systems – skin, joints, heart, lungs, kidneys, brain,
  2. It is diagnosis mostly by American College of Rheumatology (ACR) Criteria and lately, Systemic Lupus international collaborating clinics [SLICC] criteria
  3. It usually presents with fever, polyarthralgia, fatigue, loss of weight, Hair loss, skin rashes, mouth or pharyngeal ulcers.
  4. 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, thrombocytopenía
    • Pregnancy – Antiphospholipid syndrome recurrent pregnancy losses, intra-uterine growth retardation

Differential Diagnosis

  1. Rheumatoid Arthritis
  2. Scleroderma, inflammatory myopathies
  3. Fibromyalgia
  4. 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

  1. Prednisolone 1-2mg/kg orally daily
  2. Anti-malarial – Hydroxychloroquine 200mg-400mg daily
  3. Azathioprine – 2mg-3mg/kg body
  4. Mycophenolate Mofetil-1gm-3gm daily
  5. Cyclosporine
  6. Cyclophosphamide
  7. Biologic DMARDS – Rituximab,

Non-pharmacologic

Non Pharmacological approach used in the management of SLE include the following:

  1. Avoid sunlight
  2. Avoid physical and emotional stress
  3. Physical Exercise
  4. Use sunscreen