Systemic Lupus Erythematosus

Notes

## Basic introduction

  • Systemic Lupus Erythematosus (SLE) is a chronic inflammatory disease that has varied presentations that follow a relapsing and remitting course[b]
  • Over 30 different genes predispose to SLE[d]

## Classification of LE

  • Drug-induced LE
  • Only 1% of cases persist after the offending medicine has been stopped
  • The common drugs that are associated with LE include;

|||Diltiazem

|||Hydralazine

|||Isoniazid

|||Minocycline

|||Procainamide

|||Quinidine

  • Neonatal LE
  • This is caused by the passive transfer of autoantibodies from the mother.
  • Babies (5-7%) have transient rashes that can last for 6-8 months
  • Some babies (2%) will suffer from cardiac disorders
  • Cutaneous LE (CLE)
  • CLE is classified into 3 main categories;

|||Acute CLE

|||Sub acute CLE

|||Chronic CLE

  • Chronic CLE is further divided into 3 classes;

       |||Discoid LE (DLE)

       |||Tumid lupus

       |||Lupus panniculitis

  • SLE

## Pathophysiology of LE

  • There is production of autoantibodies
  • These autoantibodies form immune complexes that are deposited on the endothelia causing inflammation and tissue damage

## Similarities between LE and Systemic sclerosis (SS or scleroderma), both are;

  • Associated with genetic predisposition
  • Autoimmune diseases
  • More common in women than men
  • Characterized by the manifestations of common symptoms such as;
  • Esophageal dysfunction (but more common in SS)
  • Raynaud’s syndrome (but more common in SS)
  • Swollen joints (but more common in LE)
  • The presence of Antinuclear antibodies (ANA)

## Differences between LE and SS;

  • Anti-Scl 70 anti-centromere antibody are found only in SS
  • SLE is more affected by the genetic factors than SS
  • Sun sensitivity is found only in LE
  • The age of onset is 15-45yrs for LE and 20-60yrs for SS
  • The pathology of LE is mainly an inflammatory process while that of SS involves inflammation, cross-linking of collagen that leads to tight skin, fibrosis or scarring of tissues, and vascular endothelial changes.

## Statistics

  • > 90% of cases of SLE occur in women
  • Globally, the prevalence of SLE in the generally population is 30 to 50/100,000[e]
  • Incidence of SLE in USA:18 - 76 per million persons / year [a]
  • The female predominance of SLE in a study in Kenya was 97%[b]
Symptoms
  • Non-specific symptoms and signs
  • Fatigue
  • Weight loss
  • Malaise
  • Fever
  • Anorexia
  • Anemia
  • Hair loss
  • Articular diseases
  • These are the commonest clinical manifestations of SLE (present in 90% of cases in a study in Kenya)[b]
  • The symptoms include: arthritis (joint pains and swellings) that mainly affect the joints of the fingers, hands, wrists, and knees
  • Skin manifestations
  • Malar rash or butterfly rash or cheekbone rash (with a prevalence of 69.2% in Kenya)[c]
  • Photosensitivity
  • Discoid rashes
  • Oral ulcers
  • Neurological disease
  • Neuropathies
  • Stroke
  • Psychosis
  • Seizures
  • Renal diseases
  • Glomerulonephritis is the leading cause of death among SLE
  • Renal diseases affect 50-70% of SLE
Diagnosis
  • Clinical review
  • Antinuclear antibody (ANA) assay
  • Anti - dsDNA
  • ESR and CRP (are elevated)
  • Urinalysis
  • Complete Blood Count with differential
  • Complements C3 and C4 are low
  • LFTs are elevated
Differential
  • Acrodermatitis chronicum atrophicans
  • Behçet’s syndrome
  • Dermatomyositis and polymyositis
  • Fibromyalgia
  • Infections such as EBV and CMV
  • Kikuchi’s disease
  • Mixed connective tissue disease
  • Primary Raynaud's phenomenon
  • Rheumatoid arthritis (RA)
  • Rhupus (overlapping features of both SLE and RA)
  • Serum sickness
  • Sjögren’s syndrome
  • Systemic sclerosis or scleroderma
  • Vasculitis
Prevention
  • It is not possible to prevent LE but the following factors can reduce its incidence;
  • Avoiding stress
  • Avoiding sunshine exposure
  • Exercise
  • Good sleep hygiene
  • Healthy diet
  • Smoking cessation
  • Sun-protective clothing
  • Use of the Sun Protective Factor (SPF)
Reference

1. Centres for Disease Control and Prevention. Systemic lupus erythematosus (SLE or lupus). Available at http://www.cdc.gov/arthritis/basics/lupus.htm/#2.

  1. Genga, E. K., Shiruli, B. C., Odhiambo, J., Jepkorir, S., Omondi, E. A., Otieno, F. O., & Oyoo, G. O. (2015). Clinical Characteristics of Patients with Systemic Lupus Erythematosus in Nairobi, Kenya. African Journal of Rheumatology, 3(2), 62-66
  2. Ekwom PE. Systemic lupus erythematosus (SLE) at the Kenyatta National Hospital. Clin Rheumatol. 2013; 32(8):1215-1217.
  3. Ramos, P. S., Brown, E. E., Kimberly, R. P., & Langefeld, C. D. (2010, March). Genetic factors predisposing to systemic lupus erythematosus and lupus nephritis. In Seminars in nephrology (Vol. 30, No. 2, pp. 164-176). WB Saunders.
  4. Osio-Salido, E., and H. Manapat-Reyes. "Epidemiology of systemic lupus erythematosus in Asia." Lupus 19, no. 12 (2010): 1365-1373.
Management
  • Immunosuppressants;
  • Azathioprine
  • Belimumab
  • Cyclophosphamide
  • Cyclosporine
  • Dapsone
  • Leflunomide
  • Methotrexate
  • Mycophenylate Mofetil
  • Rituximab
  • Corticosteroids (mainly prednisolone)
  • Antimalarials
  • Hydroxychloroquine (the most commonly used drug for the treatment of SLE in Africa, and Kenya) [b][c]
  • Chloroquine
  • Quinacrine
  • NSAIDS
  • Belimumuab
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