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Systemic Lupus Erythematous

Lale Ertuglu

Background

  • Characterized autoantibody formation to nuclear material and other cellular antigens, resulting in activation of complement and multisystem inflammation
  • More common in women, age of onset usually 16-55

Presentation:

  • Constitutional: fatigue (most common complaint), fevers, myalgia, weight loss
  • Joints: arthralgias and arthritis (usually polyarticular, symmetric, migratory and nonerosive), Raynaud’s.
  • Mucocutaneous: malar rash, discoid skin lesions, photosensitivity, painless oral (usually palatal) ulcers, nasal ulcers, scarring alopecia from discoid lupus is specific (vs non-scarring diffuse alopecia which is common)
  • Cardiac: pericarditis (~25% of pts will develop at some point in disease course), verrucous (Libman-sacks) endocarditis, myocarditis, increased risk CAD
  • Hematologic: anemia of chronic disease (most common), leukopenia, ITP, AIHA
  • Renal: Lupus nephritis is the most common organ-threatening manifestation, can be refractory to therapy. Diagnosed and classified with renal biopsy.
  • Renal biopsy needed when Cr is rising, or there is persistent proteinuria or hematuria in UA
  • Class I and II are usually clinically silent. Class III and IV typically present as nephritic syndrome, class V mostly presents as nephrotic syndrome. Most patients present as an overlap (such as III + V or IV +V)
  • Pulmonary: pleuritis (if chronic may be complicated by shrinking lung syndrome), pleural effusion, ILD, pHTN
  • Neurologic: stroke, cerebritis, psychosis, mononeuritis multiplex
  • Ophtho: keratoconjunctivitis sicca (2/2 Sjogren’s syndrome)
  • GI: dysphagia due to esophageal dysmotility, intestinal pseudo-obstruction, elevation of LFTs (significant liver disease is rare)

Evaluation

  • Labs: CBC, BMP, UA with sediment, Ur Pr:Cr ratio, inflammatory markers (nonspecific), ANA (high sensitivity), anti-dS DNA, anti-Smith, complement levels (C3 & C4 usually low)
  • Consider antiphospholipid antibody testing (lupus anticoagulant, anti-cardiolipin, anti- β2 glycoprotein)
Clinical Criteria Laboratory Criteria
Constitutional Weight: 2 Antiphospholipid antibodies (Lupus AC, CL, β2GP1) Weight: 2
Hematologic
-Leukopenia
-Thrombocytopenia
-Autoimmune hemolysis		 Weight:
3
4
4		 Complement proteins
-Low C3 OR C4
-Low C3 AND C4		 Weight:
3
4
Neuropsychiatric
-Delirium
-Psychosis
-Seizure		 Weight:
2
3
4		 SLE-specific antibodies
-Anti-dsDNA OR Anti-Smith		 Weight: 6
Mucocutaneous
-Non-scarring alopecia
-Oral ulcers
-Subacute cutaneous OR discoid lupus
-Acute cutaneous lupus		 Weight:
2
2
4
6
Serosal
-Pleural or pericardial effusion
-Acute pericarditis		 Weight:
5
6
Renal
-Proteinuria (>0.5g/24h)
-Renal Bx Class II or V lupus nephritis
-Renal Bx Class III or IV lupus nephritis		 Weight:
4
8
10

Management

  • HCQ is the first line treatment: 200-400mg/day (5mg/kg/d)
    • Need retinal screening at baseline and annually after 5 years of therapy
    • Not immunosuppressive
    • Safe in pregnancy with improved pregnancy outcomes and reduced neonatal lupus
  • Glucocorticoids: ideally short-term. High doses may be used for life or organ threatening disease
  • Other therapies: MTX, MMF, AZA, RTX, belimumab, anifrolumab
  • Renal disease
    • Immunosuppression: MMF, cyclophosphamide, belimumab, voclosporin – mostly used in combination with mycophenolate
    • Renal protection: ACEi or ARB