Rheumatology

Systemic Lupus Erythematosus

전신홍반루푸스

Multi-system autoimmune disease with diverse manifestations

How common
0.05–0.1% (9:1 female)
Typical age
Childbearing age 15–45

What is it?

Genetic predisposition and environmental triggers (UV, estrogen, EBV) activate autoimmunity to nuclear antigens; immune complexes deposit in multiple organs causing inflammation.

Commonly affected: Skin, joints, kidney, blood, CNS, heart, lungs (multi-organ)

How it develops

  1. Defective apoptosis clearanceIncreased self-antigen exposure stimulates immune system
  2. Autoantibody productionAnti-dsDNA, anti-Sm, anti-Ro/La produced
  3. Immune complex depositionDeposits in glomeruli, skin, joints, lung, heart
  4. Complement & multi-organ damageC3/C4 consumption, systemic inflammation and organ damage

Symptoms

  • Lupus nephritisProteinuria, hematuria, renal failure
  • Malar (butterfly) rashPhotosensitive erythema across malar areas, sparing nasolabial folds
  • ArthritisNon-erosive symmetric polyarthritis
  • HematologicHemolytic anemia, leukopenia, thrombocytopenia
  • Oral ulcers/alopeciaPainless oral/nasal ulcers, hair loss

How it is examined

  • ACR/EULAR 2019 criteriaANA entry, weighted scoring across domains ≥10
  • Autoantibody panelANA (96%), anti-dsDNA, anti-Sm, anti-Ro/La, antiphospholipid
  • Renal evaluationUA, quantitative proteinuria, biopsy (class I–VI)

Imaging

Joint X-rays show non-erosive changes (Jaccoud: reversible deformity).

  • Non-erosive joint changes
  • Jaccoud arthropathy

Brain MRI for neuro-lupus, echo for pericardial effusion.

  • White matter lesions
  • Pericardial effusion
  • Synovitis

Non-surgical care

  • Hydroxychloroquine (all patients)Foundation therapy; reduces flares and improves survival
  • Low–moderate corticosteroidsShort-term for active disease
  • ImmunosuppressantsMMF, azathioprine, methotrexate
  • Photoprotection/SPFUV triggers rash and systemic flares
  • CV risk managementBP, lipids, smoking (accelerated atherosclerosis)

Conservative options are generally tried first. Medications listed here can have side effects — discuss them with your prescriber.

When surgery is considered

Severe organ involvement (nephritis, neuro-lupus)

Procedures that may be discussed

  • IV cyclophosphamide pulse
  • Mycophenolate mofetil
  • Belimumab (anti-BLyS)
  • Rituximab (anti-CD20)
  • Anifrolumab (anti-IFN-α receptor)

Outlook

5-year survival >90%, 10-year 80–85%. Renal, CNS, infection, CV complications drive mortality.

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