Systemic lupus erythematosus: Difference between revisions

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== Definition ==
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==Definition==
   
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* Multisystem inflammatory disorder of unknown etiology
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*Multisystem inflammatory disorder of unknown etiology
   
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== ACR Classification Criteria ==
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==ACR Classification Criteria==
   
 
4 or more of the following (4RASHES mnemonic)
  
4 or more of the following (4RASHES mnemonic)
   
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* Four dermatologic manifestations
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*Four dermatologic manifestations
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** Malar rash: spares nasolabial folds
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**Malar rash: spares nasolabial folds
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** Discoid rash: erythemaous plaques/patches
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**Discoid rash: erythemaous plaques/patches
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** Photosensitivity: rash, not sunburn
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**Photosensitivity: rash, not sunburn
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** Mucocutaneous ulcers: oropharynx or nasopharynx, often painless
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**Mucocutaneous ulcers: oropharynx or nasopharynx, often painless
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* Renal disorder
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*Renal disorder
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** Proteinuria >0.5g/d or 3+
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**Proteinuria >0.5g/d or 3+
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** Any cellular or granular casts
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**Any cellular or granular casts
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* Arthritis: non-erosive involving 2 or more joints
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*Arthritis: non-erosive involving 2 or more joints
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* Serositis: pleuritis or pericarditis
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*Serositis: pleuritis or pericarditis
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* Hematologic disorder
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*Hematologic disorder
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** Hemolytic anemia
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**Hemolytic anemia
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** Leukopenia
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**Leukopenia
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** Lymphopenia: most important
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**Lymphopenia: most important
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** Thrombocytopenia
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**Thrombocytopenia
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* Neurological excitability
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*Neurological excitability
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** Seizures
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**Seizures
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** Psychosis
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**Psychosis
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* Serology (non-ANA)
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*Serology (non-ANA)
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** Anti-dsDNA antibodies
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**Anti-dsDNA antibodies
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** Anti-Sm antibodies
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**Anti-Sm antibodies
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** Anti-phospholipid antibodies
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**Anti-phospholipid antibodies
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* Serology (ANA): most sensitive finding (~98%)
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*Serology (ANA): most sensitive finding (~98%)
   
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== Clinical Presentation ==
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==Clinical Manifestations==
   
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* See above criteria
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*See above criteria
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* Fevers and other constitutional symptoms
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*Fevers and other constitutional symptoms
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* Any other non-specific rash, even if not in criteria
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*Any other non-specific rash, even if not in criteria
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** Discoid rash forms scars
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**Discoid rash forms scars
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* Alopecia, either discrete or global
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*Alopecia, either discrete or global
   
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== Investigations ==
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==Investigations==
   
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* Most important tests
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*Most important tests
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** CBC for lymphopenia & other hematologic abnormalities
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**CBC for lymphopenia & other hematologic abnormalities
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** Urinalysis for active sediment
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**Urinalysis for active sediment
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* Serology
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*Serology
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** ANA most sensitive, anti-Smith and anti-dsDNA are most specific
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**ANA most sensitive, anti-Smith and anti-dsDNA are most specific
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** APLA
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**APLA
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** Can be either seroconcordant or -discordant for dsDNA, C3/C4, ESR/CRP during flares
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**Can be either seroconcordant or -discordant for dsDNA, C3/C4, ESR/CRP during flares
   
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== Management ==
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==Management==
   
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=== Acute ===
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===Acute===
   
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* Prednisone 0.5-1mg/kg depending on severity
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*[[Prednisone]] 0.5-1 mg/kg depending on severity
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* Cyclophosphamide if severe or renal involvement
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*[[Cyclophosphamide]] if severe or renal involvement
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* vs. MMF
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**vs. [[MMF]]
   
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=== Chronic ===
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===Chronic===
   
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* Hydroxychloroquine
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*[[Hydroxychloroquine]]
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** Load with 400-600mg TDD split BID for 1-3 months
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**Load with 400-600 mg TDD split BID for 1-3 months
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** Step down to 6.75mg/kg maintenance
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**Step down to 6.75 mg/kg maintenance
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** Needs ophtho follow-up for rare macular side effect
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**Needs Ophthalmology follow-up for rare macular side effect
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** Best for skin
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**Best for skin
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* Azathioprine as steroid-sparing agent
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*[[Azathioprine]] as steroid-sparing agent
   
 
[[Category:Rheumatology]]
  
[[Category:Rheumatology]]

Latest revision as of 22:17, 2 August 2020

Definition

  • Multisystem inflammatory disorder of unknown etiology

ACR Classification Criteria

4 or more of the following (4RASHES mnemonic)

  • Four dermatologic manifestations
    • Malar rash: spares nasolabial folds
    • Discoid rash: erythemaous plaques/patches
    • Photosensitivity: rash, not sunburn
    • Mucocutaneous ulcers: oropharynx or nasopharynx, often painless
  • Renal disorder
    • Proteinuria >0.5g/d or 3+
    • Any cellular or granular casts
  • Arthritis: non-erosive involving 2 or more joints
  • Serositis: pleuritis or pericarditis
  • Hematologic disorder
    • Hemolytic anemia
    • Leukopenia
    • Lymphopenia: most important
    • Thrombocytopenia
  • Neurological excitability
    • Seizures
    • Psychosis
  • Serology (non-ANA)
    • Anti-dsDNA antibodies
    • Anti-Sm antibodies
    • Anti-phospholipid antibodies
  • Serology (ANA): most sensitive finding (~98%)

Clinical Manifestations

  • See above criteria
  • Fevers and other constitutional symptoms
  • Any other non-specific rash, even if not in criteria
    • Discoid rash forms scars
  • Alopecia, either discrete or global

Investigations

  • Most important tests
    • CBC for lymphopenia & other hematologic abnormalities
    • Urinalysis for active sediment
  • Serology
    • ANA most sensitive, anti-Smith and anti-dsDNA are most specific
    • APLA
    • Can be either seroconcordant or -discordant for dsDNA, C3/C4, ESR/CRP during flares

Management

Acute

Chronic

  • Hydroxychloroquine
    • Load with 400-600 mg TDD split BID for 1-3 months
    • Step down to 6.75 mg/kg maintenance
    • Needs Ophthalmology follow-up for rare macular side effect
    • Best for skin
  • Azathioprine as steroid-sparing agent
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