Systemic lupus erythematosus: Difference between revisions

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== Definition ==
==Definition==


* Multisystem inflammatory disorder of unknown etiology
*Multisystem inflammatory disorder of unknown etiology


== ACR Classification Criteria ==
==ACR Classification Criteria==


4 or more of the following (4RASHES mnemonic)
4 or more of the following (4RASHES mnemonic)


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


== Clinical Manifestations ==
==Clinical Manifestations==


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


== Investigations ==
==Investigations==


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


== Management ==
==Management==


=== Acute ===
===Acute===


* Prednisone 0.5-1mg/kg depending on severity
*[[Prednisone]] 0.5-1 mg/kg depending on severity
* Cyclophosphamide if severe or renal involvement
*[[Cyclophosphamide]] if severe or renal involvement
* vs. MMF
**vs. [[MMF]]


=== Chronic ===
===Chronic===


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


[[Category:Rheumatology]]
[[Category:Rheumatology]]

Latest revision as of 02:17, 3 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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