Systemic lupus erythematosus: Difference between revisions
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− | == |
+ | ==Definition== |
− | * |
+ | *Multisystem inflammatory disorder of unknown etiology |
− | == |
+ | ==ACR Classification Criteria== |
4 or more of the following (4RASHES mnemonic) |
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=== |
− | * |
+ | *[[Prednisone]] 0.5-1 mg/kg depending on severity |
− | * |
+ | *[[Cyclophosphamide]] if severe or renal involvement |
− | * |
+ | **vs. [[MMF]] |
− | === |
+ | ===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 |
[[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
- Prednisone 0.5-1 mg/kg depending on severity
- Cyclophosphamide if severe or renal involvement
- vs. MMF
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