Systemic lupus erythematosus: Difference between revisions
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==Clinical Features==
==Clinical Features==
'''SLICC Classification Criteria 2012''' <ref>Lisnevskaia L, et al. Systemic Lupus Erythematosus. Lancet. 2014 May 29. Epub ahead of print.</ref>
[[File:Lupusfoto.jpg|thumb|Typical "butterfly" malar rash.]]
Requirements: >4 of the following criteria (at least 1 clinical and 1 laboratory) '''OR''' biopsy proven lupus nephritis with  
[[File:PMC3410306 AD2012-834291.004.png|thumb|Palatal ulcer in SLE]]
[[File:PMC3410306 AD2012-834291.005.png|thumb|Subacute cutaneous SLE]]
SLICC Classification Criteria 2012 <ref>Lisnevskaia L, et al. Systemic Lupus Erythematosus. Lancet. 2014 May 29. Epub ahead of print.</ref>
Requirements: >4 of the following criteria (at least 1 clinical and 1 laboratory) OR biopsy proven lupus nephritis with  
positive ANA or Anti-dsDNA
positive ANA or Anti-dsDNA
*Clinical criteria
*Clinical criteria
**Malar rash, bullous lupus, photosensitivity
**Malar [[rash]], bullous lupus, photosensitivity
**Discoid rash, hypertrophic lupus
**Discoid rash, hypertrophic lupus
**Oral ulcers or nasal ulcers
**Oral ulcers or nasal ulcers
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**Synovitis
**Synovitis
**Serositis
**Serositis
**Nephritis
**[[glomerulonephritis|Nephritis]]
**Cerebritis, myelitis, neuropathy
**Cerebritis, myelitis, neuropathy
**Hemolytic anemia
**[[Hemolytic anemia]]
**Leukopenia or lymphopenia
**[[Leukopenia]] or lymphopenia
**Thrombocytopenia
**[[Thrombocytopenia]]


*Immunological criteria
*Immunological criteria
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**Direct Coombs' test in the absence of haemolytic anaemia
**Direct Coombs' test in the absence of haemolytic anaemia


'''Organ system affected:'''
Organ system affected:
*Cardiopulmonary
*Cardiopulmonary
**Pneumonia
**[[Pneumonia]]
***Cover for ''[[Listeria]]'' and ''[[Pseudomonas]]''
***Cover for ''[[Listeria]]'' and ''[[Pseudomonas]]''
**CAD
**CAD
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***Infectious and Libman-Sachs
***Infectious and Libman-Sachs


*Neuropsychiatric/Altered mental status
*Neuropsychiatric/[[Altered mental status]]
**Non-convulsive status epilepticus
**Non-convulsive [[status epilepticus]]
**CNS vasculitis
**CNS [[vasculitis]]
**[[Stroke]]
**[[Stroke]]
**[[Encephalitis]]
**[[Encephalitis]]
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*Musculoskeletal
*Musculoskeletal
**Arthritis
**[[Arthritis]]
***Usually symmetric
***Usually symmetric
***Consider septic arthritis if there is a single inflamed joint
***Consider [[septic arthritis]] if there is a single inflamed joint
****Cover for [[Salmonella]] in addition to standard coverage
****Cover for [[Salmonella]] in addition to standard coverage


*GI
*GI
**Lupus enteritis (mesenteric vasculitis)
**Lupus enteritis (mesenteric [[vasculitis]])
***Most common cause of acute abdominal pain
***Most common cause of acute [[abdominal pain]]
**[[Pancreatitis]]
**[[Pancreatitis]]
**PUD
**[[PUD]]


*Dermatologic
*Dermatologic
**Malar rash across bridge of nose
**Malar [[rash]] across bridge of nose
**Discoid rash, erythematous with scale
**Discoid rash, erythematous with scale
**Treat with topical 1% hydrocortisone


*Renal
*Renal
**Usually a nephritis
**Usually a [[glomerulonephritis|nephritis]]
**Can cause a glomerulonephrosis
**Can cause a glomerulonephrosis


==Differential Diagnosis==
==Differential Diagnosis==
*[[Rheumatoid arthritis]]
*[[Rheumatoid arthritis]]
*[[Sjogren's syndrome]]
*[[Sjögren Syndrome]]
*[[Dermatomyositis]]
*[[Dermatomyositis]]
*[[Polymyositis]]
*[[Polymyositis]]
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*[[Septic Arthritis]]
*[[Septic Arthritis]]
*[[Lyme Disease]]
*[[Lyme Disease]]
*Vasculitis
*[[Vasculitis]]
*[[Acute Rheumatic Fever]]
*[[Acute Rheumatic Fever]]
*[[Toxic Shock Syndrome]]
*[[Toxic Shock Syndrome]]
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==Evaluation==
==Evaluation==
'''Undiagnosed'''
Undiagnosed
*CBC
*CBC
*Chem 10
*Chem 10
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*ANA
*ANA
*ESR
*ESR
*UA
*[[Urinalysis]]
*Bedside echo if ill or hypotensive
*Bedside [[echocardiography]] if ill or hypotensive
*(Consider anti-DNA, anti-Smith, anti-Nuclear, anti-phospholipid, C3,C4, direct Coombs')
*(Consider anti-DNA, anti-Smith, anti-Nuclear, anti-phospholipid, C3,C4, direct Coombs')


'''Flare'''
Flare
*Bedside echo if ill or hypotensive
*Bedside echo if ill or hypotensive
*CBC
*CBC
*Chem
*Chem
*UA
*[[Urinalysis]]
*Urine pregnancy
*Urine pregnancy
*As directed by organ system involved
*As directed by organ system involved


'''Drug Induced Lupus'''
Drug Induced Lupus
*Anti-histone-Ab positive 95% of the time
*Anti-histone-Ab positive 95% of the time
*Make sure to review medications
*Make sure to review medications
**High risk:
**High risk:
***Procainamide (antiarrhythmic)
***[[Procainamide]] (antiarrhythmic)
***Hydralazine (antihypertensive)
***[[Hydralazine]] (antihypertensive)
**Moderate to low risk:
**Moderate to low risk:
***Infliximab anti (TNF-α)
***Infliximab anti (TNF-α)
***Etanercept anti (TNF-α)
***Etanercept anti (TNF-α)
***Isoniazid (antibiotic)
***[[Isoniazid]] (antibiotic)
***Minocycline (antibiotic)
***[[Minocycline]] (antibiotic)
***Pyrazinamide (antibiotic)
***[[Pyrazinamide]] (antibiotic)
***Quinidine (antiarrhythmic)
***[[Quinidine]] (antiarrhythmic)
***D-Penicillamine (anti-inflammatory)
***D-[[Penicillamine]] (anti-inflammatory)
***Carbamazepine (anticonvulsant)
***[[Carbamazepine]] (anticonvulsant)
***Oxcarbazepine (anticonvulsant)
***[[Oxcarbazepine]] (anticonvulsant)
***Phenytoin (anticonvulsant)
***[[Phenytoin]] (anticonvulsant)
***Propafenone (antiarrhythmic)
***Propafenone (antiarrhythmic)
***Chlorpromazine (antipsychotic)
***[[Chlorpromazine]] (antipsychotic)


===Fever in SLE===
===[[Fever]] in SLE===
*Must differentiate disease activity (flare) from infection
*Must differentiate disease activity (flare) from infection


'''Risk Factors for Infection''' <ref>Cuchacovich, R., & Gedalia, A. (2009). Pathophysiology and clinical spectrum of infections in systemic lupus erythematosus. Rheumatic diseases clinics of North America, 35(1), 75–93. doi:10.1016/j.rdc.2009.03.003</ref>
Risk Factors for Infection <ref>Cuchacovich, R., & Gedalia, A. (2009). Pathophysiology and clinical spectrum of infections in systemic lupus erythematosus. Rheumatic diseases clinics of North America, 35(1), 75–93. doi:10.1016/j.rdc.2009.03.003</ref>
*Neutropenia/Lymphopenia
*[[Neutropenia]]/Lymphopenia
*Hypocomplementemia
*Hypocomplementemia
*Immunosuppressive therapy (especially Azathioprine <ref>Zhou, W. J., & Yang, C.-D. (2009). The causes and clinical significance of fever in systemic lupus erythematosus: a retrospective study of 487 hospitalised patients. Lupus, 18(9), 807–812. doi:10.1177/0961203309103870</ref>)
*Immunosuppressive therapy (especially [[azathioprine]] <ref>Zhou, W. J., & Yang, C.-D. (2009). The causes and clinical significance of fever in systemic lupus erythematosus: a retrospective study of 487 hospitalised patients. Lupus, 18(9), 807–812. doi:10.1177/0961203309103870</ref>)


'''Studies'''
Studies
*CRP: sensitivity 100%, specificity 90% >1.35mg/dL <ref>Kim, H.-A., Jeon, J.-Y., An, J.-M., Koh, B.-R., & Suh, C.-H. (2012). C-reactive protein is a more sensitive and specific marker for diagnosing bacterial infections in systemic lupus erythematosus compared to S100A8/A9 and procalcitonin. The Journal of rheumatology, 39(4), 728–734. doi:10.3899/jrheum.111044</ref>
*CRP: sensitivity 100%, specificity 90% >1.35mg/dL <ref>Kim, H.-A., Jeon, J.-Y., An, J.-M., Koh, B.-R., & Suh, C.-H. (2012). C-reactive protein is a more sensitive and specific marker for diagnosing bacterial infections in systemic lupus erythematosus compared to S100A8/A9 and procalcitonin. The Journal of rheumatology, 39(4), 728–734. doi:10.3899/jrheum.111044</ref>
*PCT: sensitivity 75%, specificity 75% <ref>Scirè, C. A., Cavagna, L., Perotti, C., Bruschi, E., Caporali, R., & Montecucco, C. (2006). Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune diseases. Clinical and experimental rheumatology, 24(2), 123–128.</ref>
*PCT: sensitivity 75%, specificity 75% <ref>Scirè, C. A., Cavagna, L., Perotti, C., Bruschi, E., Caporali, R., & Montecucco, C. (2006). Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune diseases. Clinical and experimental rheumatology, 24(2), 123–128.</ref>
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==Management==
==Management==
*Inflammatory complications
*Inflammatory complications
**Methylprednisolone 1-2mg/kg in most cases
**[[Methylprednisolone]] 1-2mg/kg in most cases
*Infectious
*Infectious
**Stress dose steroids with hydrocortisone 100mg IV Q8hr if on or recently on steroids
**Stress dose [[steroids]] with [[hydrocortisone]] 100mg IV Q8hr if on or recently on steroids
*Dermatologic
*Dermatologic
**Hydrocortisone 1% cream
**[[Hydrocortisone]] 1% cream
*If drug induced lupus, stop medication and consider alternative
*If drug induced lupus, stop medication and consider alternative


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*Mild flairs can have expedited out patient management
*Mild flairs can have expedited out patient management
*Musculoskeletal symptoms can usually be managed as out patients
*Musculoskeletal symptoms can usually be managed as out patients
*Chest pain requires urgent ACS evaluation
*[[Chest pain]] requires urgent [[ACS]] evaluation
*Infections usually require admission for antibiotics and systemic corticosteroids
*Infections usually require admission for [[antibiotics]] and systemic [[corticosteroids]]
 
 
==Medication Dosing==
{{MedicationDose
| drug = Methylprednisolone
| dose = 1-2mg/kg IV
| route = IV
| context = Acute SLE flare management
| indication = Systemic lupus erythematosus
| population = Adult
}}


==See Also==
==See Also==

Latest revision as of 09:37, 22 March 2026

Background

  • Autoimmune disorder affecting all systems
  • Also consider drug induced lupus

Epidemiology

  • Female:Male 10:1
  • Peak in 20s-30s
  • More common in Black patients

Clinical Features

Typical "butterfly" malar rash.
Palatal ulcer in SLE
Subacute cutaneous SLE

SLICC Classification Criteria 2012 [1] Requirements: >4 of the following criteria (at least 1 clinical and 1 laboratory) OR biopsy proven lupus nephritis with positive ANA or Anti-dsDNA

  • Immunological criteria
    • ANA
    • Anti-dsDNA
    • Anti-Sm
    • Antiphospholipid antibody
    • Low complement C3, low C4
    • Direct Coombs' test in the absence of haemolytic anaemia

Organ system affected:

  • Dermatologic
    • Malar rash across bridge of nose
    • Discoid rash, erythematous with scale
  • Renal
    • Usually a nephritis
    • Can cause a glomerulonephrosis

Differential Diagnosis

Polyarthritis

Algorithm for Polyarticular arthralgia

Causes of Glomerulonephritis

Evaluation

Undiagnosed

  • CBC
  • Chem 10
  • Urine pregnancy
  • ANA
  • ESR
  • Urinalysis
  • Bedside echocardiography if ill or hypotensive
  • (Consider anti-DNA, anti-Smith, anti-Nuclear, anti-phospholipid, C3,C4, direct Coombs')

Flare

  • Bedside echo if ill or hypotensive
  • CBC
  • Chem
  • Urinalysis
  • Urine pregnancy
  • As directed by organ system involved

Drug Induced Lupus

Fever in SLE

  • Must differentiate disease activity (flare) from infection

Risk Factors for Infection [2]

Studies

  • CRP: sensitivity 100%, specificity 90% >1.35mg/dL [4]
  • PCT: sensitivity 75%, specificity 75% [5]

Management

Disposition

  • Suspected new diagnosis can have out patient workup if well appearing
  • Mild flairs can have expedited out patient management
  • Musculoskeletal symptoms can usually be managed as out patients
  • Chest pain requires urgent ACS evaluation
  • Infections usually require admission for antibiotics and systemic corticosteroids


Medication Dosing

Methylprednisolone 1-2mg/kg IV IV

See Also

References

  1. ↑ Lisnevskaia L, et al. Systemic Lupus Erythematosus. Lancet. 2014 May 29. Epub ahead of print.
  2. ↑ Cuchacovich, R., & Gedalia, A. (2009). Pathophysiology and clinical spectrum of infections in systemic lupus erythematosus. Rheumatic diseases clinics of North America, 35(1), 75–93. doi:10.1016/j.rdc.2009.03.003
  3. ↑ Zhou, W. J., & Yang, C.-D. (2009). The causes and clinical significance of fever in systemic lupus erythematosus: a retrospective study of 487 hospitalised patients. Lupus, 18(9), 807–812. doi:10.1177/0961203309103870
  4. ↑ Kim, H.-A., Jeon, J.-Y., An, J.-M., Koh, B.-R., & Suh, C.-H. (2012). C-reactive protein is a more sensitive and specific marker for diagnosing bacterial infections in systemic lupus erythematosus compared to S100A8/A9 and procalcitonin. The Journal of rheumatology, 39(4), 728–734. doi:10.3899/jrheum.111044
  5. ↑ Scirè, C. A., Cavagna, L., Perotti, C., Bruschi, E., Caporali, R., & Montecucco, C. (2006). Diagnostic value of procalcitonin measurement in febrile patients with systemic autoimmune diseases. Clinical and experimental rheumatology, 24(2), 123–128.
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