Difference between revisions of "Systemic lupus erythematosus"

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*Female:Male 10:1
 
*Female:Male 10:1
 
*Peak in 20s-30s
 
*Peak in 20s-30s
−
*More common in African Americans
+
*More common in Black patients
  
 
==Clinical Features==
 
==Clinical Features==
 +
[[File:Lupusfoto.jpg|thumb|Typical "butterfly" malar rash.]]
 +
[[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>
 
'''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  
 
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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'''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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{{Glomerulonephritis causes}}
 
{{Glomerulonephritis causes}}
  
−
==Diagnosis==
+
==Evaluation==
 
'''Undiagnosed'''
 
'''Undiagnosed'''
 
*CBC
 
*CBC
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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')
  
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*CBC
 
*CBC
 
*Chem
 
*Chem
−
*UA
+
*[[Urinalysis]]
 
*Urine pregnancy
 
*Urine pregnancy
 
*As directed by organ system involved
 
*As directed by organ system involved
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*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'''
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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
  

Latest revision as of 04:16, 27 November 2019

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

  • Inflammatory complications
  • Infectious
    • Stress dose steroids with hydrocortisone 100mg IV Q8hr if on or recently on steroids
  • Dermatologic
  • If drug induced lupus, stop medication and consider alternative

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

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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