Difference between revisions of "Systemic lupus erythematosus"

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==Background==
 
==Background==
 
*Autoimmune disorder affecting all systems
 
*Autoimmune disorder affecting all systems
 +
*Also consider drug induced lupus
  
==Epidemiology==
+
===Epidemiology===
 
*Female:Male 10:1
 
*Female:Male 10:1
*More common in African Americans
+
*Peak in 20s-30s
 +
*More common in Black patients
  
 +
==Clinical Features==
 +
'''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
 +
*Clinical criteria
 +
**Malar [[rash]], bullous lupus, photosensitivity
 +
**Discoid rash, hypertrophic lupus
 +
**Oral ulcers or nasal ulcers
 +
**Non-scarring alopecia
 +
**Synovitis
 +
**Serositis
 +
**[[glomerulonephritis|Nephritis]]
 +
**Cerebritis, myelitis, neuropathy
 +
**[[Hemolytic anemia]]
 +
**[[Leukopenia]] or lymphopenia
 +
**[[Thrombocytopenia]]
  
==Clinical Features==
+
*Immunological criteria
'''Diagnostic Criteria:'''
+
**ANA
4 out of 11 of the following:
+
**Anti-dsDNA
#Malar rash
+
**Anti-Sm
#Discoid rash
+
**Antiphospholipid antibody
#Photosensitivity
+
**Low complement C3, low C4
#Oral ulcers
+
**Direct Coombs' test in the absence of haemolytic anaemia
#Arthritis (polyarticular)
 
#Serositis (Pericarditis or pleuritis)
 
#Renal disorder (renal failure, protenuria, casts)
 
#Neurologic or psychotic symptoms
 
#Hematologic (anemia, thrombocytopenia, or leukopenia)
 
#Immunologic (+antibofy testing)
 
#ANA (positive ANA)
 
  
 
'''Organ system affected:'''
 
'''Organ system affected:'''
 
*Cardiopulmonary
 
*Cardiopulmonary
**Pneumonia
+
**[[Pneumonia]]
***Cover for ''[[Listeria]]'' and ''[[Pseudmonas]]''
+
***Cover for ''[[Listeria]]'' and ''[[Pseudomonas]]''
 
**CAD
 
**CAD
 
***More common and more complications post-PCI
 
***More common and more complications post-PCI
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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
+
**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]]
 
*[[Stevens Johnson Syndrome and Toxic Epidermal Necrolysis|Stevens-Johnson syndrome]]
 
*[[Stevens Johnson Syndrome and Toxic Epidermal Necrolysis|Stevens-Johnson syndrome]]
 
*[[Stevens Johnson Syndrome and Toxic Epidermal Necrolysis|Toxic Epidermal Necrolysis]]
 
*[[Stevens Johnson Syndrome and Toxic Epidermal Necrolysis|Toxic Epidermal Necrolysis]]
*[[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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*[[DIC]]
 
*[[DIC]]
  
==Workup==
+
{{Differential Diagnosis Polyarthritis}}
 +
 
 +
{{Glomerulonephritis causes}}
 +
 
 +
==Evaluation==
 
'''Undiagnosed'''
 
'''Undiagnosed'''
 
*CBC
 
*CBC
 
*Chem 10
 
*Chem 10
 +
*Urine pregnancy
 
*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)
+
*(Consider anti-DNA, anti-Smith, anti-Nuclear, anti-phospholipid, C3,C4, direct Coombs')
  
'''Flair'''
+
'''Flare'''
 
*Bedside echo if ill or hypotensive
 
*Bedside echo if ill or hypotensive
 
*CBC
 
*CBC
 
*Chem
 
*Chem
*UA
+
*[[Urinalysis]]
 +
*Urine pregnancy
 
*As directed by organ system involved
 
*As directed by organ system involved
 +
 +
'''Drug Induced Lupus'''
 +
*Anti-histone-Ab positive 95% of the time
 +
*Make sure to review medications
 +
**High risk:
 +
***[[Procainamide]] (antiarrhythmic)
 +
***[[Hydralazine]] (antihypertensive)
 +
**Moderate to low risk:
 +
***Infliximab anti (TNF-α)
 +
***Etanercept anti (TNF-α)
 +
***[[Isoniazid]] (antibiotic)
 +
***[[Minocycline]] (antibiotic)
 +
***[[Pyrazinamide]] (antibiotic)
 +
***[[Quinidine]] (antiarrhythmic)
 +
***D-[[Penicillamine]] (anti-inflammatory)
 +
***[[Carbamazepine]] (anticonvulsant)
 +
***[[Oxcarbazepine]] (anticonvulsant)
 +
***[[Phenytoin]] (anticonvulsant)
 +
***Propafenone (antiarrhythmic)
 +
***[[Chlorpromazine]] (antipsychotic)
 +
 +
===[[Fever]] in SLE===
 +
*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>
 +
*[[Neutropenia]]/Lymphopenia
 +
*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>)
 +
 +
'''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>
 +
*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>
  
 
==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
  
 
==Disposition==
 
==Disposition==
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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
  
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*[[Adrenal Crisis]]
 
*[[Adrenal Crisis]]
  
==Sources==
+
==References==
*Rosen's
 
*Up to date
 
 
 
 
<references/>
 
<references/>
  
[[Category: Rheum]]
+
[[Category:Rheumatology]]

Latest revision as of 15:58, 18 October 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

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
    • Treat with topical 1% hydrocortisone
  • 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.