Systemic lupus erythematosus: Difference between revisions
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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 | *Peak in 20s-30s | ||
*More common in Black patients | |||
==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> | |||
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]] | |||
*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:''' | '''Organ system affected:''' | ||
*Cardiopulmonary | *Cardiopulmonary | ||
**Pneumonia | **[[Pneumonia]] | ||
***Cover for ''[[Listeria]]'' and ''[[ | ***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 [[ | ****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 | ||
*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]] | ||
* | *[[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 | *[[Septic Arthritis]] | ||
*[[Lyme | *[[Lyme Disease]] | ||
*Vasculitis | *[[Vasculitis]] | ||
*[[Acute Rheumatic Fever]] | *[[Acute Rheumatic Fever]] | ||
*[[Toxic Shock Syndrome]] | *[[Toxic Shock Syndrome]] | ||
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*[[DIC]] | *[[DIC]] | ||
== | {{Differential Diagnosis Polyarthritis}} | ||
{{Glomerulonephritis causes}} | |||
==Evaluation== | |||
'''Undiagnosed''' | '''Undiagnosed''' | ||
*CBC | *CBC | ||
*Chem 10 | *Chem 10 | ||
*Urine pregnancy | |||
*ANA | *ANA | ||
*ESR | *ESR | ||
* | *[[Urinalysis]] | ||
*Bedside | *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') | ||
''' | '''Flare''' | ||
*Bedside echo if ill or hypotensive | *Bedside echo if ill or hypotensive | ||
*CBC | *CBC | ||
*Chem | *Chem | ||
* | *[[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]] | ||
== | ==References== | ||
<references/> | <references/> | ||
[[Category: | [[Category:Rheumatology]] |
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
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
- Clinical criteria
- Malar rash, bullous lupus, photosensitivity
- Discoid rash, hypertrophic lupus
- Oral ulcers or nasal ulcers
- Non-scarring alopecia
- Synovitis
- Serositis
- Nephritis
- Cerebritis, myelitis, neuropathy
- Hemolytic anemia
- Leukopenia or lymphopenia
- Thrombocytopenia
- 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:
- Cardiopulmonary
- Pneumonia
- Cover for Listeria and Pseudomonas
- CAD
- More common and more complications post-PCI
- PE
- Pericarditis
- Endocarditis
- Infectious and Libman-Sachs
- Pneumonia
- Neuropsychiatric/Altered mental status
- Non-convulsive status epilepticus
- CNS vasculitis
- Stroke
- Encephalitis
- Meningitis
- Musculoskeletal
- Arthritis
- Usually symmetric
- Consider septic arthritis if there is a single inflamed joint
- Cover for Salmonella in addition to standard coverage
- Arthritis
- GI
- Lupus enteritis (mesenteric vasculitis)
- Most common cause of acute abdominal pain
- Pancreatitis
- PUD
- Lupus enteritis (mesenteric vasculitis)
- Dermatologic
- Malar rash across bridge of nose
- Discoid rash, erythematous with scale
- Renal
- Usually a nephritis
- Can cause a glomerulonephrosis
Differential Diagnosis
- Rheumatoid arthritis
- Sjögren Syndrome
- Dermatomyositis
- Polymyositis
- Stevens-Johnson syndrome
- Toxic Epidermal Necrolysis
- Septic Arthritis
- Lyme Disease
- Vasculitis
- Acute Rheumatic Fever
- Toxic Shock Syndrome
- TTP
- ITP
- DIC
Polyarthritis
- Fibromyalgia
- Juvenile idiopathic arthritis
- Lyme disease
- Osteoarthritis
- Psoriatic arthritis
- Reactive poststreptococcal arthritis
- Rheumatoid arthritis
- Rheumatic fever
- Serum sickness
- Systemic lupus erythematosus
- Serum sickness–like reactions
- Viral arthritis
Causes of Glomerulonephritis
- Poststreptococcal glomerulonephritis
- Hemolytic-uremic syndrome
- Henoch-Schonlein purpura
- IgA nephropathy
- Lupus nephritis
- Alport syndrome
- Goodpasture syndrome
- Paraneoplastic
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
- 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)
- High risk:
Fever in SLE
- Must differentiate disease activity (flare) from infection
Risk Factors for Infection [2]
- Neutropenia/Lymphopenia
- Hypocomplementemia
- Immunosuppressive therapy (especially azathioprine [3])
Studies
Management
- Inflammatory complications
- Methylprednisolone 1-2mg/kg in most cases
- Infectious
- Stress dose steroids with hydrocortisone 100mg IV Q8hr if on or recently on steroids
- Dermatologic
- Hydrocortisone 1% cream
- 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
- Arthritis
- Fever and Rash
- Lupus Anticoagulant
- Pericarditis
- Pericardial Effusion and Tamponade
- Acute Renal Failure
- Adrenal Crisis
References
- ↑ Lisnevskaia L, et al. Systemic Lupus Erythematosus. Lancet. 2014 May 29. Epub ahead of print.
- ↑ 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
- ↑ 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
- ↑ 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
- ↑ 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.