Difference between revisions of "Systemic lupus erythematosus"
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Line 6: | Line 6: | ||
*Female:Male 10:1 | *Female:Male 10:1 | ||
*Peak in 20s-30s | *Peak in 20s-30s | ||
− | *More common in | + | *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 | ||
Line 19: | Line 22: | ||
**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 | ||
Line 44: | Line 47: | ||
***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]] | ||
Line 52: | Line 55: | ||
*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 | ||
− | |||
*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]] | ||
Line 81: | Line 83: | ||
*[[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}} | ||
− | == | + | ==Evaluation== |
'''Undiagnosed''' | '''Undiagnosed''' | ||
*CBC | *CBC | ||
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*ANA | *ANA | ||
*ESR | *ESR | ||
− | * | + | *[[Urinalysis]] |
− | *Bedside | + | *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') | ||
Line 107: | Line 109: | ||
*CBC | *CBC | ||
*Chem | *Chem | ||
− | * | + | *[[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 | + | *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''' | ||
Line 145: | Line 147: | ||
==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
Contents
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.