Systemic lupus erythematosus

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

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

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

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

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

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 [2]

Neutropenia/Lymphopenia
Hypocomplementemia
Immunosuppressive therapy (especially azathioprine [3])

Studies

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

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

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.

This article is issued from Wikem. The text is licensed under Creative Commons - Attribution - Sharealike. Additional terms may apply for the media files.

[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.