SLE resident survival guide

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SLE
Resident Survival Guide
Overview
Causes
FIRE
Diagnosis
Treatment
Do's
Don'ts


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2] ; Aditya Ganti M.B.B.S. [3]

Overview

Causes

  • Exposure to ultraviolet (UV) light[3]
    • Can exacerbate or induce systemic manifestations of SLE 
  • Drug-induced lupus

FIRE

Diagnosis

Diagnostic criteria:

In 2012, Systemic Lupus International Collaboration Criteria (SLICC) developed a new criteria for SLE diagnosis. SLICC criteria for the classification of systemic lupus erythematosus was developed based on the old ACR criteria for the classification of systemic lupus erythematosus to address a more sensitive diagnostic criteria and also to cover weaknesses of the previous ACR criteria.[4][5]

Based on SLICC criteria, diagnosis of SLE is defined as:[6]

  • Meeting at least 4 of 17 criteria, including at least 1 of the 11 clinical criteria and one of the six immunologic criteria 

OR

A criterion is considered positive if one or more of the observations listed in the definition for the criterion are present in the patient. A criterion should only be counted once, regardless of the number of observations in the definition that the patient presents with.

Category Criterion Definition
Clinical Acute cutaneous lupus
Chronic cutaneous lupus
Nonscarring alopecia
Oral or nasal ulcers
Joint disease
Serositis
Renal
Neurologic
Hematologic Hemolytic anemia
  • Hemoglobin less than 12 g/dL in females and 13 g/dL in males
Leukopenia or lymphopenia
Thrombocytopenia
Immunologic ANA
  • ANA level above laboratory reference range
Anti-dsDNA
Anit-SM
Antiphospholipid
Low complement
  • Low C3
  • Low C4
  • Low CH50
Direct Coombs' test

Complete diagnostic approach:

SLE Presentation

Less common Presentation

  • Dysphagia
  • Peptic ulcer disease
  • Intestinal pseudo-obstruction
  • Protein-losing enteropathy
  • Acute pancreatitis
  • Pneumonitis
  • Pleuritis
  • Pulmonary hemorrhage
  • Interstitial lung disease
  • Pulmonary emboli
  • Pulmonary hypertension
  • Pericarditis
  • Myocarditis
  • Seizures
  • Stroke
  • Psychosis
  • Nephrotic syndrome
 
 
 
 
 
 
 
 

Focused History

 
 
 
 
 
 
 
 

Physical Examination

Appearance of the Patient

  • Patient appears well in the earlier stages of the disease
  • Patient appears ill in the late stages of the disease due to multi-organ involvement

Vital Signs

Skin[10][11][12]

Systemic lupus erythematosus 055.jpg For more pictures of the rash presentation in lupus, click here.

HEENT

Neck[18][19]

Lungs[20][21][22]

Heart[23][19][24]

Abdomen[25][26][27][28]

Extremities[29][30][31][32][33]

Neuromuscular[34][35][36][37]

  • Patient is usually oriented to persons, place, and time based on the disease course
  • Cognitive impairment
  • Hallucinations
  • Memory deficit
    • Loose associations
    • Impoverished thought content
    • Illogical thinking
    • Bizarre disorganised or catatonic behaviour
 
 
 
 
 
 
 
 

Laboratory Workup

 
 
 
 
 
 
 
 

Imaging Study

Plain radiographs of swollen joints

  • Erosions

Ultrasonography of painful joints

  • Detect synovitis and tenosynovitis in the hands and wrists in patients with SLE

Renal ultrasonography

  • To assess kidney size
  • To rule out urinary tract obstruction

Chest radiography

  • For suspected pleural effusion, interstitial lung disease, cardiomegaly

Echocardiography

  • For suspected pericardial involvement, to assess for a source of emboli, or noninvasive estimation of pulmonary artery pressure; and for evaluation of suspected valvular lesions, such as verrucae

Computed tomography (CT)

  • For abdominal pain, suspected pancreatitis, interstitial lung disease

Magnetic resonance imaging (MRI)

  • For focal neurologic deficits or cognitive dysfunction
 
 
 
 
 
 
 
 

Other Investigation

Bronchoscopy

Fiberoptic bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial lung biopsies:[38][39]

  • To substantiate the diagnosis of alveolar hemorrhage

Barium swallow or esophagography

Biopsy

  • Renal biopsies:[41][42]
    • Determining the degree of renal involvement
    • Delineating treatment decisions and prognosis in certain clinical scenarios
    • Assess activity and damage (i.e., chronicity)
    • Helping with classification

Paracentesis

Arthrocentesis

  • In the presence of joint effusion to differentiate between different causes of arthritis[47]
 
 

Treatment

Treatment goals

Treatment goals in systemic lupus erythematosus (SLE) include:

  • Ensure long-term survival
  • Achieve the lowest possible disease activity
  • Prevent organ damage
  • Minimize drug toxicity
  • Improve quality of life
General treatment
  • Hydroxychloroquine: 200 to 400 mg daily as a single daily dose or in 2 divided doses.
    • Generally, all patients with any type of SLE manifestation should be treated with hydroxychloroquine regardless of the severity of the disease.

The treatment choice for systemic lupus erythematosus (SLE) is varied based on the severity of the disease and symptoms:

  • Mild cases are defined as disease pattern with one or two organ involvement.
  • Moderate cases are defined as more than 2 organ involvement during disease flares with low grade of involvement and complications or one or two organ involvement with more extensive involvements.
  • Severe cases are defined as presentation of the disease with life threatening complications and multiple (more than 2) organ involvements.

Severe disease

  • Preferred regimen (1): Hydroxychloroquine PO 200 to 400 mg daily as a single daily dose or in 2 divided doses AND methylprednisolone as intravenous "pulse"; 0.5 to 1 g/day for three days in acutely ill patients, or 1 to 2 mg/kg/day in more stable patients
  • Alternative regimen(1): Hydroxychloroquine PO 200 to 400 mg daily as a single daily dose or in 2 divided doses AND prednisone oral; 40-60 mg/day
  • Alternative regimen (2): Mycophenolate
    • For induction: 1 g twice daily for 6 months in combination with a glucocorticoid
    • For maintenance: 0.5-3 g daily or 1 g twice daily
  • Alternative regimen (3): Cyclophosphamide IV 500 mg once every 2 weeks for 6 doses or 500 to 1,000 mg/m2 once every month for 6 doses or 500 to 1,000 mg/m2 every month for 6 months, then every 3 months for a total of at least 2.5 years
  • Alternative regimen (4): Rituximab IV: 375 mg/m2 once weekly for 4 doses or 1,000 mg (flat dose) on days 0 and 15 or 500 to 1,000 mg on days 1 and 15

Less Severe (mild and moderate) disease

  • Preferred regimen (1): Hydroxychloroquine PO 200 to 400 mg daily as a single daily dose or in 2 divided doses
  • Preferred regimen (2): Prednisone PO low doses of 10 mg/d or less for a short term therapy
    • For milder SLE
    • For treatment of cutaneous and musculoskeletal symptoms not responding to other therapies
    • It should be tapered once hydroxychloroquine has taken effect
  • Alternative regimen (1): Azathioprine PO initial 2 mg/kg/day; may reduce to 1.5 mg/kg/day after 1 month
    • Can be used to control symptoms
  • Alternative regimen (2): Methotrexate PO initial therapy with 7.5 mg once weekly; may increase by 2.5 mg increments weekly
    • Can be used to control symptoms

Other organ specific treatments

Fever management[48][49]

Raynaud's phenomenon treatment[50]

Chronic pain management[52]
  • Moderate pain should be treated with mild prescription opiates such as:
  • Moderate to severe chronic pain should be treated with stronger opioids such as:
    • Preferred regimen (1): Hydrocodone: Single doses >40 mg or >60 mg with a total daily dose ≥80 mg
    • Preferred regimen (2): Oxycodone: 5 to 15 mg every 4 to 6 hours as needed
    • Alternative regimen (1): MS Contin: Opioid naive patients can have 5 to 10 mg every 4 hours; usual dosage range between 5 to 15 mg every 4 hours
      • Higher initial doses in patients with prior opioid exposure
    • Alternative regimen (2): Methadone: Maximum initial dose 30 mg
    • Alternative regimen (3): Fentanyl Duragesic Transdermal patch: A convenient treatment option for lupus chronic pain. It has a long lasting effect as well
Cutaneous lupus erythematosus[53][54][55][56][57]
Lupus nephritis treatment[58][59][60]
  • Severe active disease: 
    • Preferred regimen: Glucocorticoid therapy is initiated with intravenous pulse methylprednisolone (250 mg to 1000 mg given over 30 minutes daily for three days) to induce a rapid immunosuppressive effect, followed by conventional doses  
    • Alternative regimen: Conventional doses of oral glucocorticoids (eg, 0.5 to 1 mg/kg per day of prednisone) without a pulse.
      • Oral prednisolone at a dose of 60 mg/day, tapered every two weeks by 10 mg/day until 40 mg/day is reached, then tapered by 5 mg/day until 10 mg/day is reached 
Considerations[60]
 
 

Do's

Don'ts

References

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