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Revision as of 18:24, 5 July 2017

Systemic lupus erythematosus Microchapters

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Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Systemic lupus erythematosus from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

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Echocardiography or Ultrasound

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Treatment

Medical Therapy

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Lupus and Quality of Life

Cost-Effectiveness of Therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Systemic lupus erythematosus (SLE) one of several diseases known as the great imitator[1] because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years.

History and symptoms

Gastrointestinal involvement:

Organ system Disease Sympton
Gastrointestinal involvement Dysphagia 
  • Retrosternal chest pain
  • Heartburn
  • Regurgitation
  • Odynophagia
Peptic ulcer disease
  • Epigastric pain
  • Food-provoked epigastric discomfort and fullness
  • Early satiety
  • Nausea
Intestinal pseudo-obstruction
  • Abdominal pain
  • Bloating
  • Distension
Protein-losing enteropathy
  • Profound edema
  • Hypoalbuminemia
  • Severe diarrhea
Acute pancreatitis
  • Severe persistent epigastric pain often radiating to the back
Mesenteric vasculitis
  • Abdominal pain
  • Food aversion
  • Weight loss
  • Nausea
  • Vomiting
  • Diarrhea
    • Due to chronic mesenteric ischemia
Primary peritonitis
  • Abdominal bloating or distention
  • Nausea and vomiting
  • Diarrhea
  • Constipation or the inability to pass gas
  • Anorexia
Pulmonary involvement Pleural disease
  • Cough
  • Dyspnea
  • Fever
Acute pneumonitis
  • Fever
  • Cough (sometimes with hemoptysis)
  • Dyspnea/ physical examination reveals tachypnea, tachycardia, basilar crackles (may be late inspiratory), and hypoxemia
Pulmonary hemorrhage
  • Dyspnea
  • Cough
  • Hemoptysis
Interstitial lung disease
  • May be asymptomatic
  • Chronic nonproductive cough
  • Dyspnea
  • Decreased exercise tolerance
Thromboembolic disease 
  • Dyspnea
  • Sharp chest pain that may become worse with deep breathing or coughing
Pulmonary hypertension
  • Dyspnea
  • Palpitations
  • Fatigue
  • Impaired exercise tolerance
  • Weakness
  • Syncope
  • Edema
  • Increased abdominal girth/ phE=pulmonary hypertension (loud second heart sound) or cor pulmonale (eg, peripheral edema, ascites, hepatomegaly)/
Shrinking lung
  • Dyspnea
  • Pleuritic chest pain (episodic)
Cardiac involvement Valvular disease
  • most often valve thickening, nodules, and regurgitation, and less frequently vegetations or stenotic lesions, is noted echocardiographically, and the presence of valvular lesions may increase the risk of serious complications
Nonbacterial thrombotic endocarditis (Libman-Sacks, verrucous endocarditis)
  • may occur in patients with SLE and is associated with antiphospholipid antibodies (aPL).
Pericardial disease is noted in approximately one-half of patients with SLE at some time in the course of their disease. Diagnostic pericardiocentesis is suggested for those in whom purulent pericarditis is suspected and for those who do not respond to treatment with nonsteroidal antiinflammatory drugs (NSAIDs) and/or glucocorticoids.
Acute pericarditis Symptomatic often responds to an NSAID; those who do not tolerate or cannot take NSAIDs may use prednisone (0.5 to 1 mg/kg/day in divided doses)
Myocarditis  in SLE can cause resting tachycardia, cardiomegaly, heart failure, conduction abnormalities, and/or arrhythmias.

myocarditis, we suggest initial treatment with high-dose glucocorticoids (Grade 2C). A typical regimen is methylprednisolone 1000 mg intravenously daily for three days.

Coronary heart disease
Neurological involvement Cognitive dysfunction
Stroke mechanisms are heterogenous in SLE and include arterial and venous thrombosis, cardiogenic embolism, and small vessel infarcts
Seizures
Psychosis
  • Hallucinations visual
  • Auditory
Headache
Neuropathies
Genitourinary Nephrotic syndrome
  • Hypertension
  • Peripheral edema
  • Foamy urine
  • Weight gain
Musculoskeletal Arthritis
  • arthralgias
  • effusions
  • Decreased range of motion of both small and large joints
  • Morning stiffness
Avascular necrosis
  • Asymptomatic
  • Mild to moderate pain in groin and lower abdomen
  • Mainly occur in patients treated with corticosteroids
Bone fragility fractures
Secondary pain amplification
Osteoporosis 
  • Loss of height
  • Sudden back pain
Skin disorder acute cutaneous lupus erythema (ACLE) (also known as “the butterfly rash”)
  • Localized ACLE (ie, malar rash, butterfly rash)
  • Generalized ACLE
  • Toxic epidermal necrolysis-like ACLE
  • Erythema in a malar distribution over the cheeks and nose (but sparing the nasolabial folds), which appears after sun exposure
discoid lesions more inflammatory and which have a tendency to scar
Photosensitivity common theme for skin lesions associated with SLE
oral and/or nasal ulcers usually painless
Nonscarring alopecia may occur at some point during the course of their disease
Subacute cutaneous lupus erythematosus (SCLE) Annular SCLE

Papulosquamous SCLE

Drug-induced SCLE

Less common variants: erythrodermic, poikilodermatous, erythema multiforme-like (Rowell syndrome), and vesiculobullous annular SCLE

Chronic cutaneous lupus erythematosus (CCLE) Discoid lupus erythematosus (DLE)

Localized DLE

Generalized DLE

Hypertrophic DLE

Lupus erythematosus tumidus (LE tumidus)

Lupus profundus (also known as lupus panniculitis)

Chilblain lupus erythematosus (chilblain LE)

Lichenoid cutaneous lupus erythematosus-lichen planus overlap syndrome (LE-LP overlap syndrome)

Common initial and chronic complaints are fever, malaise, joint pains, myalgias and fatigue. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms, however, they are considered suggestive.

Common Symptoms

  • Constitutional symptoms
    • Fatigue
    • Fever
    • Myalgia
    • Muscle tenderness
    • Muscle weakness
    • Weight change
      • Weight gain due to increase in water retention and increase in appetite
      • Weight loss due to medication side effect and gastro-intestinal problems
Constitutional symptoms Fatigue the most common complaint 11085805

multidimensional phenomenon due to different factors 7779127

does not correlate with disease activity 9598886

frequently associated with depression, sleep disturbances, and concomitant fibromyalgia
Fever a manifestation of active disease-lupus flare, especially within short-term evolution of the disease 14530779

Can be hard to differentiate the cause of fever from other inflammatory and infection diseases

Leukopenia or normal range of leukocytes in the setting of fever is more suggestive of lupus activity rather than infection

Myalgia may be first complaint and initial reason for the patient to seeks medical attention

May happen as a result of treatment with glucocorticoids or glucocorticoid withdrawal

perivascular and perifascicular mononuclear cell infiltrates in 25 percent of patients

muscle atrophy, microtubular inclusions, mononuclear infiltrate, fiber necrosis, and, occasionally, vacuolated muscle fibers 2319520

Muscle tenderness
Muscle weakness
Weight change
  • Weight loss due to:
    • Decreased appetite
    • Side effects of medications (particularly diuretics)
    • Gastrointestinal symptoms related to lupus (e.g. gastroesophageal reflux, abdominal pain, peptic ulcer disease, or pancreatitis)
  • Weight gain due to:
    • Hypoalbuminemia:
      • Result in salt and water retention (e.g. due to nephrotic syndrome or protein losing enteropathy)
    • Increased appetite associated with the use of glucocorticoids

References

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