Systemic lupus erythematosus

Changed by Bruno Di Muzio, 7 Oct 2015

Updates to Article Attributes

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Systemic lupus erythematosus (SLE) is a complex autoimmune disease with multisystem involvement. It is also sometimes classified as a vasculitis

SLE can have a myriad of clinical features and radiographic presentations whichthat are probably best discussed under individual sub topicssubtopics

Epidemiology

There is an overall increased female predilection. In adults, femaleswomen are affected 9-13 times more than males. In children, this ratio is reversed, and males are affected two to three times more often. While it can affect any age group, the peak age at onset around the 2nd to 4th decades. 

The disease is sometimes classified according to early and late onset groups 8-9.

Pathology

SLE can affect multiple components of the immune system, including the complement system, T suppressor-suppressor cells, and cytokine production. It can result in a generation of autoantibodies, which can circulate for many years prior to the development of clinical SLE. 

The disease generally tends to have a relapsing and remitting course.

Diagnosis

The diagnosis of SLE made be if four of eleven ACR (American College of Rheumatology) criteria are present, either serially or simulaneouslysimultaneously 2.  TheseThese criteria were initially published in 1982 but have been revised in 1997.

ACR criteria:

  • malar rash: fixed, flat or raised, occurs over malar eminences and tends to spare the nasolabial folds.
  • discoid rash: erythematous raised patches with adherent keratotic scaling and follicular plugging
  • photosensitivity
  • oral ulcers
  • non-erosive arthritis: where there is involvement of two or more peripheral joints with tenderness, swelling or joint effusions; can occur in ~80% of cases 1
  • pleuritis and/or pericarditis: present in around 17-50% of cases 1
  • proteinuria: >0.5 grams/day
  • haematological abnormality
    • haemolytic anaemia or
    • leukopenia: <4000/mm3 on more than 2two occasions
    • lymphopenia: <1500/mm3 on more than 2two occasions
    • thrombocytopenia: <100,000/mm3 without any precipitant medications
  • neuropsychiatric manifestations
    • seizures or psychotic events in the absence of any underlying precipitating medicationsdrugs or metabolic abnormalities
  • immune abnormality on serology
  • positive antinuclear antibodies
  • -<p><strong>Systemic lupus erythematosus (SLE)</strong> is a complex autoimmune disease with multisystem involvement. It is also sometimes classified as a <a href="/articles/vasculitis">vasculitis</a>. </p><p>SLE can have a myriad of clinical features and radiographic presentations which are probably best discussed under individual sub topics. </p><ul>
  • +<p><strong>Systemic lupus erythematosus (SLE)</strong> is a complex autoimmune disease with multisystem involvement. It is also sometimes classified as a <a href="/articles/vasculitis">vasculitis</a>. </p><p>SLE can have a myriad of clinical features and radiographic presentations that are probably best discussed under individual subtopics. </p><ul>
  • -<li><a href="/articles/thoracic-manifestations-of-systemic-lupus-erythematosus">thoracic manifestations of systemic lupus erythematosus</a></li>
  • +<li><a href="/articles/systemic-lupus-erythematosus-thoracic-manifestations">thoracic manifestations of systemic lupus erythematosus</a></li>
  • -<li><a href="/articles/musculoskeletal-manifestations-of-systemic-lupus-erythematosus">musculoskeletal manifestations of systemic lupus erythematosus</a></li>
  • -</ul><h4>Epidemiology</h4><p>There is an overall increased female predilection. In adults, females are affected 9-13 times more than males. In children this ratio is reversed and males are affected two to three times more often. While it can affect any age group the peak age at onset around the 2<sup>nd</sup> to 4<sup>th </sup>decades. </p><p>The disease is sometimes classified according to early and late onset groups<sup> 8-9</sup>.</p><h4>Pathology</h4><p>SLE can affect multiple components of the immune system, including the complement system, T suppressor cells, and cytokine production. It can result in generation of autoantibodies, which can circulate for many years prior to the development of clinical SLE. </p><p>The disease generally tends to have a relapsing and remitting course.</p><h4>Diagnosis</h4><p>The diagnosis of SLE made be if four of eleven ACR (American College of Rheumatology) criteria are present , either serially or simulaneously <sup>2</sup>.  These criteria were initially published in 1982 but have been revised in 1997.</p><p>ACR criteria:</p><ul>
  • +<li><a href="/articles/systemic-lupus-erythematosus-musculoskeletal-manifestations">musculoskeletal manifestations of systemic lupus erythematosus</a></li>
  • +</ul><h4>Epidemiology</h4><p>There is an overall increased female predilection. In adults, women are affected 9-13 times more than males. In children, this ratio is reversed, and males are affected two to three times more often. While it can affect any age group, the peak age at onset around the 2<sup>nd</sup> to 4<sup>th </sup>decades. </p><p>The disease is sometimes classified according to early and late onset groups<sup> 8-9</sup>.</p><h4>Pathology</h4><p>SLE can affect multiple components of the immune system, including the complement system, T-suppressor cells, and cytokine production. It can result in a generation of autoantibodies, which can circulate for many years prior to the development of clinical SLE. </p><p>The disease tends to have a relapsing and remitting course.</p><h4>Diagnosis</h4><p>The diagnosis of SLE made be if <strong>four</strong> of eleven ACR (American College of Rheumatology) criteria are present, either serially or simultaneously <sup>2</sup>. These criteria were initially published in 1982 but have been revised in 1997.</p><p>ACR criteria:</p><ul>
  • -<li>leukopenia: &lt;4000/mm<sup>3</sup> on more than 2 occasions</li>
  • -<li>lymphopenia: &lt;1500/mm<sup>3</sup> on more than 2 occasions</li>
  • +<li>leukopenia: &lt;4000/mm<sup>3</sup> on more than two occasions</li>
  • +<li>lymphopenia: &lt;1500/mm<sup>3</sup> on more than two occasions</li>
  • -<li>neuropsychiatric manifestations<ul><li>seizures or psychotic events in the absence of any underlying precipitating medications or metabolic abnormalities</li></ul>
  • +<li>neuropsychiatric manifestations<ul><li>seizures or psychotic events in the absence of any underlying precipitating drugs or metabolic abnormalities</li></ul>
  • -<li>antiphospholipid antibodies (requires certain criteria)<ul><li>27-42% of SLE patient can have the <a href="/articles/anti-phopholipid-antibody-syndrome">anti-phopholipid antibody syndrome</a> <sup>1</sup>
  • +<li>antiphospholipid antibodies (requires certain criteria)<ul><li>27-42% of SLE patient can have the <a href="/articles/anti-phopholipid-antibody-syndrome">anti-phospholipid antibody syndrome</a> <sup>1</sup>
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