top of page

Systemic Lupus Erythematous (SLE)

Systemic Lupus Erythematous (SLE) is a multi-system, autoimmune, inflammatory disease that commonly affects Afro-Caribbeans women of child-bearing age (peak age of onset 20-40 years). It's the presence of Anti-Nuclear Ab’s (ANA) and anti-dsDNA that leads to the chronic inflammation.


The main causes of death in these patients is CVD and Lupus Nephritis.


Presentation

Follows a relapsing-remitting course, with flares being triggered by:

  • Overexposure to sunlight

  • Infections

  • Stress

  • Oestrogen-containing contraception (e.g. COCP)


Clinical features include:

  • Non-specific symptoms - Fatigue, Weight loss, Fever, Malaise

  • Arthralgia

  • Photosensitive Malar rash - butterfly-shaped rash worsened by sunlight

  • Mouth/nose/genital ulcers

  • Lymphadenopathy, Splenomegaly

  • CVS - Raynaud’s, Pericarditis, Myocarditis

  • Lupus nephritis - usually asymptomatic before presenting as nephritic/otic syndrome

“Butterfly rash in systemic lupus” © Doktorinternet CC BY-SA 4.0 (https://creativecommons.org/licenses/by-sa/4.0/)

Investigations

Bloods:

  • ANA - +ve in most cases but not diagnostic

  • Anti-dsDNA and Anti-Smith - Much more SLE specific than ANA, so can be diagnostic

    • Anti-dsDNA can also be used to monitor disease activity and response to treatment

  • C3, C4 - Low

  • Anti-phospholipid (e.g. Anticardiolipin)

  • ESR

  • FBC and Clotting - A raised PT suggests presence of lupus anticoagulant and should prompt checking for anti-phospholipid syndrome. These patients often also have anaemia of chronic disease.

  • U+Es and urinalysis - screen for renal involvement


Important to assess for Lupus Nephritis with Urinalysis (check for haematuria), ACR (check for proteinuria), and Renal biopsy.


Diagnosis

For a diagnosis, there has to be 4+ findings (at least 1 clinical and 1 laboratory) OR Biopsy-proven Lupus nephritis with +ve ANA/Anti-dsDNA.


Clinical findings - SOAP BRAIN MD:

  • Serositis (pleuritis, pericarditis)

  • Oral ulcers

  • Arthritis

  • Photosensitivity

  • Blood (pancytopenia)

  • Renal (proteinuria)

  • ANA

  • Immunological (dsDNA etc.)

  • Neurologic (psych, seizures)

  • Malar rash

  • Discoid rash


Labaratory findings:

  • ANA +ve

  • Anti-dsDNA +ve

  • Anti-Smith +ve

  • Anti-cardiolipin +ve

  • Low complement (C3 or C4)

  • +ve Direct Coombs test


Management

The mainstay of treatment is:

  • NSAIDs and Hydroxychloroquine for mild disease +/- short-term corticosteroids for flares

  • Sunscreen for photosensitive malar rash


If there's prominent organ involvement, patients are given long-term corticosteroids, which is usally co-prescribed with DMARDs as a ‘steroid sparing agent’ therefore allowing for a lower steroid dose to be used.


In the severe flares with renal, neurological or haematological effects, high-dose corticosteroids in combination with immunosuppressants, such as Cyclophosphamide, are usually most effective. The renal involvement may lead to HTN, for which an ACEi should be prescribed.


Antiphospholipid Syndrome (APS)

This is a hypercoagulable state due to the presence of anti-cardiolipin, leading to clots as well as pregnancy-related complications, like miscarriage, stillbirth and pre-eclampsia.


Presentation - CLOT:

  • Coagulation defect - usually VTE, but can be arterial

  • Livedo reticularis - mottled, cyanotic skin discolouration

  • Obstetric - recurrent miscarriage, pre-eclampsia

  • Thrombocytopenia


Diagnosed with 1+ of the following +ve on 2 occasions, 12-weeks apart:

  • Anti-cardiolipin

  • Anti-beta2-GPI

  • Positive lupus anticoagulant assay


Management:

  • 75 mg Aspirin and LMWH - warfarin avoided as it’s teratogenic

  • Avoid COCP/HRT to reduce risk of VTE


N.B. It's important to note that, outside of pregnancy, APS is treated with Warfarin.


bottom of page