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Juvenile Systemic Lupus Erythematosus

Juvenile Systemic Lupus Erythematosus (JSLE) is rare but is more common in non-caucasians, with a predominance of girls affected in the adolescent group and a more equal sex distribution in young children.

The arthritis of JSLE is usually polyarticular, often the pain is the major symptom but there is little objective swelling. 

Extra - articular features are variable, and a diagnosis of JSLE is made with a combination of clinical and laboratory features (which are summarised below). 

Common presenting symptoms of JSLE:

  • Skin - Malar rash (photosensitivity), Alopecia, vasculitis rashes, Raynaud ’s phenomenon.
  • Arthritis - polyarticular (small joints mainly).
  • Systemic features - Fatigue / Fever / Weight loss / Oral ulcers.
  • Pleuritis/pericarditis.
  • Central nervous system (e.g., headaches, seizures, psychosis).
  • Lymphadenopathy.
  • Hepatosplenomegaly.
  • Hypertension, peripheral oedema, facial puffiness (periorbital oedema).

Laboratory findings:

  • Anaemia (may be haemolytic with positive red cell autoantibodies).
  • Leukopaenia, lymphopaenia, thrombocytopaenia.
  • Elevated liver enzymes.
  • Elevated kidney function tests (blood urea nitrogen, creatinine).
  • Abnormal urinary sediment.
  • Decreased complement components C3 and C4.
  • Positive antinuclear antibody (ANA).
  • High titre positive anti-double stranded (ds) DNA antibody.
  • Other positive autoantibodies  (anti-Ro (SSA); anti-La (SSB); anti-Sm; anti-RNP).
  • Positive anti-phospholipid antibodies (anti-cardiolipin, lupus anticoagulant).

Drug - induced JSLE can develop from the use of anti-convulsants, oral contraceptives or minocycline.

The medical management of JSLE is complex and requires specialist supervision, with nearly all patients requiring corticosteroid and immunosuppressive medications

In addition, patients often require anti-hypertensives, anti-coagulation (related to anti-phospholipid syndrome), and medications to control dyslipidaemias and avoid osteoporosis (e.g., calcium & vitamin D).

The photographs below show skin vasculitis in JSLE.

 

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