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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. 

The photograph below shows a malar rash (which can be photosensitive) in JSLE - typically this spares the nasolabial folds and helps differentiate from other rashes such as acne)

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 (AST, ALT) 
  • Elevated kidney function tests (blood urea nitrogen, creatinine)
  • Abnormal urinary sediment
  • Decreased complement components C3 and C4
  • Positive antinuclear antibody
  • High titre positive anti - double - stranded (ds) DNA antibody
  • Positive autoantibodies to extractable antigens (anti - Ro (SSA); anti - La (SSB); anti - Sm; anti - RNP)
  • Positive antiphospholipid 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 hydroxychloroquine, corticosteroid and immunosuppressive medications. Investigations before starting immunosuppression are important to exclude infection and malignancy.

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

The photograph below shows mouth ulcers of the palate and also skin changes of JSLE.

 

The photograph below shows skin vasculitis in JSLE

The photograph below shows diffuse alopecia in JSLE

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