Aims This case identifies a rare condition, presenting in an unusual and life-threatening manner. Aims are to (1) raise awareness of a diagnosis which may not be expected in this age group, (2) emphasise that systemic lupus erythematosus (SLE) renders patients susceptible to overwhelming sepsis, and that this may be the first presentation of SLE.
Methods A previously healthy 6 year old Afro-Caribbean boy presented acutely with a 3 day history of diarrhoea and vomiting. On examination he was lethargic, fever and tachycardic. Positive findings included a swollen right elbow, which was painful to touch. Initial impression was gastroenteritis with mild dehydration, and a secondary reactive arthritis. A joint washout was performed to rule out septic arthritis; frank pus was identified. Management involved analgesia, intravenous fluids and intravenous antibiotics. Streptococcus pneumoniae was later cultured from joint aspirate and blood cultures. Clinical deterioration occurred over 10 days, despite repeated joint washouts and alternative antibiotic therapy. He continued to be pyrexial with worsening tachycardia and tachypnoea. Blood tests showed rising inflammatory markers, and new coombes-positive anaemia. Additional clinical features on day 12 included a dusky, painful left index finger, hepatomegaly and pansystolic murmur. Chest x-ray showed pulmonary congestion, urine tests showed microscopic haematuria. A diagnosis of infective endocarditis (IE) with heart failure was made. Further deterioration lead to him requiring intensive care support and aggressive treatment for IE.
Results Multi-system involvement preceded investigation for autoimmune disease. Results were ANA+, dsDNA+, lupus anticoagulant+, with low complement. After slow recovery from this acute episode of illness, repeat testing later confirmed the above results. Retrospectively a diagnosis of SLE was made, presenting as pneumococcal sepsis secondary to functional asplenia and hypocomplementaemia.
Conclusions SLE is an autoimmune connective tissue disorder. It is uncommon in adolescents and extremely rare in children under 8 years. Clinical course is usually insidious, with multisystem involvement collectively leading to diagnosis. Reports of acute onset associated with pneumococcal sepsis are limited. Though sepsis is recognised as a complication of established SLE, it may also occur as a primary presenting feature. Although a rare condition, SLE should be considered as a differential diagnosis for sepsis with multi-system involvement presenting in children.
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