Acute epiglottitis as the initial presentation of pediatric Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE) is a periodic, multisystem, autoimmune disease characterized by the presence of antinuclear antibodies. Approximately 15% of SLE cases are diagnosed before the age of 16 and children often have more severe disease at onset and higher rates of organ involvement than adults [1]. We report a case of SLE with initial presentation of Haemophilus influenzae epiglottitis. Laryngeal involvement and pneumococcal epiglottitis have infrequently been reported in the adult literature [2-4]. This is the first pediatric case and the first case of Haemophilus influenzae epiglottitis reported in SLE.A 5-year old fully immunized and previously healthy girl of Filipino origin presented to her local hospital with a one day history of high fever (39°C), sore throat, stridor and shortness of breath. She was transferred to BC Children's Hospital and was intubated by an otolaryngologist in the operating room. Subsequently, she was admitted to the ICU and was intubated and ventilated for 3 days. A lateral neck radiograph revealed a profoundly swollen epiglottis and complete airway obstruction (Figure 1). The diagnosis of acute epiglottitis was made based on her clinical picture and imaging findings. She was empirically treated with intravenous Cefotaxime. Her throat swab and blood culture were positive for Haemophilus influenzae type f.On day 2 of admission, she developed multi-organ system dysfunction with anemia, thrombocytopenia, acute renal failure and hypertension (BP 123/80 mmHg). Investigations revealed hemoglobin at 63 g/L, white blood cell 7 × 109/L with lymphocyte of 1.85 × 109/L. Her platelets were 53,000/mm3. DAT was positive. PT and INR were normal. APTT and dilute Russell Viper time were elevated at 41 sec (20.2-32.9) and 2.17 ratio (0.76-1.15) respectively. Fibrinogen and D Dimer were elevated. Her serum creatinine was 143 umol/L (<51). Urinalysis showed 10-20 RBC/hpf, protein 0.3 g/L with hyaline and granular casts. Treatment included suppo