Waterhouse-Friderichsen Syndrome Due to Haemophilus Influenzae Type B in an Immunocompetent, Fully Vaccinated Adult

Abstract

Waterhouse-Friderichsen syndrome (WFS) is a rare, highly fatal disease that is the result of adrenal gland hemorrhage and infarction, typically due to Neisseria meningitidis infection. [1] We report a case of a patient who developed WFS secondary to Haemophilus influenzae type b (Hib) bacteremia. CASE PRESENTATION:A 19-year-old male with no medical history presented with vomiting and diarrhea for 2 days. Patient was febrile to 103.1 degrees Fahrenheit, hypotensive to 60/23, and tachycardic to 170/min. Labs showed WBC 7.9, platelets 98, creatinine 5.3, lactic acid 9.9, procalcitonin 256, and troponin peak 44.509. Respiratory viral panel and blood cultures were positive for rhinovirus and Hib, respectively. CT chest revealed scattered bilateral groundglass opacities. Patient had cardiac arrest x2. He was intubated and started on vasopressors/inotropes, antimicrobials, and renal replacement therapy. Echocardiogram showed ejection fraction (EF) 10-15%, necessitating intra-aortic balloon pump placement and veno-arterial extracorporeal membrane oxygenation (VA ECMO). Hospital course was complicated by the development of purpura fulminans, disseminated intravascular coagulation (DIC), rhabdomyolysis, heart block requiring transvenous pacemaker, and candida albicans fungemia. Patient was ultimately diagnosed with WFS and treated with hydrocortisone, resulting in hemodynamic improvement and weaning of circulatory support. Despite this, he eventually succumbed to his illness and the complications mentioned above. DISCUSSION: While Neisseria meningitidis accounts for the majority of cases of WFS, other bacteria, such as Haemophilus influenzae, Streptococcus pneumoniae, Staphylococcus aureus, and Pseudomonas aeruginosa, have been implicated.[2] Suspicion should be raised in any patient presenting with fever, purpura, coagulopathy, and signs of adrenal insufficiency. [1] Diagnosis is confirmed by CT scan showing adrenal hemorrhage.[2] Mortality rates are as high as 50%, particularly when there are delays in diagnosis and subsequent treatment with corticosteroids.[1,2]Our patient presented with Hib bacteremia of unclear etiology. One possible explanation is pneumonia, as evidenced by groundglass opacities on CT chest, but these may be better explained by concurrent rhinovirus infection. He developed the bacteremia despite being up to date on immunizations and having vaccine titers, including Hib, within the protective range. Other immune deficiency workup was unrevealing. WFS was suspected but could not be confirmed with imaging due to hemodynamic instability and significant vasopressor support.CONCLUSIONS: WFS is an uncommon cause of adrenal insufficiency that requires prompt diagnosis and treatment. It should be considered in any patient presenting with septic shock, regardless of the causative organism.