Silent Collapse: Think Outside the Box - Adult-Onset Waterhouse-Friderichsen Syndrome
Recommended Citation
Umar A, Bilal M, Ahsan B. Silent Collapse: Think Outside the Box - Adult-Onset Waterhouse-Friderichsen Syndrome. Am J Respir Crit Care Med 2026; 212:1.
Document Type
Conference Proceeding
Publication Date
5-15-2026
Publication Title
Am J Respir Crit Care Med
Keywords
General & Internal Medicine, Respiratory System
Abstract
Background Waterhouse-Friderichsen syndrome (WFS) is a rare, life-threatening condition caused by adrenal hemorrhage leading to acute adrenal insufficiency. It is classically seen in children with fulminant meningococcal sepsis but can occasionally occur in adults with atypical or insidious presentations. Awareness of such cases remains limited. Case Presentation: A 76-year-old woman with atrial fibrillation on apixaban and inflammatory arthritis on sulfasalazine presented with right-upper-quadrant pain. She was hemodynamically stable. Laboratory work-up revealed leukocytosis (WBC 28 × 10³/µL), and CT abdomen showed acute cholecystitis. She was started on piperacillin-tazobactam, and surgery recommended medical management.On hospital day 2, she developed diarrhea; Clostridioides difficile testing was indeterminate, and oral vancomycin was initiated. Blood cultures remained negative, and she improved clinically despite persistent leukocytosis.On day 4, a non-blanching purpuric rash appeared on her trunk and extremities. Suspecting a drug reaction, antibiotics were discontinued, while apixaban was continued as her blood counts and coagulation profile remained stable. She developed mild dyspnea with bilateral effusions (BNP 1500 pg/mL) and received a single dose of furosemide.Despite stopping antibiotics, the rash progressed. Infectious Disease was consulted, and repeat CT chest/abdomen/pelvis revealed a new left adrenal hemorrhage (7.7 × 2.6 cm), heterogeneous right adrenal enhancement, and multifocal pneumonia, raising concern for WFS. She had no hemoglobin drop or hypotension, making the hemorrhage clinically silent.Unfortunately, within hours of the CT scan, she developed refractory shock, requiring multiple vasopressors and bilevel positive airway pressure (BiPAP) for respiratory failure. Stress-dose steroids and broad-spectrum antibiotics were initiated, but she rapidly progressed to multiorgan failure and passed away per her DNR/DNI wishes. Discussion: This case represents a possible adult-onset Waterhouse-Friderichsen syndrome (WFS) presenting without the classic features of meningococcal sepsis. The patient was afebrile, culture-negative, and clinically stable until her abrupt decline, illustrating that WFS in adults can evolve silently, with imaging abnormalities preceding hemodynamic collapse. Several factors made this case particularly complex — apixaban use likely predisposed to hemorrhage, sulfasalazine therapy may have blunted the inflammatory response, and the rash, initially attributed to a vancomycin reaction or vasculitis, delayed recognition. Conclusion: This case underscores that WFS can develop de novo during hospitalization, even in previously stable adults. Early imaging and prompt treatment are critical once suspected, as deterioration can be swift and fatal. Clinicians should maintain a broad differential when assessing new rashes — what appears to be a benign reaction may signal an underlying catastrophic process.
Volume
212
First Page
1
