Invasive meningococcal disease can present without classic clinical features, complicating early recognition and delaying life-saving therapy. A recent New England Journal of Medicine case study describes a fatal case of meningococcal disease caused by Neisseria meningitidis serogroup W that presented with gastrointestinal symptoms, septic shock, and disseminated intravascular coagulation, but without a meningococcal rash. The case underscores key diagnostic considerations for infectious diseases physicians.

The patient, a middle-aged woman with chronic liver disease, presented at a Massachusetts emergency department following recent international travel to Brazil, with signs of fever, abdominal pain, and diarrhea. She rapidly developed hypotension, metabolic acidosis, and coagulopathy. In this context, the differential diagnosis reasonably included severe enteric infection, malaria, viral hemorrhagic fever, and gram-negative sepsis. However, meningococcal disease was not initially apparent due to the absence of rash, meningismus, or focal neurologic findings.

This presentation reflects a well-described but often underrecognized phenotype of meningococcemia, particularly with serogroup W, which has been associated with gastrointestinal symptoms and delayed diagnosis. For infectious diseases clinicians, the key challenge is avoiding premature diagnostic closure when early findings point away from classic meningitis or purpura.

Several features of the case should prompt early inclusion of meningococcal disease in the differential diagnosis. The patient developed rapidly progressive shock with severe lactic acidosis and disseminated intravascular coagulation – physiologic findings that are characteristic of meningococcemia regardless of skin manifestations. Severe limb pain, reported early in the course, is another symptom increasingly recognized as an early sign of invasive meningococcal disease.

Importantly, the patient’s underlying liver disease likely contributed to immune dysfunction, including impaired complement activity and splenic clearance. Cirrhosis and functional hyposplenism are established risk factors for invasive bacterial infections and should heighten concern for meningococcemia when patients present with unexplained shock.

Serogroup W has emerged globally as a cause of invasive meningococcal disease with distinct clinical patterns, including abdominal pain, diarrhea, and higher case fatality rates compared with other serogroups. The absence of rash in this case aligns with prior observations and reinforces that rash is not required for diagnosis.

This case reinforces several principles relevant to everyday practice:

• Consider meningococcal disease in patients with shock and coagulopathy, even without rash.

• Include invasive meningococcal disease in the differential diagnosis for returning travelers with abdominal symptoms and rapid clinical deterioration.

• Recognize chronic liver disease and splenic dysfunction as meaningful risk factors.

• Initiate empiric ceftriaxone promptly when meningococcemia is a consideration.

• Ensure early public health notification and chemoprophylaxis of close contacts once the diagnosis is suspected.

While this case does not alter existing guidelines, it does reinforce the importance of clinical vigilance for infectious disease physicians. Invasive meningococcal disease remains a diagnostic challenge when it presents atypically, and early recognition depends less on identifying classic signs, and more on recognizing dangerous physiologic patterns and host risk factors potentially associated with the disease.

Disclosures are available with the full text of this article at NEJM.org

Source: New England Journal of Medicine