A39.1

HCC Category Mapping

What This Code Means

Waterhouse-Friderichsen syndrome is a life-threatening condition caused by meningococcal infection that leads to severe bleeding and failure of the adrenal glands. This rare but critical complication typically develops rapidly and requires immediate emergency treatment.

Coding Tips

• •This code should be used only when meningococcal septicemia with acute adrenal insufficiency and hemorrhage is documented; verify the meningococcal etiology is confirmed in the medical record
• •This condition is often fatal and may be coded alongside sepsis codes (A39.0) and other complications; ensure all documented manifestations including DIC, shock, and organ failure are captured with appropriate additional codes

Clinical Significance

Waterhouse-Friderichsen syndrome is a catastrophic adrenal hemorrhagic necrosis most commonly caused by fulminant meningococcal septicemia. It presents with rapidly progressive purpura fulminans, DIC, and acute bilateral adrenal gland destruction leading to acute adrenal insufficiency and cardiovascular collapse. Mortality is extremely high even with aggressive treatment.

Documentation Requirements

• ✓Clinical triad: purpura fulminans, DIC, and acute adrenal insufficiency
• ✓Laboratory evidence of adrenal insufficiency (low cortisol, electrolyte abnormalities)
• ✓Coagulation studies showing DIC (elevated D-dimer, prolonged PT/PTT, low fibrinogen, thrombocytopenia)
• ✓Blood cultures or clinical evidence of meningococcal infection
• ✓Imaging of adrenal glands (CT showing bilateral adrenal hemorrhage) when available

Commonly Confused Codes