Whipple's disease

This code represents Whipple's disease, a rare, chronic, multi-system infectious disease caused by the bacterium Tropheryma whipplei. It primarily affects the small intestine, leading to malabsorption, but can also involve the joints, heart, brain, and other organs. Untreated, it is a progressive and often fatal condition.

Use this code when documentation confirms a diagnosis of Whipple's disease, typically supported by small bowel biopsy showing characteristic periodic acid-Schiff (PAS)-positive macrophages. This code is appropriate for initial diagnosis, ongoing management, and complications directly attributable to the disease.

• Ensure the documentation clearly specifies Whipple's disease and not another malabsorption syndrome.
• Code any associated manifestations or complications (e.g., arthropathy, endocarditis, neurological involvement) separately.
• This is a definitive diagnosis; avoid using it for suspected or rule-out cases.