Whipple's disease

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

Use this code when the provider's documentation explicitly diagnoses Whipple's disease based on biopsy findings (e.g., PAS-positive macrophages in the small intestine) or PCR detection of T. whipplei. It is appropriate for both initial diagnosis and ongoing management of this condition.

• Ensure documentation clearly states a definitive diagnosis of Whipple's disease, not just suspected cases.
• Code any associated manifestations (e.g., arthropathy, endocarditis, neurological involvement) separately as secondary diagnoses.
• Do not confuse with other malabsorption syndromes; Whipple's disease has a specific bacterial etiology.