Wegener's granulomatosis with renal involvement

This diagnosis code signifies Granulomatosis with Polyangiitis (GPA), formerly known as Wegener's granulomatosis, specifically when the kidneys are affected. GPA is a rare, systemic autoimmune disease characterized by inflammation of small and medium-sized blood vessels, leading to granuloma formation in various organs. Renal involvement is a critical and common manifestation, often presenting as rapidly progressive glomerulonephritis.

Apply this code when documentation clearly indicates a diagnosis of Granulomatosis with Polyangiitis and explicitly states the presence of renal involvement, such as glomerulonephritis, renal failure, or other kidney-related complications directly attributed to the GPA. Supporting documentation would include pathology reports confirming vasculitis and granulomas, positive ANCA serology (especially c-ANCA/PR3-ANCA), and evidence of renal dysfunction.

• Ensure the documentation specifies renal involvement as part of the GPA; do not assume kidney involvement if not explicitly stated.
• Do not confuse with other forms of vasculitis or glomerulonephritis not directly linked to GPA.
• Sequence this code as the primary diagnosis when the GPA with renal involvement is the reason for the encounter or admission.