Wegener's granulomatosis without renal involvement

This code signifies a systemic autoimmune disease characterized by granulomatous inflammation and vasculitis affecting small to medium-sized blood vessels, primarily involving the upper and lower respiratory tracts. It represents a specific presentation of granulomatosis with polyangiitis (GPA) where there is no evidence of kidney involvement.

Use this code when documentation clearly states a diagnosis of Wegener's granulomatosis or granulomatosis with polyangiitis, and specifically indicates the absence of renal manifestations such as glomerulonephritis, proteinuria, or hematuria. This code is appropriate for initial diagnosis or follow-up encounters where renal involvement has been ruled out.

• Ensure documentation explicitly states "without renal involvement" or similar phrasing to support this specific code.
• Do not use this code if any renal involvement, however mild, is noted; instead, refer to codes for GPA with renal involvement.
• Consider additional codes for specific organ manifestations (e.g., lung, sinus) if documented.