Sjogren syndrome with lung involvement

This code signifies a systemic autoimmune disorder characterized by chronic inflammation of exocrine glands, specifically affecting the lungs. It represents the pulmonary manifestations of Sjögren syndrome, which can include interstitial lung disease, bronchiolitis, or other lung pathologies directly attributable to the autoimmune process.

Apply this code when documentation clearly indicates a diagnosis of Sjögren syndrome and specifies lung involvement, such as interstitial pneumonia, pulmonary fibrosis, or bronchiectasis, as a direct complication. Supporting documentation should include physician notes detailing the Sjögren diagnosis and the specific pulmonary findings, often confirmed by imaging (e.g., CT scan) or pulmonary function tests.

• Ensure the medical record explicitly links the lung involvement to Sjögren syndrome; do not assume the connection.
• Do not use this code for general lung conditions in a patient with Sjögren syndrome if the lung condition is not directly attributed to the autoimmune disease.
• Consider sequencing this code as the primary diagnosis if the lung involvement is the main reason for the encounter, followed by codes for specific lung manifestations if further detail is required.