In simple words: Sjögren's syndrome is a disease where the body's immune system attacks its own moisture-producing glands, leading to dry eyes and mouth. This particular type of Sjögren's also affects the lungs, causing breathing problems like chronic bronchitis.

Sjögren’s syndrome with lung involvement is a systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, particularly the salivary and lacrimal glands, resulting in dryness of the mouth and eyes (sicca syndrome).This specific code indicates the presence of lung involvement in addition to the typical Sjögren's syndrome symptoms.Pulmonary manifestations can range from airway disease, such as chronic bronchitis and bronchiolitis, to interstitial lung disease, characterized by inflammation and scarring of lung tissue.

Example 1: A patient with known Sjögren's syndrome presents with a persistent dry cough, shortness of breath, and decreased lung function tests, indicative of lung involvement., A patient experiences dry eyes and mouth alongside a chronic cough and recurrent respiratory infections. Further investigation reveals interstitial lung disease, leading to a diagnosis of Sjögren's syndrome with lung involvement., A patient initially presents with interstitial lung disease. During the diagnostic workup, the patient also reports dry eyes and mouth, which leads to further testing and a diagnosis of Sjögren's syndrome with lung involvement.

Documentation should include symptoms (dry eyes, dry mouth, cough, shortness of breath), physical exam findings, results of Schirmer's test, ANA test, spirometry, imaging studies (chest X-ray, CT scan), and biopsy results if performed. The type of lung involvement (airway disease, interstitial lung disease) should be specified.

** The presence of cystic lung lesions in a patient with Sjögren's syndrome may warrant further investigation for lymphoproliferative disorders.