1. NYU Medical Grand Rounds Clinical Vignette Lucy Doyle MD, PGY-2 March 24, 2010 U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

2. A 54-year-old male smoker presents with progressively worsening dyspnea for several years. Chief Complaint U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

3. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS The patient was in his usual state of health until 8 years prior to admission when he first began to experience dyspnea on exertion. During an early emergency room visit, a chest CT demonstrated ground glass opacities, sub-pleural honeycombing and fibrosis. Over the next several years, however, that patient did not return for further medical attention.

4. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS As the patient’s symptoms progressed, the patient returned four years later for evaluation. Pulmonary function tests were obtained and consistent with restrictive physiology and mildly decreased diffusion capacity. The patient was reluctant to undergo bronchoscopy and again did not return for medical care for several years.

5. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Several months prior to admission, the patient returned complaining of cough, worsened dyspnea and further decreases in exercise tolerance. Bronchoscopy with trans-bronchial biopsy was performed but non-diagnostic. The patient now presents for further evaluation of his markedly worsened symptoms and functional status.

6. Additional History U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Past Medical History PPD (+) Treated in 1999 Past Surgical History None Family History Father: Lung cancer Social History Former steel worker Current smoker 1/2 pack per day 35 pack-years Social alcohol use Remote drug use Cannabis Cocaine

7. Outpatient Medications U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Albuterol metered dose inhaler as needed Allergies: None

8. Physical Examination U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS General: Well-appearing man in no acute distress Vitals: T 98.7 F, BP 107/78, HR 100, RR 16 O 2 saturation: 95% on room air, 98% on 2L nasal cannula Lungs: Bilateral basilar dry rales Extremities: Bilateral clubbing The remainder of the physical exam was normal.

9. Initial Studies U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS CBC: Within normal limits Basic Metabolic Panel: Within normal limits ACE: 38 (within normal) LDH: 246 Anti-SCL-70: 108 (0-99) ANA: negative

10. Chest X-ray U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

11. Imaging Reports U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Chest X-Ray No new consolidations or pleural effusions Interstitial lung disease, unchanged Chest CT Interstitial lung disease with honeycombing and traction bronchiectasis most significant in the upper airways. New diffuse bilateral airspace disease which may represent pulmonary edema.

12. Working Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Interstitial lung disease, unknown etiology

13. U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Hospital Course The patient underwent open lung biopsy of right middle and lower lobes. The biopsy revealed dense fibrosis with honeycomb changes and fibroblastic foci, consistent with usual interstitial pneumonia. The patient tolerated the procedure well, but eventually required intubation for hypoxic respiratory failure. In accordance with the patient’s wishes, further care was not escalated, and the patient passed away 2 weeks later.

14. U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Final Diagnosis Usual Interstitial Pneumonia

15. U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS http://clinicalcorrelations.org Clinical Correlations The NYU Internal Medicine Blog A Daily Dose of Medicine