NYU Medical Grand Rounds Clinical Vignette

1. NYU Medical Grand Rounds Clinical Vignette Lucy Doyle MD, PGY-2 March 24, 2010 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

2. Chief Complaint A 54-year-old male smoker presents with progressively worsening dyspnea for several years. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

3. History of Present Illness • 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

4. History of Present Illness • 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

5. History of Present Illness • 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

6. Additional History • 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 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

7. Outpatient Medications Albuterol metered dose inhaler as needed Allergies: None UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

8. Physical Examination General: Well-appearing man in no acute distress Vitals: T 98.7 F, BP 107/78, HR 100, RR 16 O2 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

9. Initial Studies • CBC: Within normal limits • Basic Metabolic Panel: Within normal limits • ACE: 38 (within normal) • LDH: 246 • Anti-SCL-70: 108 (0-99) • ANA: negative UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

10. Chest X-ray UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

11. Imaging Reports • 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

12. Working Diagnosis Interstitial lung disease, unknown etiology UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

13. 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

14. Final Diagnosis Usual Interstitial Pneumonia UNITED STATES DEPARTMENT OF VETERANS AFFAIRS

15. Clinical Correlations The NYU Internal Medicine Blog A Daily Dose of Medicine http://clinicalcorrelations.org UNITED STATES DEPARTMENT OF VETERANS AFFAIRS