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Case Studies from the Pulmonary Function Laboratory

Case Studies from the Pulmonary Function Laboratory. Focus Conference on Respiratory Care and Sleep Medicine, May 2013 Nashville, TN. Angela Lorenzo, MS, RRT, RPFT Respiratory Care Division School of Health Sciences. Indications for PFTs. Establish baseline of pulmonary function

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Case Studies from the Pulmonary Function Laboratory

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  1. Case Studies from the Pulmonary Function Laboratory Focus Conference on Respiratory Care and Sleep Medicine, May 2013 Nashville, TN Angela Lorenzo, MS, RRT, RPFT Respiratory Care Division School of Health Sciences

  2. Indications for PFTs • Establish baseline of pulmonary function • Determine presence/severity of disease • Monitor disease progression/improvement • Monitor response to therapy • Pre-operative assessment • Disability evaluations • Occupational lung disease

  3. Case 1: Ankylosing Spondylitis and MAC c/o DOE 53 y/o male, Ht 72 “, Wt 90 lbs, Smoker- 39 pk/yr hx, c/o DOE 4 Months ago released from hospital for hemoptysis, Rx’s with antibiotics & Home O2 Chest Imaging: LUL cavitating lesion with an enlarging mycetoma, new densities in RUL & LLL, widespread pulmonary fibrosis Sputum + for M. xenopi in past (now -)

  4. Case 1: Ankylosing Spondylitis and MAC c/o DOE Also has Crohn’s Disease and treated with 6MP (mercaptopurine) which in rare cases can cause pulmonary fibrosis Pt had been treated with Remicade in past (anti-TNF) associated with TB infections

  5. Case 1: Ankylosing Spondylitis and MAC FVC: 1.47 L (28%) FEV1: 1.37 L (33%) FEV1%: 93% MIP: 52% predicted MEP: 26% predicted Unable to perform DLCO or N2 washout due to leak Limited ability to open mouth-could not use mouthpiece

  6. Case 2: Alpha-1 Antitrypsin Deficiency • 64 y/o male with history of A-1AD • Has pulmonary and liver involvement • Up to 15% pts have liver involvement • Currently taking Aralast 5 g IV weekly • Alpha-1 proteinase inhibitor • Increased exercise tolerance since beginning A1PI therapy • Progressive weight loss has stabilized

  7. Case 2: A1AD • FVC: 4.47 L (98%) • FEV1: 2.63 L (74%) • FEV1%: 59% • FEF 25-75% 0.99 L/sec (29%) • PEFR 8.09 L/sec (93%) • TLC 7.64 L (111%) • FRC PL 5.00 L (141%) • RV 3.09 L (129%) • DLCO 70%; DLCO/Hb 67%; DLCO/VA 42%

  8. Alpha-1 antitrypsin is produced in the liver Protects the lungs from the effects of elastase A-1A inactivates elastase carried on WBCs in the lungs Elastase destroys alveoli Prolastin, Zemaira, Aralast Case 2: A1AD

  9. Case 2: A1AD

  10. Case 2: A1AD Diagnosed 6 years ago presenting with DOE, reactive erythrocytosis 2/2 hypoxia On A1PI 5 years Liver cirrhosis diagnosed 7 years ago, stable at this time Weight loss has stabilized No limitations on activity

  11. Case 3 CREST/ILD • 64 y/o male with CREST is being evaluated for ILD. • Pt also has goiter. • Previous PFTs WNL when pt tested to see if goiter was obstructing trachea

  12. FVC 2.60 L 60% FEV1 2.11 L 62% FEV1% 81% FEF25-75% 2.15 67% PEF 7.56 89% No bronchodilator Post-loops assessed effect of goiter on airway Case 3: CREST/ILD

  13. TLC 4.45L 66% VC 2.75 L 63% FRC N2 2.63L 74% ERV 0.94 L 65% RV 1.69 L 69% RV/TLC 38% 98% DLCO 58% DLCO/VA 52% Case 3: CREST/ILD

  14. Case 4: Asthma, Allergies, DM2 • 54 y/o asthmatic with hx DM tested • Pulmonary Medication profile: advair singulair, albuterol prn (used 5 canisters in a 6 month time frame) • At age 49 pt sought treatment for a suspected latex allergy • Pt had known seasonal allergies.

  15. Case 4: Asthma, Allergies, DM2 • Allergy skin testing + for pollens, grass, trees, dust mites, feathers, dogs, cats, tree nuts, shell fish, some other foods • Blood tests were – for latex x 2, + some foods • Pt agreed to Immunotherapy. • 1st PFT 4 years prior to beginning IT • 2nd PFT 2 years IT • FeNO 4 years IT

  16. FVC 2.68L 85% FEV1 1.98L 75% FEV1% 74% FEF25-75 1.44 27% L/SEC PEFR 6.32 104% Case 4: Asthma, Allergies, DM2 Pre/Post

  17. FVC 2.58L 85% FEV1 2.52L 79% FEV1% 80% FEF25-75 2.82 57% L/SEC PEFR 5.96 134% Case 4: Asthma, Allergies, DM2

  18. 2003 FVC 2.68L 85% FEV1 1.98L 75% FEV1% 74% FEF25-75 1.44 27% L/SEC PEFR 6.32 104% L/SEC 2009 FVC 2.58L 85% FEV1 2.52L 79% FEV1% 80% FEF25-75 2.82 57% L/SEC PEFR 5.96 134% L/SEC Case 4: Asthma, Allergies, DM2

  19. Case 4: Asthma, Allergies, DM2 2012 worsening symptoms after URI 2 weeks after URI, sinus complaints, productive cough. No fever, chills Baseline FeNO 17 ppb Peak symptoms FeNO 54 ppb Advair increased to 250/50: FeNO 34 ppb Advair increased to 500/50: FeNO 17 ppb Baseline dose 100/50 resumed: FeNO 17 ppb

  20. Case 4: Asthma, Allergies, DM2

  21. Case 5 72 y/o physician “curious” to see what cigarettes have done to her lungs and what a PFT feels like. Approx 75 pack year history No respiratory complaints

  22. Case 5

  23. Case 5

  24. Case 6: Bronchiectasis 49 y/o male with hx severe COPD, pulm TB for which he began tx 1994 TB relapsed due to noncompliance. Completed multi-drug tx > 1 yr 1996 Now has stable biapical scarring/cavitation Sputum + MAC 3 of 4 cultures, no MAI tx 25 pack year smoking hx, quit 8 years ago

  25. Case 6: Bronchiectasis Presents for f/up and sputum induction No wt loss, night sweats, fever, chills Good appetite, good ET >3-4 flights stairs + phlegm varies clear to brownish, no blood Chest CT: extensive bilat old granulomatous disease with bullous and cavitary changes, significant volume loss in upper lobes, bronchiectatic changes, fibrosis/scarring noted

  26. Case 6: Bronchiectasis Pt began Tx with spiriva, foradil, asmanex and albuterol prn in 2007. Asmnex d/c’d 2009

  27. Case 7: Parkinson’s Disease 62 y/o male with hx Parkinson’s disease (1998) eosinophilic PNA 2006-nml eos since 2008 Worsening SOB at rest, band-like radiating chest tightness that has increased over the last several weeks and gets worse as his Parkinson’s meds wear off Parkinson’s meds: carbidopa levodopa (Sinemet) q 2h (past 8 years), Selegiline (4 years); past use comtan d/c’d 2/2 side effects

  28. Case 7: Parkinson’s Disease

  29. Case 7: Parkinson’s Disease Mask facies, tachyneic, speaks in short sentences, lungs clear with shallow respirations As dopa wore off, PFT pattern changed to restricted- muscle rigidity affecting respiratory muscles which is rare Dyspnea is a described side effect of carbidopa levodopa

  30. Case 7: Parkinson’s Disease

  31. Case 8: Amiodarone • 79 y/o male with h/o cardiomyopathy and subsequent Afib. • Pt now on amiodarone • Pt c/o DOE • The following tests were ordered: spirometry and static lung volumes • DLCO was not ordered • Amiodarone known to cause pulmonary fibrosis

  32. FVC 2.68L 85% FEV1 1.98L 75% FEV1% 74% FEF25-75 1.44 27% L/SEC PEFR 6.32 104% L/SEC Case 8 : Amiodarone

  33. TLC 7.49L 94% VC 4.68L 95% FRC PL 3.80L 95% ERV 0.99L 61% RV 2.81L 95% RV/TLC 38% 84% Case 8 : Amiodarone

  34. Case 8 : Amiodarone • DLCO: 41% predicted • Discussion followed: was decreased DLCO due to interstitial edema 2o to cardiac failure or lung disease? • Side effects: • Interstitial pneumonitis, fibrosis • Dyspnea, non-productive cough - common presenting symptoms • Decreased TLC and DLCO

  35. Case 9: DOE, CLL, Lymphoma • 63 y/o male with Small Cell Lymphoma and Chronic Lymphocytic Leukemia c/o DOE and persistent unprod cough, 20 pack year hx, quite 11 years ago • Rituximab: cardiac= angina, arrhythmia; pulm = Obliterans bronchiolitis, pneumonitis • Fludarabine: pulm- cough, hypersensitivity reactions, pulmonary fibrosis, interstitial infiltrates • Cytoxan: cardiac- cardiomyopathy; pulm – interstitial pneumonia

  36. FVC 2.90L 59% FEV1 2.12L 56% FEV1% 73% FEF25-75 1.50 43% PEFR 6.02 67% Case 9: DOE with CLL, Lymphoma

  37. TLC 4.74L 63% VC 2.97L 60% FRC PL 2.54L 68% ERV 0.77L 47% RV 1.77L 70% RV/TLC 37% 90% DLCO (Hb 10.8) 44% DLCO/VA 49% Case 9: DOE with CLL, Lymphoma

  38. Anti-Neoplastic Drugs • Hypersensitivity reactions: characterized by cough, SOB, bronchospasm, urticaria, allergic alveolitis/pneumonitis • Procarbazine, Azathioprine (Imuran), Asparginase • Pulmonary fibrosis: • Busulfan (Myleran), Cyclophosphamide (Cytoxan), Bleomycin, Chlorambucil (Leukeran), Melphalan

  39. Case 10: Lung Cancer/Resection 68 y/o male referred to PFT Lab for pre-op evaluation for LUL lung cancer Pt is a 40 pack year ex-smoker who quit 10 years ago. DOE, Productive cough He presented nearly one year later for follow up PFTs

  40. Case 10: Lung Cancer/Resection

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