Case Studies from the Pulmonary Function Laboratory

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Case Studies from the Pulmonary
Function Laboratory
Angela Lorenzo, MS, RRT, RPFT
Respiratory Care Division
School of Health Sciences
Focus Conference on Respiratory Care and Sleep
Medicine, May 2013 Nashville, TN
Indications for PFTs
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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
Case 1: Ankylosing Spondylitis and MAC
c/o DOE
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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 -)
Case 1: Ankylosing Spondylitis
and MAC c/o DOE
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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 (antiTNF) associated with TB infections
Case 1: Ankylosing Spondylitis and MAC
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FVC: 1.47 L (28%)
FEV1: 1.37 L (33%)
FEV1%: 93%
MIP: 52% predicted
MEP: 26% predicted
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Unable to perform
DLCO or N2 washout
due to leak
Limited ability to open
mouth-could not use
mouthpiece
Case 2: Alpha-1 Antitrypsin
Deficiency
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64 y/o male with history of A-1AD
Has pulmonary and liver involvement
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Currently taking Aralast 5 g IV weekly
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Up to 15% pts have liver involvement
Alpha-1 proteinase inhibitor
Increased exercise tolerance since beginning
A1PI therapy
Progressive weight loss has stabilized
Case 2: A1AD
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FVC:
FEV1:
FEV1%:
FEF 25-75%
PEFR
TLC
FRC PL
RV
DLCO 70%;
4.47 L
2.63 L
59%
0.99 L/sec
8.09 L/sec
7.64 L
5.00 L
3.09 L
DLCO/Hb 67%;
(98%)
(74%)
(29%)
(93%)
(111%)
(141%)
(129%)
DLCO/VA 42%
Case 2: A1AD
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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
Year
2009
1/2010
12/2010
FVC
3.53
4.47
4.14
92%
4.05
92%
FEV1
2.38
2.63
2.50
70%
2.51
73%
FEV1/FVC
67%
59%
60%
FRC
2012
62%
4.30
121%
4.53
129%
132%
3.62
149%
RV
3.31
3.09
3.17
DLCO
58%
70%
61%
58%
Case 2: A1AD
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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
Case 3 CREST/ILD
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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
Case 3: CREST/ILD
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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
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TLC
4.45L
VC
2.75 L
FRC N2 2.63L
ERV
0.94 L
RV
1.69 L
RV/TLC 38%
DLCO
DLCO/VA
66%
63%
74%
65%
69%
98%
58%
52%
Case 4: Asthma, Allergies, DM2
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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.
Case 4: Asthma, Allergies, DM2
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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
Case 4: Asthma, Allergies, DM2
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FVC
FEV1
FEV1%
FEF25-75
2.68L
1.98L
74%
1.44
85%
75%
27%
6.32
104%
L/SEC
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PEFR
Pre/Post
Case 4: Asthma, Allergies, DM2
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FVC
FEV1
FEV1%
FEF25-75
2.58L 85%
2.52L 79%
80%
2.82 57%
L/SEC
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PEFR
5.96
134%
Case 4: Asthma, Allergies, DM2
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2003
FVC
FEV1
FEV1%
FEF25-75
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2.68L
1.98L
74%
1.44
85%
75%
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27%
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L/SEC
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PEFR
L/SEC
2009
FVC
FEV1
FEV1%
FEF25-75
2.58L 85%
2.52L 79%
80%
2.82 57%
L/SEC
6.32
104%
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PEFR
L/SEC
5.96
134%
Case 4: Asthma, Allergies, DM2
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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
Case 4: Asthma, Allergies, DM2
Advair Dose
FeNO
Resting Blood Sugar
100/50
15-19
100-110
100/50
54
160’s
250/50
34
140’s
500/50
17
100
100/50
17-19
90-110
Case 5
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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
Case 5
Case 5
Case 6: Bronchiectasis
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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
Case 6: Bronchiectasis
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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
Case 6: Bronchiectasis
Year
2007
2009
FVC
3.12L
63%
3.89L
76%
FEV1
1.27L
32%
1.10L
27%
FEV1/FVC
41%
28%
SVC
92%
3.89L
IC
75%
2.70L
ERV
153%
1.19L
70%
FRCpleth
130%
4.35L
122%
RV
145%
3.17L
144%
DLCO/VA
WNL
64%
R/A Pulsox
97%
98%
76%
Pt began Tx with spiriva, foradil, asmanex and albuterol
prn in 2007. Asmnex d/c’d 2009
Case 7: Parkinson’s Disease
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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
Case 7: Parkinson’s Disease
2 hours later,
feeling SOB
FVC
After
Parkinson’s
Meds
3.98 L
FEV1
2.98 L
1.77 L
FEV1FVC
75%
99%
1.79 L
Case 7: Parkinson’s Disease
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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
Case 7: Parkinson’s Disease
Case 8: Amiodarone
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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
Case 8 : Amiodarone
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FVC
FEV1
FEV1%
FEF25-75
2.68L
1.98L
74%
1.44
85%
75%
27%
6.32
104%
L/SEC
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PEFR
L/SEC
Case 8 : Amiodarone
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TLC
7.49L
VC
4.68L
FRC PL 3.80L
ERV
0.99L
RV
2.81L
RV/TLC 38%
94%
95%
95%
61%
95%
84%
Case 8 : Amiodarone
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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
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Case 9: DOE, CLL, Lymphoma
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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
Case 9: DOE with CLL, Lymphoma
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FVC
FEV1
FEV1%
FEF25-75
PEFR
2.90L
2.12L
73%
1.50
6.02
59%
56%
43%
67%
Case 9: DOE with CLL, Lymphoma
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TLC
4.74L
VC
2.97L
FRC PL
2.54L
ERV
0.77L
RV
1.77L
RV/TLC 37%
DLCO (Hb 10.8)
DLCO/VA
63%
60%
68%
47%
70%
90%
44%
49%
Anti-Neoplastic Drugs
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Hypersensitivity reactions: characterized by
cough, SOB, bronchospasm, urticaria, allergic
alveolitis/pneumonitis
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Procarbazine, Azathioprine (Imuran), Asparginase
Pulmonary fibrosis:
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Busulfan (Myleran), Cyclophosphamide (Cytoxan),
Bleomycin, Chlorambucil (Leukeran), Melphalan
Case 10: Lung Cancer/Resection
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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
Case 10: Lung Cancer/Resection
Pre-op
s/p Left Upper
Lobectomy
FVC
2.44L
53%
2.27L
50%
FEV1
1.55L
44%
1.47L
42%
FEV1/FVC
64%
FEF 25-75
0.58L/Sec
18%
0.73L/Sec
TLC
4.18L
59%
3.89L
54%
VC
2.56L
59%
2.34L
51%
FRCpleth
2.29L
62%
2.24L
61%
ERV
0.66L
44%
0.69L
46%
RV
1.63L
63%
1.55L
60%
RV/TLV
39%
DLCO
65%
40%
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