Transcript www.ingid.org

Lorraine Ozerovitch (MSc, BSc, RGN)
Clinical Nurse Specialist
in Respiratory Infection and Immunology
• Background on Bronchiectasis and CVID
• Reported quality of life outcomes in
Bronchiectasis and CVID
• Recent nursing research in patients with
CVID-Bx compared to Id-Bx
Lorraine Ozerovitch 2012 - INGID
• Unwell in childhood (bronchitis)
• Period of good health
• Age 30 to 40 years: persistent coughs & colds
• Copious volume of purulent tenacious sputum
• Lethargy or decreased exercise tolerance
• Breathlessness/ Chest tightness/ Pleuritic pain
Lorraine Ozerovitch 2012 - INGID
• HRCT with CXR and sinus XR
• Full PFT with reversibility
• Ciliary Studies (exhaled nasal & breath NO
testing/ EM)
• Sputum cultures for AFB and M,C&S
• Bloods (FBC, U’s&E’s, LFT’s, CRP, IgG, IgA, IgM,
IgE, SpAB, Asp Rast, Asp IgG,)
Lorraine Ozerovitch 2012 - INGID
• Serum protein electrophorectic strip
• Skin prick testing
• Shuttle walking Test/ Borg Breathlessness Scale
• St George’s Respiratory Questionnaire
• Physiotherapy review
• ENT review, bronchoscopy, video-fluoroscopy,
detailed immunology workup
Lorraine Ozerovitch 2012 - INGID
• 33pts with confirmed Bx on HRCT
• 25pts completed the “CAT” (Jones et al 2009)
CAT
Questions
Q1
Cough
Q2
Phlegm
Q3
Chest tightness
Q4
Breathlessness
Q5
Activities
Q6
Confidence
Q7
Sleep
Q8
Energy
Total
Score
Mean
(SD)
3.1
(0.95)
3.1
(0.91)
2.2
(1.30)
2.4
(1.64)
1.6
(1.82)
1.4
(1.8)
2.4
(1.71)
2.2
(1.45)
18.4
(9.72)
• CAT total scores correlated with worse
bronchiectasis on HRCT scans: extent and severity
of disease and airway wall thickness
Lorraine Ozerovitch 2012 - INGID
• Chronic dilatation of peripheral
airways, localised or widespread,
with loss of ciliated epithelium
• Occurs from destruction of muscular
and elastic components of the
bronchial walls
• Stationary mucus acts as a breeding
environment for bacteria to grow and
which is the source of recurrent
infections
Lorraine Ozerovitch 2012 - INGID
Lorraine Ozerovitch 2012 - INGID
• Common
– Pseudomonas aeruginosa
– Haemophilus influenzae
• Less common
–
–
–
–
–
Staphylococcus aureus
Streptococcus pneumoniae
Moraxella catarrhalis
Stenotrophamonas maltophilia
Klebsiella pneumoniae
Lorraine Ozerovitch 2012 - INGID
•
Cole (1986): The Vicious Cycle
A VICIOUS CYCLE OF INFECTION AND INFLAMMATION
Microbial Infection
(e.g.Haemophilus
influenzae,
Pseudomonas
aeruginosa)
Inflammation
Impaired Lung Defences
(e.g. Antibody
Deficiency, Primary
Ciliary Dyskinesia,
Cystic Fibrosis)
(Neutrophilic inflammation
causes damage to the tissue
through proteolytic enzymes
and oxidative stress)
Tissue Damage
(To epithelial cells and the
structure of the airway
wall leading to increased
mucus production which is
poorly cleared)
•
GOAL: Halt the bacterial process which in turn will impact on the inflammatory
process
•
The clinical course is variable
Lorraine Ozerovitch 2012 - INGID
• UK: 1:1000 hosp beds have a Bx pt (Sita-Lumsden
and Wilson 2009)
• US /NZ: 3.7-4.2: 100, 000 higher in the elderly
≥ 75yrs (Weycher et al 2005; Twiss et al 2005)
• 1000 die a year, 3% increase yr on yr (Roberts and
Hubbard 2010)
• BTS guideline (2010) may assist clinicians’
awareness in early detection and
management
Lorraine Ozerovitch 2012 - INGID
• Modern bronchiectasis is the end result of a
number of different pathologies
Bronchiectasis
Innate weakness in
the lung’s defenses
(e.g. PCD) or
deficiency in the
body’s ability to fight
infection (e.g. CVID)
Born with normal host
defenses then catches
a severe chest
infection (e.g.
tuberculosis) or
experience some other
insult to the airway
(e.g. smoke inhalation)
Acquire an excessive
immune response e.g.
allergic bronchopulmonary
aspergillosis (ABPA)
Lorraine Ozerovitch 2012 - INGID
Idiopathic – research
suggest an upset in
the immune response
causing an
exaggerated
inflammatory response
Causes
N (% of study popn)
Age (SD)
No: Males (% group)
Post Infection
52 (32)
49 (16)
17 (33)
Idiopathic
43 (26)
51 (14)
15 (35)
PCD
17 (10)
36 (13)
5 (29)
ABPA
13 (8)
54 (13)
6 (46)
Immune deficiency
11 (7)
47 (18)
1 (9)
Ulcerative Colitis
5 (3)
48 (20)
2 (40)
Young’s Syndrome
5 (3)
56 (5)
3 (60)
Pan Bronchiolitis
4 (2)
46 (21)
3 (75)
Yellow Nail Syndrome
4 (2)
55 (14)
2 (50)
Mycobacterium infections
4 (2)
62 (20)
0 (0)
Rheumatoid Arthritis
3 (2)
65( 4)
1 (33)
Aspiration
2( 1)
67 (13)
1(50)
Cystic Fibrosis
2 (1)
41 (13)
2 (100)
Total
165
49 (16)
58 (35)
ABPA = allergic brochopulmonary aspergillosis; PCD = primary ciliary dyskinesia
Shoemark et al (2007)
Lorraine Ozerovitch 2012 - INGID
• CVID is a heterogeneous group of conditions
characterised by: Antibody deficiency, Autoimmune
disorders and Granulomatous disease
• Commonest cause of primary antibody deficiency (PID)
• ESID criteria of CVID is “marked decrease in IgG and a
reduction of a least one isotypes; IgM or IgA”
• Average time between onset of symptoms and
diagnosis is 7 years in the UK
Lorraine Ozerovitch 2012 - INGID
• Prevalence 1 in 25, 000 individuals (Parks et al 2008)
• ESID database identifies 20.7% with PID has
CVID (Gathmann et al 2009)
• Mean age of CVID diagnosis is early 30’s
• RBH bx study identified 2% had CVID, 4% had
other immune deficiencies (Ozerovitch et al 2006)
Lorraine Ozerovitch 2012 - INGID
• Symptoms of cough and phlegm did not impact on
patients’ activity or confidence levels (Ozerovitch et al 2010)
• CRP and Total WCC are systemic markers of
inflammation that correlate with quality of life (Wilson et al
1998)
• Dyspnoea, FEV1 and sputum production are the
strongest factors of HRQL in stable bronchiectasis
patients (Martinez-Garcia et al 2005)
• Improved quality of life scores on follow-up compared
to time of referral (Ozerovitch et al 2004)
Lorraine Ozerovitch 2012 - INGID
• ↑HRQoL in patients with PID on IVIG – based on
self-reported measures of physical functioning
(Hedderick et al 1986)
• Patients reported on QoL, function and self-rated
health status with IgG therapy (Gardulf et al 1993)
• Studies remark of medical and clinical measures
of success measures (Gardulf et al 2006)
• Positive outcome in days off sick 6.1 compared to
23.3 (Eades-Perner et al 2007)
Lorraine Ozerovitch 2012 - INGID
• To assess QoL and functional ability in adult
stable patients with Bx due to CVID, compared
with historical controls with idiopathic
bronchiectasis (Ozerovitch et al 2004)
(Note: stable patients – no acute infective event requiring additional
antibiotics in the preceding month)
Lorraine Ozerovitch 2012 - INGID
• Bx confirmed on HRCT
• CVID confirmed by ESID criteria
• Severity of Bx noted by presence
of Pseudomonas aeruginosa (Pa)
• Analysis: Student t-tests
Lorraine Ozerovitch 2012 - INGID
• Spirometry
0
• SGRQ (Wilson et al 1997; Jones 2002)
• Exercise Capacity – SWT
(Singh et al 1992)
• The Borg Breathlessness Scale
(Borg 1982)
0.5
Very, very slight
(just noticeable)
1
Very slight
2
Slight
3
Moderate
4
Somewhat severe
5
Severe
6
7
• Sputum Results
Nothing at all
Very severe
8
(Wells et al 1993; Davies et al 2006;
Loebinger et al 2009)
Lorraine Ozerovitch 2012 - INGID
9
Very, very severe
(almost maximal)
10
Maximal
Lorraine Ozerovitch 2012 - INGID
Biomedical and social characteristics
of study participants
CVID associated bronchiectasis patients
within the immunology database
Study participants
Subjects excluded
Male participants (%)
Female participants (%)
Mean age
(SD)
(age range)
n of participants positive to Pa in the
preceding 6 months
Mean FEV1 % pred (SD)
Participants on IgG replacement therapy (%)
Lorraine Ozerovitch 2012 - INGID
CVID-Bx n(%) Id-Bx n(%)
34
0
22 (65)
12 (35)
9 (41)
13 (59)
36
0
12(33)
24 (67)
45yrs
(22.8)
(17-67)
54yrs
(11.30)
(32-75)
1 (5)
13 (36)
64 (26)
20 (91)
71 (28)
0
•
CVID-Bx patients had better scores for all SGRQ components and better SWT
distance, than Idiopathic Bx (these were clinically relevant although not statistically
significant).
Mean scores (SD):
CVID-Bx (n=22)
(Age range 17-67yrs)
Mean Scores (SD):
Idiopathic Bx (n=36)
(Age range 32-75yrs)
p:
Symptoms
58.3 (23.7)
65.8 (22.3)
0.23
Activity
37.0 (27.0)
45.3 (25.0)
0.23
Impact
27.8 (22.2)
34.4 (18.1)
0.22
Total Score
35.8 (23.0)
43.0 (18.7)
0.19
SWT (m)
513 (213.0)
SGRQ Components
432
Lorraine Ozerovitch 2012 - INGID
(157.7)
0.10
Total QofL Symptom QofL Activity QofL Impact QofL SWT metres
SCIG
(SD)
IVIG
(SD)
Mean Score
Mean Score
Mean Score
Mean Score
Mean Score
28.9
53.2
27.5
21.9
614*
-18.5
-17.2
-21.4
-19
-180.5
45.5
66.8
48.6
36.8
402*
-26.3
-29.0
-31.3
-28.8
-218.6
*There was only a statically significant difference between the
exercise tolerance scores (t-test p<0.03).
Lorraine Ozerovitch 2012 - INGID
Pathogen
CVID-Bx
(n=22)
CVID-Bx
(baseline)
Id-Bx
(N=36)
Id-Bx
(baseline)
1
1
14
9
0
4
2
0
2
8
1
5
Staphlococcus
Aureus
0
0
1
0
Stenotrophomonas
maltophilia
0
0
1
1
Moroxella
Catarrhalis
0
1
0
0
Multi Pathogens
(Hi & Strep/Staph)
0
2
0
3
No growth
1
3
5
16
18
3
12
2
Pseudomonas
aeruginosa
Steptococcus
pneumoniae
Haemophilus
influenzae
No sputum (well)
Lorraine Ozerovitch 2012 - INGID
• SWT –59% walked 4-600m; total range 50-940m
– Id-Bx 42% walked 4-600m; total range 0-890m
• Borg scores: 59% no breathlessness pre-exertion;
64% scored between 2 to 3 (slight to mod) post
exertion
– Id-Bx 33% no breathlessness pre-exertion; 39% scored
between 2 to 3 post exertion (Borg score≥4=39%)
• Spirometry: (FEV1 64% pred) correlated
negatively with Activity component only
– Id-Bx no relationship
Lorraine Ozerovitch 2012 - INGID
• Patients with CVID-Bx have clinically better health
status and functional ability than demographically
similar Id-Bx
• SCIG therapy was found to be associated with better
exercise tolerance and health status scores: however
• Small no of patients studied in each group
• ? Interaction of other confounding factors such as age
or presence/absence co-morbidities
• Little data on the utility of the Borg breathlessness scores in
this specialist area.
Lorraine Ozerovitch 2012 - INGID
• Baseline values obtained at diagnosis or
referral
• Comparison group rather than historical
controls
• Research study used disease specific QoL tool
- ?fitted to existed published work
Lorraine Ozerovitch 2012 - INGID
• This study provides the first report on the
impact of CVID-Bx on quality of life and
physical functioning using a disease specific
respiratory tool
• CVID-Bx QoL scores were generally better than
Id-Bx possibly due as a result of specific
therapy (IgG replacement) in the majority of
these patients
Lorraine Ozerovitch 2012 - INGID
• Patients
• Dr Peter Kelleher
• Dr Rob Wilson
• Samantha Prigmore
• Winston Banya
• Dr Jill Riley
Lorraine Ozerovitch 2012 - INGID