Transcript The Spectrum Of pulmonary diseases

The spectrum of pulmonary diseases
Abubakr A Bajwa. MD, FCCP
Associate Professor of Medicine
Division Chief
Pulmonary, Critical Care and Sleep Medicine
Director Pulmonary Hypertension and Interstitial Lung Disease Clinic
University of Florida College of Medicine/Jacksonville
Objectives
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Describe the spectrum of common pulmonary diseases
Explain physiology of diseases
Describe various manifestations
Discuss briefly about evaluation and management of
various pulmonary disorders
Slovenia
Germany Brazil Ireland
United States Australia
Canada
Philippines Mongolia
Moldova Norway Thailand
Kazakhstan
United Kingdom Syria
Italy
New Zealand
Argentina
Mexico
United Arab Emirates
Poland Korea
Portugal
Greece
Bangladesh
Saudi Arabia
Yugoslavia Croatia
Austria
Taiwan ROC
Yeman
Malta
China
South Africa
Hong Kong ROC
Nepal Chile
Israel
Pakistan Russia
GOLD National Leaders
Peru
Japan
Netherlands
Egypt France
Venezuela
KyrgyzstanGeorgia
Macedonia Albania
Iceland
Denmark
Turkey Czech
Belgium
Slovakia
Republic
Singapore Spain
Columbia
Ukraine
Romania
Sweden
Uruguay
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Vietnam
Switzerland India
GOLD Website Address
http://www.goldcopd.org
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Confusion
Over
Pulmonary
Diagnosis
G lobal Initiative for Chronic
O bstructive
L ung
D isease
© 2014 Global Initiative for Chronic Obstructive Lung Disease
GOLD Objectives
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Increase awareness of COPD among
health professionals, health
authorities, and the general public
Improve diagnosis, management and
prevention
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Decrease morbidity and mortality
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Stimulate research
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and
Prevention of COPD, 2014: Chapters
Updated 2014
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Definition and Overview
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Diagnosis and Assessment
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Therapeutic Options
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Manage Stable COPD
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Manage Exacerbations
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Manage Comorbidities
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Asthma COPD Overlap
Syndrome (ACOS)
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and
Prevention of COPD, 2014: Chapters
Updated 2014
n
Definition and Overview
n
Diagnosis and Assessment
n
Therapeutic Options
n
Manage Stable COPD
n
Manage Exacerbations
n
Manage Comorbidities
n
Asthma COPD Overlap
Syndrome (ACOS)
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Definition of COPD
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COPD, a common preventable and treatable
disease, is characterized by persistent airflow
limitation that is usually progressive and
associated with an enhanced chronic
inflammatory response in the airways and the
lung to noxious particles or gases.
Exacerbations and comorbidities contribute to
the overall severity in individual patients.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Mechanisms Underlying
Airflow Limitation in COPD
Small Airways Disease
Parenchymal Destruction
• Airway inflammation
• Airway fibrosis, luminal plugs
• Increased airway resistance
• Loss of alveolar attachments
• Decrease of elastic recoil
AIRFLOW LIMITATION
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Burden of COPD
 COPD is a leading cause of morbidity and
mortality worldwide.
 The burden of COPD is projected to increase
in coming decades due to continued
exposure to COPD risk factors and the aging
of the world’s population.
 COPD is associated with significant economic
burden.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Risk Factors for COPD
Genes
Infections
Socio-economic
status
Aging Populations
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and
Prevention of COPD, 2014: Chapters
Updated 2014
n
Definition and Overview
n
Diagnosis and Assessment
n
Therapeutic Options
n
Manage Stable COPD
n
Manage Exacerbations
n
Manage Comorbidities
n
Asthma COPD Overlap
Syndrome (ACOS)
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Diagnosis and Assessment: Key Points
 A clinical diagnosis of COPD should be
considered in any patient who has dyspnea,
chronic cough or sputum production, and a
history of exposure to risk factors for the
disease.
 Spirometry is required to make the diagnosis;
the presence of a post-bronchodilator FEV1/FVC
< 0.70 confirms the presence of persistent
airflow limitation and thus of COPD.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Diagnosis and Assessment: Key Points
 The goals of COPD assessment are to determine
the severity of the disease, including the severity of
airflow limitation, the impact on the patient’s health
status, and the risk of future events.
 Comorbidities occur frequently in COPD patients,
and should be actively looked for and treated
appropriately if present.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Diagnosis of COPD
SYMPTOMS
shortness of breath
chronic cough
sputum
EXPOSURE TO RISK
FACTORS
tobacco
occupation
indoor/outdoor pollution
è
SPIROMETRY: Required to establish
diagnosis
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of Airflow Limitation:
Spirometry
 Spirometry should be performed after the
administration of an adequate dose of a shortacting inhaled bronchodilator to minimize
variability.
 A post-bronchodilator FEV1/FVC < 0.70 confirms
the presence of airflow limitation.
 Where possible, values should be compared to
age-related normal values to avoid overdiagnosis
of COPD in the elderly.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Spirometry: Normal Trace Showing
FEV1 and FVC
FVC
Volume, liters
5
4
FEV1 = 4L
3
FVC = 5L
2
FEV1/FVC = 0.8
1
1
2
3
4
5
6
Time, sec
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Spirometry: Obstructive Disease
Normal
5
Volume, liters
4
3
FEV1 = 1.8L
2
FVC = 3.2L
FEV1/FVC = 0.56
1
1
2
3
4
5
6
Time, seconds
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Obstructive
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of COPD: Goals
Determine the severity of the disease, its
impact on the patient’s health status and the
risk of future events (for example
exacerbations) to guide therapy. Consider the
following aspects of the disease separately:




current level of patient’s symptoms
severity of the spirometric abnormality
frequency of exacerbations
presence of comorbidities.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of COPD
 Assess symptoms
 Assess degree of airflow
limitation using spirometry
 Assess risk of exacerbations
 Assess comorbidities
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Symptoms of COPD
The characteristic symptoms of COPD are chronic and
progressive dyspnea, cough, and sputum production
that can be variable from day-to-day.
Dyspnea: Progressive, persistent and characteristically
worse with exercise.
Chronic cough: May be intermittent and may be
unproductive.
Chronic sputum production: COPD patients commonly
cough up sputum.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of COPD
 Assess symptoms
Assess degree of airflow limitation using
spirometry
COPD Assessment Test (CAT)
Assess risk of exacerbations
Assess comorbidities or
Clinical COPD Questionnaire (CCQ)
or
mMRC Breathlessness scale
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of Symptoms
COPD Assessment Test (CAT): An 8-item
measure of health status impairment in COPD
(http://catestonline.org).
Clinical COPD Questionnaire (CCQ): Self-
administered questionnaire developed to
measure clinical control in patients with COPD
(http://www.ccq.nl).
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of Symptoms
Breathlessness Measurement using the
Modified British Medical Research Council
(mMRC) Questionnaire: relates well to other
measures of health status and predicts future
mortality risk.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Modified MRC (mMRC)Questionnaire
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of COPD
 Assess symptoms
 Assess degree of airflow limitation
using spirometry
Assess
risk of exacerbations
Use spirometry
for grading severity
Assess
comorbidities
according
to spirometry, using four
grades split at 80%, 50% and 30% of
predicted value
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Classification of Severity of Airflow
Limitation in COPD*
In patients with FEV1/FVC < 0.70:
GOLD 1: Mild
FEV1 > 80% predicted
GOLD 2: Moderate
50% < FEV1 < 80% predicted
GOLD 3: Severe
30% < FEV1 < 50% predicted
GOLD 4: Very Severe FEV1 < 30% predicted
*Based on Post-Bronchodilator FEV1
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assessment of COPD
 Assess symptoms
 Assess degree of airflow limitation
using spirometry
 Assess risk of exacerbations
Assess
comorbidities
Use history
of exacerbations and spirometry.
Two exacerbations or more within the last year
or an FEV1 < 50 % of predicted value are
indicators of high risk. Hospitalization for a COPD
exacerbation associated with increased risk of death.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Assess Risk of Exacerbations
To assess risk of exacerbations use history of
exacerbations and spirometry:
 Two or more exacerbations within the last
year or an FEV1 < 50 % of predicted value
are indicators of high risk.
 One or more hospitalizations for COPD
exacerbation should be considered high
risk.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Combined Assessment of COPD
 Assess symptoms
 Assess degree of airflow limitation using
spirometry
 Assess risk of exacerbations
Combine these assessments for the
purpose of improving management of COPD
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
4
(C)
(D)
3
2
(A)
(B)
1
or
> 1 leading
to hospital
admission
1 (not leading
to hospital
admission)
0
CAT < 10
CAT > 10
Symptoms
mMRC > 2
mMRC 0–1
Breathlessness
© 2014 Global Initiative for Chronic Obstructive Lung Disease
(Exacerbation history)
≥2
Risk
(GOLD Classification of Airflow Limitation))
Risk
Combined Assessment of COPD
Global Strategy for Diagnosis, Management and Prevention of COPD
Combined Assessment of COPD
Assess symptoms first
(C)
(D)
(A)
(B)
CAT < 10
CAT > 10
Symptoms
mMRC 0–1 mMRC > 2
Breathlessness
If CAT < 10 or mMRC 0-1:
Less
Symptoms/breathlessness (A
or C)
If CAT > 10 or mMRC > 2:
More
Symptoms/breathlessness
(B or D)
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Combined Assessment of COPD
4
3
(C)
(D)
or
> 1 leading
to hospital
admission
(B)
1 (not leading
to hospital
admission)
0
2
(A)
1
CAT < 10
(Exacerbation history)
≥2
Risk
(GOLD Classification of Airflow Limitation)
Risk
Assess risk of exacerbations next
If GOLD 3 or 4 or ≥ 2
exacerbations per year or
> 1 leading to hospital
admission:
High Risk (C or D)
If GOLD 1 or 2 and only
0 or 1 exacerbations per
year (not leading to
hospital admission):
Low Risk (A or B)
CAT > 10
Symptoms
mMRC 0–1
mMRC > 2
Breathlessness
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
4
(C)
(D)
3
2
(A)
(B)
1
or
> 1 leading
to hospital
admission
1 (not leading
to hospital
admission)
0
CAT < 10
CAT > 10
Symptoms
mMRC > 2
mMRC 0–1
Breathlessness
© 2014 Global Initiative for Chronic Obstructive Lung Disease
(Exacerbation history)
≥2
Risk
(GOLD Classification of Airflow Limitation))
Risk
Combined Assessment of COPD
Global Strategy for Diagnosis, Management and
Prevention of COPD
Combined Assessment
of COPD
When assessing risk, choose the highest risk
according to GOLD grade or exacerbation
history. One or more hospitalizations for COPD
exacerbations should be considered high risk.)
Patient
Characteristic
Spirometric
Classification
Exacerbations
per year
CAT
mMRC
A
Low Risk
Less Symptoms
GOLD 1-2
≤1
< 10
0-1
B
Low Risk
More Symptoms
GOLD 1-2
≤1
> 10
>2
C
High Risk
Less Symptoms
GOLD 3-4
>2
< 10
0-1
D
High Risk
More Symptoms
GOLD 3-4
>2
> 10
© 2014 Global Initiative for Chronic Obstructive Lung Disease
>2
Global Strategy for Diagnosis, Management and Prevention of COPD
Assess COPD Comorbidities
COPD patients are at increased risk for:
•
•
•
•
•
•
•
Cardiovascular diseases
Osteoporosis
Respiratory infections
Anxiety and Depression
Diabetes
Lung cancer
Bronchiectasis
These comorbid conditions may influence mortality and
hospitalizations and should be looked for routinely, and
treated appropriately.
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Differential Diagnosis:
COPD and Asthma
COPD
ASTHMA
• Onset in mid-life
• Symptoms slowly
•
• Onset early in life (often
childhood)
progressive
• Symptoms vary from day to day
Long smoking history
• Symptoms worse at night/early
morning
• Allergy, rhinitis, and/or eczema
also present
• Family history of asthma
© 2014 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Additional Investigations
Chest X-ray: Seldom diagnostic but valuable to exclude
alternative diagnoses and establish presence of significant
comorbidities.
Lung Volumes and Diffusing Capacity: Help to characterize
severity, but not essential to patient management.
Oximetry and Arterial Blood Gases: Pulse oximetry can be
used to evaluate a patient’s oxygen saturation and need for
supplemental oxygen therapy.
Alpha-1 Antitrypsin Deficiency Screening: Perform when COPD
develops in patients of Caucasian descent under 45 years or
with a strong family history of COPD.
© 2013 Global Initiative for Chronic Obstructive Lung Disease
Global Strategy for Diagnosis, Management and Prevention of COPD
Additional Investigations
Exercise Testing: Objectively measured exercise
impairment, assessed by a reduction in self-paced walking
distance (such as the 6 min walking test) or during
incremental exercise testing in a laboratory, is a powerful
indicator of health status impairment and predictor of
prognosis.
Composite Scores: Several variables (FEV1, exercise
tolerance assessed by walking distance or peak oxygen
consumption, weight loss and reduction in the arterial
oxygen tension) identify patients at increased risk for
mortality.
© 2013 Global Initiative for Chronic Obstructive Lung Disease
Bronchiectasis
Abubakr A. Bajwa MD, FCCP
Pulmonary, Critical Care, Sleep Medicine
Objectives
• Recognize bronchiectasis with its pulmonary and systemic manifestations
• Review diagnostic evaluation
• Review treatment strategies for acute and chronic phase
Case 1
• 59 woman with TB in past in her 20s
• Now with cough and sputum production since her 40s
• 1-2 hospitalizations/yr
Case 2
•
•
•
•
24 woman with chronic cough and wheezing
Diagnosed with asthma/chronic bronchitis/chronic sinusitis
Intermittent treatment with BDs and antibiotics
Greenish-yellow phlegm production
Case 3
• 71 woman non smoker with chronic cough for 4 years
• Persistant cough and 15 lbs weight loss
• Sputum has MAI
Prevalence of non-CF bronchiectasis
Definition
• Permanently dilated airways
• Result from various processes
– Congenital
– Post-infectious
– Anatomical
Other signs
• Signet ring
• Lack of bronchial tapering
Clinical picture
• Most common:
– Chronic cough
– Mucopurulent sputum
• Less common:
–
–
–
–
–
–
Dyspnea
Hemoptysis
Chest pain
Fever
Fatigue
Weight loss
Bronchiectasis and COPD
• Out of 54 stable patients with COPD 50% had bronchiectasis on HRCT
–
–
–
–
–
Mean age 69
Mean FEV1 0.96L
More severe exacerbations
More bacterial colonizations
Increased sputum inflammatory markers
Focal bronchiectasis
• Post-infectious:
– Bacterial
– Viral
– Tuberculous (MTB, NTM)
• Airway obstruction:
– Foreign body
– Bronchial stricture (RML syndrome)
– Endobronchial mass
Diffuse bronchiectasis
• Post infectious:
– Measeles, pertussis
– Mycobacterial
• Postinflammatory:
– Sarcoidosis
• Inhalation:
– Aspiration
– Inhalation injury
• Congenital syndromes:
– CF
– Primary ciliary dyskinesia
• Immunodeficiency states:
– Immunoglobulin deficiency
– HIV
• Immune mediated diseases:
– ABPA
– RA
– IBD
Natural history
• Martinez-Garcia Chest 2007
– Mean age 70, 48.7% males
– 52.7 ml decline in FEV1 per year
– Faster decline:
• Psuedomonas
• Frequent exacerbations
• Increased inflammatory markers
– If in ICU then 40% mortality
Transplantation
• Royal Brompton Hospital:
– 22 patients from 1998-2001
– 1 year survival 68%
Pulmonary Manifestations of
Connective Tissue Diseases
Abubakr A Bajwa MD
Pulmonary , Critical Care and Sleep Medicine
Types of Pulmonary Manifestations
 Pulmonary Vascular and Alveolar HGE syndromes
 Interstitial
 Pleural
 Airway
1. Vascular
 Pulmonary Arterial Hypertension
 Alveolar Hemorrhage Syndrome/Vasculitis/Capillaritis
Pulmonary Arterial Hypertension
Pathogenesis
RISK FACTORS AND ASSOCIATED
CONDITIONS
VASCULAR INJURY
Endothelial Dysfunction
Collagen Vascular Disease
Congenital Heart Disease
Portal Hypertension
HIV Infection
SUSCEPTIBILITY
Drugs and Toxins
Abnormal BMPR2 Gene
Pregnancy
Loss of Response to Short-Acting
Vasodilator Trial
↓Nitric Oxide Synthase
↓Prostacyclin Production
↑Thromboxane Production
↑Endothelin 1 Production
Other Genetic Factors
Smooth Muscle
Hypertrophy
Vascular Smooth Muscle Dysfunction
Impaired Voltage-Gated
Potassium Channel (KV1.5)
Adventitia
Smooth Muscle
Hypertrophy
Media
DISEASE PROGRESSION
In Situ
Thrombosis
Intima
Early Intimal
Proliferation
Plexiform
Lesion
Vasoconstriction
NORMAL
Adapted from Gaine S. JAMA. 2000;284:3160-3168.
Adventitial and Intimal
Proliferation
REVERSIBLE DISEASE
Advanced Vascular Lesion
IRREVERSIBLE DISEASE
 Limited scleroderma (CREST)
 Most common
 Prevalence of PH, ranging from 4.9% to 38% with a mean 16%
 PH is the cause of death in up to 50% of patients
 Diffuse scleroderma
 Less common
 Often seen with patients with the nucleolar antibody, anti-U3-RNP
REVEAL Database:
Most Frequent Symptoms at Diagnosis
11%
11%
13%
13.0%
14%
16.0%
20%
23.0%
20%
21.0%
20%
23%
27%
24.0%
29%
26.0%
Dyspnea at rest
Cough
Dizzy/lightheaded
Presyncope/syncope
Edema
Chest pain/discomfort
Other
Fatigue
IPAH
APAH
n = 1479
83%
84.0%
Dyspnea on exertion
0
Elliott EG, et al. Chest. 2007;132(4 suppl):631S.
25
50
75
Incidence (%)
100
PAH Disease Progression:
Prognostic Factors for Risk
Lower Risk
No
Higher Risk
Yes
Progression
Gradual
Rapid
WHO Class
II, III
IV
6-minute walk distance
> 380 m
< 325 m
Brain natriuretic peptide
< 180 pg/mL
Minimal right ventricular
dysfunction
> 180 pg/mL
Pericardial effusion;
significant right ventricular
dysfunction
Normal/near normal RAP
(< 12 mm Hg) and CI (>
2.5 L/min/m2)
High RAP, Low CI
Evidence of RV failure
Echo findings
Hemodynamics
McLaughlin VV, et al. Circulation. 2006;114:1417-1431.
DAH /Vasculitis/Capillaritis
Definitions
 Diffuse alveolar hemorrhage
 Syndrome of diffuse bleeding into the lungs at the alveolar level
 Histopathologically - flooding of alveoli with RBCs
 Pulmonary Vasculitis
 Histopathologic occurrence of vasculitis occurring at any level of
the pulmonary vasculature
 Pulmonary Capillaritis
 Histopathologic occurrence of vasculitis occurring at the
capillary/alveolar level
DAH
 DAH is a Syndrome, not a diagnosis
 A search for underlying cause is imperative
 Fluminant presentations are frequent
 Should always be considered life threatening
 Symptoms
 Dyspnea and cough
 Hemoptysis (absent in up to 1/3)
 Fever usually due to underlying illness
 Findings
 Anemia (if chronic or subacute)
 Hypoxemia
DAH - radiographs
 Bilateral alveolar
infiltrates
 Can start unilateral
 Can be patchy in more
sub-acute presentations
A consideration of possible causes…
 Exposures
 Trimellitic anhydride, pyromellitic dianhydrate, crack cocaine,
smoking, …
 Abciximab, penicillamine, sirolimus …
 Underlying diseases / conditions
 Autoimmune / rheumatologic conditions
 Cardiac disease – e.g. mitral stenosis
 Coagulopathy (severe and rare as isolated cause)
 Post-trauma
 Recent BMT or hematologic malignancies
 Immunosuppression
 Endobronchial bleed
Treatment Concepts
 Stabilization of respiratory status
 Exclusion of infection
 Confirmation of DAH
 Identification of contributing comorbidities or underlying
systemic disorders
 Timely implementation of therapy
Treatment concepts
 Autoimmune / Vasculitic processes
 Induction of remission…
 Maintenance of remission…
 Methotrexate
 Azathioprine
 Mycophenolate mofetil
2. Interstitial
 Scleroderma
 Rheumatoid Arthritis
 Dermatopolymyositis
 Sjogren’s Syndrome
 Mixed Connective Tissue Disease
Scleroderma
 ILD may occur in either limited or diffuse cutaneous .
 70–80% of patients exhibit pathological abnormalities of pulmonary fibrosis at
autopsy.
 The incidence of radiographically detectable ILD varies from 25–65%.
 Pathologically similar to UIP.
Rheumatoid Arthritis
 Initial radiographical studies found a low incidence of 1.6 –5% of ILD in RA.
 Decreased Dlco found in 41% of patients
 50% exhibited features of fibrosis associated with lymphoid infiltrates in lung biopsy.
 Lung biopsy performed in a group of unselected patients demonstrated interstitial
lesions in 80% of cases
 Nearly half were asymptomatic .
 Arthritis precedes lung disease in 80%.
Risk Factors:
 Rheumatoid nodules
 High RF titres
 ANA titres
 Non M1M1 alpha 1 antitrypsin phenotypes
Radiological Pattern
 Early acinar pattern.
 Nodular-reticular infiltrates.
 Lower lobe predominance.
 End-stage fibrosis results in the
classical honeycombing pattern.
Pathological pattern in RA-ILD
 UIP – predominant
 Peribronchovascular and interlobular lymphoid hyperplasia
 Cellular NSIP
 DIP
 Ig and RF deposit on immuno-staining
Rheumatoid-pneumoconiosis
 Circulating rheumatoid factors are found in:
 25% of patients with pneumoconiosis.
 70% of those with Caplan's syndrome.
 Progressive massive fibrosis develops in 50% of miners with RA.
Dermatopolymyositis
 ILD seen in 5-9% of cases.
 Lung involvement may precede muscle involvement in 30%.
 3 distinct patterns seen:
 Rapidly progressive with acute fever, dyspnea and lung infiltrates similar to a Hamman-
Rich-syndrome.
 Slowly progressive dyspnea upon exertion with chest radiographical abnormalities.
 Some patients may have no pulmonary symptoms, but abnormal radiographs and/or
PFTs.
Evaluation
 Anti-Jo-1 antibody – although maybe absent in some.
 Anti PL-7 or PL-12 antibody.
 Cardiopulmonary exercise testing to differentiate cause of dyspnea:
 Muscular weakness.
 ILD.
 Pulmonary HTN.
Pathological Patterns
 Cryptogenic Organizing Pneumonia (COP) – Consolidation.
 UIP – Parenchymal bands, sub-pleural bands, peri-bronchovascular and inter-septal
thickening.
 DAD – Ground glass.
Treatment Concepts
 Steroids – for all presentations except UIP.
 UIP - DPM 5 yr survival – 33%.
 Cyclophosphamide.
Sjögren's syndrome




Lymphocytic Interstitial Pneumonitis – LIP.
Psuedolymphoma
Lymphoma
UIP
Management concepts
 BAL CD4/CD8 ratio is reduced – more frequent dyspnea, cough, PFTs
abnormalities and radiological ILD.
 Steroids for extraglandular involvement.
 Chlorambucil.
 LIP or Psuedolymphomas may evolve into lymphomas.
Mixed Connective Tissue Disease
 Pulmonary involvement has been described in 20–85% of patients.
 Spectrum of ILD predominantly similar to that of scleroderma i.e. UIP.
 DLCo decreased in 67%.
 Restrictive TLC in 50%.
Management concepts
 Steroids for acute excerbation
 Chlorambucil or cyclophosphamide in combination with steroids effective in 2/3
of patients in a series of 34.
Sullivan WD, Hurst DM, Harmon CE. A prospective evaluation emphasizing pulmonary
involvement in patients with mixed connective tissue disease. Medicine 1984;63:92–107
3. Pleural
 Pleural effusions/pleuritis
 Fibrothorax
Pleural effusion
 Typically exudative.
 Seen with:
 SLE
 Sjögren's syndrome
 Rheumatoid arthritis
Type of Effusion
Incidence
Congestive heart failure
500,000
Pneumonia (bacterial)
300,000
Malignant disease
200,000
Lung
60,000
Breast
50,000
Lymphoma
40,000
Other
50,000
Pulmonary embolization
150,000
Viral disease
100,000
Post-coronary artery bypass
surgery
60,000
Cirrhosis with ascites
50,000
Gastrointestinal disease
25,000
Collagen vascular disease
6,000
Tuberculosis
2,500
Asbestos exposure
2,000
Mesothelioma
1,500
Disease
Diagnostic pleural fluid tests
Empyema
Observation (pus, putrid odor); culture
Malignancy
Positive cytology
Lupus pleuritis
LE cells present; pleural fluid serum ANA >1.0
Tuberculous pleurisy
Positive AFB stain, culture
Esophageal rupture
High salivary amylase, pleural fluid acidosis (often as low
as 6.00)
Fungal pleurisy
Positive KOH stain, culture
Chylothorax
Triglycerides (>110 mg/dL); lipoprotein
electrophoresis (chylomicrons)
Hemothorax
Hematocrit (pleural fluid/blood >0.5)
Urinothorax
Creatinine (pleural fluid/serum >1.0)
Peritoneal dialysis
Protein (<1 g/dL); glucose (300 to 400 mg/dL)
Extravascular migration of central
venous catheter
Observation (milky if lipids are infused); pleural
fluid/serum glucose >1.0
Rheumatoid pleurisy
Characteristic cytology
Management concepts
 Diagnostic thoracentesis.
 Therapeutic thoracentesis – symptomatic relief.
 Rarely pleurodesis required for large recurrent effusions.
 Treat the underlying cause.
Fibrothorax
 Usually follows intense inflammation of the pleura
 Empyema or a hemothorax
 Tuberculosis
 Collagen vascular disease
 Uremia
 Paragonimiasis
 Drug reactions
 Ergot alkaloids (e.g., bromocriptine, pergolide, and methysergide).
 Asbestos exposure
4. Airway
 Bronchiolitis
 OP
Bronchiolitis
 Generic term is Bronchiolitis.
 Classification Scheme*
 Primary bronchiolar disorders
 Interstitial lung disease with a prominent bronchiolar component
 Large airways disease with a bronchiolar component
* Proposed by Ryu et al. Am J Respir Crit Care Med. 2003
Clinical picture
 Variable symptoms of dyspnea and cough.
 Obstruction on PFT’s.
 Normal chest radiograph.
High resolution CT scan
 Direct signs:
 Bronchiolar wall thickening (2-4 mm nodular and linear branching centrilobular
opacities)
 Bronchiolectasis
 Luminal impaction (tree-in-bud opacities)
 Indirect signs
 Subsegmental atelectasis
 Mosaic attenuation (expiratory films helpful)
 Air trapping
 Perfusion changes
Constrictive Bronchiolitis
 Also termed bronchiolitis obliterans, obliterative bronchiolitis.
 Pathologically characterized by a pattern of peribronchiolar fibrosis with complete
cicatrization of the bronchiolar lumen.
 Typically seen in RA.
OP
 6/40 lung biopsies in
RA
 Predominantly
reticonodular pattern
 Prognosis better if
RA-OP compared to
RA-ILD
Management concepts
 Consider addition of azithromycin in constrictive bronchiolitis when
immunosuppression alone fails.
 OP – highly responsive to steroids.