Restrictive lung diseases are broadly classified into parenchymal and extra-parenchymal groups Parenchymal restriction results from diseases involving the lung itself. Interstitial pulmonary fibrosis (IPF) is the most common and most serious among these parenchymal disorders. Collagenic diseases: like rheumatoid arthritis, scleroderma, systemic lupus erythematosus Granulomatous: as sarcoidosis Irradiation: eg, for cancer breast Resection of part of lung tissue leads to actual loss of functioning parenchyma. Ankylosing spondylitis: chronic inflammatory condition of the spine (vertebral column: spondylitis) with partial fusion of the vertebrae and limitation of their movement (ankylosis). The mobility of the joints between ribs and vertebral column (costovertebral joints) is encroached upon.
Reminder about respiratory functions In obstructive disease, air trapping leads to marked increase of RV. Consequently, FRC and TLC (which include RV) are also increased. Vital capacity as a whole may be slightly decreased, but limitation of expiratory airflow leads to marked diminution of the earliest (and normally greatest) part of expiratory FVC (FEV1). So, we get marked decrease of FEV1/FVC. In restrictive disease, all lung volumes and capacities are decreased.
Smoking index: number of packs consumed daily, multiplied by number of years of smoking
Parenchymal RLD Extraparenchymal RLD
FVC Decreased Decreased
MVV Normal Decreased
DLCO Decreased Normal
FVC: Forced Vital Capacity
MVV: Maximum Voluntary Ventilation
DLCO: Carbon Monoxide Diffusion
Primary IPFPrimary IPF
Diffuse Fibrosing AlveolitisDiffuse Fibrosing Alveolitis
Usual Interstitial Pneumonia (UIP)Usual Interstitial Pneumonia (UIP)
IPF is defined as a specific form of chronic,
fibrosing interstitial pneumonia of unknown
cause, typically affecting adults over 50 years,
limited to the lungs, and associated with the
histopathologic and/or radiologic pattern of
usual interstitial pneumonia (UIP)
It is the most frequent and devastating form of
IPF (median survival 3 years).
persist after cough
Acute or Subacute
)Hamman Rich Syndrome(
• Rapidly progressive,
• Fatal within few months
• Most cases.
• Slowly progressive
• Cyanosis may be only
exertional )short time for
Acute Exacerbation of IPFAcute Exacerbation of IPF
• Previous or concurrent diagnosis of idiopathic pulmonary fibrosis.
• Unexplained worsening or development of dyspnea within 30 days.
• HRCT with new bilateral ground-glass abnormality and/or
consolidation superimposed on background reticular or honeycomb
pattern consistent with UIP.
• Exclusion of alternative causes:
Left heart failure
• A test of aerobic capacity and endurance.
• Tests the global and integrated responses of all systems
involved during exercise, including pulmonary,
cardiovascular and neuromuscular systems.
• Does not provide specific information on each
• Easy, affordable )no need for expensive equipment or
advanced technician training(.
• Reflective of ADL )activities of daily living(: self-paced
and done at submaximal exercise capacity, like ADL.
• Strongly correlated with peak O2 uptake in many
It is used in many chronic cardiac (coronary heart disease, heart
failure) and respiratory (COPD, IPF) conditions for:
• Assessment of Functional Status.
• Prediction of Morbidity, Mortality.
• Pre-Treatment, Post-Treatment Comparison.
Unstable angina or myocardial infarction during last month.
- HR > 120 or < 50 bpm.
- BP > 180/100 mmHg.
Revise a recent ECG and revise the need for the test
- O2 Saturation < 88%>
• Usual walking aids used.
• Usual drug regimen continued.
• Supplemental O2 continued.
• Warming up period not allowed.
• Sit at rest on a chair near the start line for > 10 mins before
the test starts.
• STOP in case of:
- Chest pain - Staggering
- Intolerable dyspnoea - Diaphoresis
- Leg cramps - Pallor
What to Record at End of Test
• 6MWD (absolute value and percent of predicted value).
• Pulse, BP, RPP (Rate Pulse Product, a measure of myocardial exertion).
• Perceived dyspnoea, perceived exertion (modified Borg scale).
• O2 saturation.
Treatment of IPF:Treatment of IPF:
Non-Pharmacologic TreatmentNon-Pharmacologic Treatment
• Home Oxygen Therapy
If there is hypoxaemia (O2 Saturation < 88%) at
rest or induced by 6MWT.
• Respiratory Rehabilitation
improves distance of 6MWT and improves health-
related quality of life.
• Lung Transplantation
The only treatment in advanced IPF that results
in a major functional improvement.
PPI + Anti-Reflux measures
IV Methyl Prednisolone 500-1000 mg/day for 3 days
Prednisone 0.5 mg/Kg/day, gradually tapered
Pirfenidone (Pirfenex)Pirfenidone (Pirfenex)
• The only drug with confirmed efficacy against IPF.
• The only drug that was specifically licensed for treatment of IPF.
• FDA approved for this indication in 2014.
• ↓ Fibroblast proliferation, ↓ TGF-β stimulated collagen production
→ Anti-Fibrotic effect
• Has also an anti-inflammatory effect.
• IPF is a fibroblast- activated process. Inflammation is a 2ry event.
• ↓ Disease progression, ↓ decline in FVC, ↑ exercise capacity.
• Dose: One capsule (200 mg) / 8 h for 1 week
2 capsules / 8 h for 1 week
3 capsules / 8 h thereafter, for at least 12 months.
• Continued longer if there is some disease improvement or stabilization.
Adverse Effects, CautionsAdverse Effects, Cautions
Adverse EffectAdverse Effect CautionCaution
GI Upset (N, V,
Taken after food to ↓ these
upsets (though food
significantly ↓ its absorption)
Anorexia and Weight
Monitor weight, ↑ caloric
intake if needed.
Photosensitivity Avoid exposure to sunlight,
↑ Transaminases Check at baseline,
monthly for 6 M,
then every 3 M
Dizziness Avoid before driving vehicles
It is metabolized through CYP1A2 enzyme pathway:
Inducers ↓ Effect
• Smoking: quit.
• Omeprazole: Use pantoprazol
Inhibitors ↑ Toxicity
• Fluvoxamine (Faverine): Contraindicated.
• Ciprofloxacin, Amiodarone: Caution
Progression can occur
after exposure has
ceased, due to the
retention of fibers in the
lung and persistent
Asbestosis Containing MaterialsAsbestosis Containing Materials
• Asbestos is a broad term that includes a group of naturally
occurring fibrous mineral silicates of magnesium and iron.
• Asbestos content varies in different materials (1%-100%).
• Asbestos rich materials include:
• Wall and ceiling insulators
• Floor and Ceiling tiles
• Cement pipes
• Brake and clutch pads.
• Asbestos fibers are remarkably insulator to heat,
electricity and sound, so they were widely used in industry.
• Asbestos tends to break into very tiny fibers which remain
suspended in air for hours or days.
•Asbestos fibers are resistant to acid, alkali, water, heat and
flame. So, they are environmentally persistent, not
biodegradable and virtually indestructible.
Routes of ExposureRoutes of Exposure
• Asbestos containing material is not generally
considered harmful unless it is releasing dust or fibers
into the air where they can be inhaled or (less frequently)
• Many of the fibers will become trapped in the mucous
membranes of the nose and throat where they can then
be removed, but some may pass deep into the lungs, or,
if swallowed, into the digestive tract.
• People at risk:
o Construction workers.
o Car mechanics.
o Those exposed at home.
Pleuro-Pulmonary Disease due to AsbestosisPleuro-Pulmonary Disease due to Asbestosis
• Pleural Disease
– Pleural Thickening
(localized or diffuse):
– Pleural Effusion
– Rounded Atelectasis
• Lung Disease (Asbestosis):
IPF (Latency 15 - 20 Y)
• Pleural mesothelioma
• Peritoneal mesothelioma
• Lung Cancer
Cause of Death:
• Cancer: - Bronchial Carcinoma: most common cause of death
- Mesothelioma less common but more characteristic.
• Respiratory Failure.
Determining Factors for Development ofDetermining Factors for Development of
Asbestos- Related DiseaseAsbestos- Related Disease
– Level, frequency, and duration of exposure.
There is no “safe” level; any exposure is risky.
– Time elapsed since exposure
– Age at time of exposure
Younger persons more likely to develop disease.
– Smoking history (significantly ↑ risk of lung cancer).
– Individual susceptibility factors (?)
• Considered a “signal neoplasm” because of its rarity in
absence of exposure to asbestos.
• Latency: ≥ 20 years.
• Presenting symptoms often are chest pain and dyspnea,
due to pleural effusions.
• Rapidly invasive.
• At high concentrations: cancer of GIT, kidney, pancreas.
Mesothelioma of Pleura / Peritoneum