Interstitial lung disease (ILD) is a group of diffuse lung diseases that affect the lung parenchyma and cause breathlessness, cough, and fatigue. Physiotherapy plays an important role in managing ILD through pulmonary rehabilitation programs that include aerobic endurance training, resistance training, stretching, and supplemental oxygen as needed. These programs aim to improve exercise capacity, quality of life, and lung function by optimizing ventilation, gas exchange, and muscle strength in patients with ILD.
1. CENTRE FOR PHYSIOTHERAPYAND REHABILITATION SCIENCES,
JAMIA MILLIA ISLAMIA
TOPIC: INTERSTITIAL LUNG DISEASE (ILD)
PHYSIOTHERAPY IN CARDIOPULMONARY CONDITIONS (BPT - 402)
SUBMITTED TO – DR JAMAL ALI MOIZ
SUBMITTED BY – MAHEEN HASAN
BPT 4TH YEAR
ROLL NO. 17BPT014
DATE OF PRESENTATION – 04.01.2021
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2. INTRODUCTION
• Interstitial lung disease (ILD) is an umbrella term for group of diffuse acute and chronic lung
diseases of various types and subtypes affecting lung parenchyma of variable intensity of
inflammation, fibrosis and anatomical distortions, related with high mortality and morbidity.
• ILD is a highly disabling group of conditions including idiopathic pulmonary fibrosis (IPF), acute
and chronic interstitial pneumonias, connective tissue diseases and sarcoidosis.
• Individuals with ILD have reduced health-related quality of life (HRQL) that tends to worsen with
disease progression. These patients frequently experience breathlessness on exertion, which limits
their ability to undertake daily activities.
CLINICAL FEATURES
The most common symptoms are
• Dyspnea on exertion, chronic unproductive cough
• Poor functional capacity and fatigue
• On auscultation, bilateral inspiratory crackles are present.
• Chest expansion is reduced
• At later stages, clubbing of the digits is often present because of hypoxia.
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4. Lung respond to the damage and try to autorepair. If this exposure is for long time or if the repair
process is affected, the lung become permanently damaged, and there is increased interstitial
tissue which replaces the normal structures such as capillaries, alveoli and healthy interstitium.
The tissue around the alveoli becomes scarred and thickened. This
makes it more difficult for the oxygen to pass into the bloodstream.
Decrease diffusion leads to V/Q mismatching and shunting.
Together these abnormal physiology lead to low functional capacity and poor exercise
tolerance seen in ILDs
Hence these patients are commonly hypoxic and desaturate soon.
Work of breathing is considerably increased because of low compliance.
Unfortunately, if the initiating injury or abnormal repair from injury is not
stopped, progressive damage can lead to worsening impairment and even death. 4
6. DIAGNOSTIC EVALUATION OF ILD
HISTORYAND CLINICAL EXAMINATION
• Detailed history taking is required to identify respiratory risk factors both past and present. This
includes history of current and prior occupations, animal exposures, medications and radiation
exposure.
PULMONARY FUNCTION TESTS (PFTs)
• Reduced vital capacity (VC) and total lung capacity (TLC). Reduced FEV1 (<80% of the predicted
normal), reduced FVC (<80% of the predicted normal) and normal FEV1/FVC ratio (>0.7)
• A reduced diffusing capacity (DLCO) is the earliest and most consistent change and compliance is
markedly reduced.
At first, the arterial PaO2 may be normal at rest but may drop significantly with exercise. Later the
PaO2 is markedly reduced because of the thickened alveolar membrane and ventilation-perfusion
mismatching.
CHEST X-RAY
• Usually indicates diffuse reticular markings, most prominent in the lower lung fields.
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7. HIGH RESOLUTION CT (HRCT)
• HRCT provides a detailed depiction of the lung parenchyma that can be used to focus and prioritize
diagnostic possibilities in a patient with suspected ILD.
• HRCT features of ILD can include ground glass opacities, consolidation, nodules, honeycombing,
traction bronchiectasis.
LUNG TISSUE ANALYSIS
• Bronchoscopy
• Bronchoalveolar lavage (BAL) – used to assess the amount of inflammation and the accumulation of
immune effector cells and proteins in the alveoli.
• Surgical Lung Biopsy – Lung biopsies that demonstrate an abundance of inflammatory cells suggest
early disease, whereas a prevelance of fibrosis is indicative of advanced disease.
LABORATORY TESTS
• Laboratory tests performed in patients with suspected ILD should include a complete blood cell count
with differential leukocyte counts, renal and liver function tests, and urinalysis.
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9. EXERCISE LIMITATION IN INTERSTITIAL LUNG DISEASE
• Reduced exercise capacity is a cardinal feature of the ILDs.
• Exercise limitation in ILD is multifactorial, with contributions including impairment of gas
exchange and pulmonary circulation, ventilatory dysfunction and muscle dysfunction.
1. Impairments to gas exchange and pulmonary circulation
• Gas exchange limitation occurs due to pulmonary capillary destruction or membrane thickening,
resulting in impaired diffusion capacity and ventilation–perfusion inequality.
• Consequently, exercise-induced oxyhaemoglobin desaturation is often profound, and hypoxaemia
may be present at rest
• Lung inflammation and fibrosis further cause pulmonary vascular resistance and pulmonary
hypertension.
• Pulmonary hypertension (PH) is a common comorbidity in ILD, especially in advanced disease.
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10. 2. Ventilatory limitation
• People with ILD may exhibit an abnormal respiratory pattern with decreased tidal volume and a
rapid respiratory rate, particularly during exercise.
• Despite this, ventilatory mechanics are not the major limitation to exercise in most patients, with
impaired gas exchange and circulatory limitation playing a more important role.
3. Muscle dysfunction
• Inflammatory contributors to the pathogenesis of ILD may potentiate peripheral muscle dysfunction.
• People with ILD suffered from significant weight loss, atrophy and deconditioning, which is the
major cause of exercise intolerance in them .
• In addition, ILD patients may receive treatments such as glucocorticoids and immunosuppressive
therapy, which are known to cause drug-induced myopathy.
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11. PHARMACOLOGICAL MANAGEMENT
• The mainstay of pharmaceutical management of ILDs are anti-inflammatory medications and
corticosteroids such as prednisolone which are associated with increase in oxidative stress
mitochondrial dysfunction and induce proteolysis which leads to cachexia and atrophy in ILD.
• Immunosuppressive agents as azathioprine or methotrexate are also used to decline the disease
progression but causes drug induce myopathies.
• Anticoagulants are also given to increase the survival in people with ILD who are having
associated cardiovascular complications such as pulmonary hypertension.
MANAGEMENT
NON PHARMACOLOGICAL MANAGEMENT
• The non-pharmacologic treatment option is pulmonary rehabilitation. Pulmonary rehabilitation
programs involve aerobic conditioning, strength and flexibility training, educational lectures,
nutritional interventions, and psychosocial support.
Lung Transplantation is an accepted form of treatment for patients with ILD that is progressive,
clearly leading to respiratory failure, and refractive to other therapies.
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12. Goals of PT Management of the patient with ILD
• Maximize the patient’s QOL, general health and well being and hence physiological reserve
capacity.
• Educate about ILD, self-management, nutrition, smoking reduction and cessation, medications and
their uses, prevention and health promotion.
• Optimize alveolar ventilation.
• Optimize lung volumes and capacities
• Optimize ventilation and perfusion mismatching.
• Reduce the work of breathing.
• Reduce the work of the heart.
• Maximize aerobic capacity and efficiency of oxygen transport.
• Optimize physical endurance and exercise capacity.
• Optimize general muscle strength.
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13. PROTOCOL OF PULMONARY REHABILITATION IN ILD
• The American Thoracic Society and European Respiratory Society (ATS/ERS) definition of PR
describes a comprehensive intervention of patient tailored therapies, that may include exercise
training (that includes aerobic and resistance training), education, and behaviour change.
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14. Endurance Training
• Aims to improve aerobic capacity, increase exercise endurance, and improve daily function and
physical activity with less breathlessness and fatigue.
• A minimum frequency of two supervised sessions per week is suggested.
• Initial endurance training intensity is usually set at 70–80% of maximum exercise capacity, such as
walking speed on baseline 6MWT (for walking exercise) or peak work rate on CPET (for cycling).
• The target duration of endurance exercise in each session should be 30 minutes for ILD patients,
broken into shorter intervals if needed (e.g. 15 minutes stationary cycling and 15 minutes of
walking, either on a treadmill or in a corridor).
• Once a duration of 30 minutes is achieved, progression occurs by regular increases in the intensity
of exercise (e.g. weekly increase in walking speed or cycle work rate).
The ideal duration of PR for people with ILD is unclear. The British guidelines for PR recommend
programmes of 6–12 weeks duration, but no recommendations specific to ILD are made.
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15. Resistance Training
• ACSM recommends training 2–3 days per week, with 10–15 repetitions and a single set for older
persons.
• For intensity, the ACSM recommends 40–50% of the 1 repetition maximum (1 RM) (very light to
light intensity) to improve strength and 20–50% of the 1 RM to improve power. Modalities include
resistance bands and free weights.
• Progression of resistance training may involve increasing the weight, number of repetitions per set,
number of sets of each exercise or decreasing the rest period between sets.
• ACSM suggests emphasizing functional activities (e.g. stair climbing, sit-to-stand) as these are
directly relevant to daily activities.
• Intermittent monitoring of oxyhaemoglobin saturation and pulse rate via pulse oximetry is also
recommended.
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16. Flexibility training and stretching
• Flexibility training aims to increase the range of motion (ROM) of joints and muscles. There are no
specific guidelines for flexibility training in ILD.
• The ACSM recommends flexibility exercise on at least 2–3 days per week, with 30–60-second
stretches repeated two-to-four times for older people.
Supplemental Oxygen
• The ATS/ERS statement for PR recommends supplemental oxygen during exercise training for
ILD.
• Exercise-induced desaturation and pulmonary hypertension are common. Supplemental oxygen
should be available and appropriate monitoring of oxyhemoglobin saturation during exercise is
indicated.
• Supplemental oxygen would routinely be administered to such patients during exercise training in
PR, to ensure safety in the presence of profound desaturation.
• More evidence is needed to improve the understanding of the role of oxygen therapy during
exercise training. Currently, usual practice would be to deliver oxygen therapy for any patient who
desaturates to less than 85% during training, with the aim of maintaining SpO2 at greater than
88%.
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17. PULMONARY REHABILITATION: OUTCOME ASSESSMENT
Assessment of exercise capacity
• The 6-minute walk test (6MWT) – The 6MWT is the most commonly used test of exercise
capacity in PR. The reproducibility of the 6MWD has been confirmed in ILD.
• Shuttle walking test.
• Cardiopulmonary exercise test (CPET) – provides detailed information about exercise responses
and exercise capacity in ILD.
Health-related quality of life, dyspnea and mood assessment
• Many PR programmes use HRQL measures originally designed for patients with COPD (e.g. the
CRDQ and the SGRQ).
• More recently, ILD-specific measures have become available, including the IPF-specific version of
SGRQ and the King’s Brief Interstitial Lung Disease Questionnaire.
• Dyspnea assessment tools – Borg scale, mMRC scale.
• Outcome tools used to measure mood in PR studies for ILD include the Hospital Anxiety and
Depression (HAD) Scale, the Center for Epidemiologic Studies-Depression score, the Geriatric
Depression Scale and The General Anxiety Disorder-7 scale. 17
18. REFERENCES
• Nakazawa, A., Cox, N. S., & Holland, A. E. (2017). Current best practice in rehabilitation in
interstitial lung disease. Therapeutic Advances in Respiratory Disease, 11(2), 115-128.
• Spruit, M. A., Singh, S. J., Garvey, C., ZuWallack, R., Nici, L., Rochester, C., ... & Pitta, F. (2013).
An official American Thoracic Society/European Respiratory Society statement: key concepts and
advances in pulmonary rehabilitation. American journal of respiratory and critical care
medicine, 188(8), e13-e64.
• Dean, E & Frownfelter, D. Cardiopulmonary physical therapy, (3rd ed)
• Ryu, J. H., Daniels, C. E., Hartman, T. E., & Eunhee, S. Y. (2007, August). Diagnosis of interstitial
lung diseases. In Mayo Clinic Proceedings (Vol. 82, No. 8, pp. 976-986). Elsevier.
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