Respiratory pulmonary disease

1. CENTRE FOR PHYSIOTHERAPY AND REHABILITATION SCIENCES,
JAMIA MILLIA ISLAMIA
RESTRICTIVE PULMONARY
DISEASE
SUBJECT – PHYSIOTHERAPY IN
CARDIOPULMONARY CONDITIONS (BPT - 402)
SUBMITTED TO – DR JAMAAL ALI MOIZ
SUBMITTED BY – MUBASHIRAH KHANAM
CLASS – BPT, 4TH YEAR
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2. • Restrictive lung diseases(RLD) are the heterogeneous set of pulmonary
disorders defined by restrictive patterns on spirometry.
• These disorders are characterized by a reduced distensibility making the lungs
unable to fully expand, compromises the lung expansion and reduces LV,
particularly it reduces TLC.
• So they limit the amount of inhaled O2 restricts exhalation when
compared to an average person.
ETIOLOGY
RLD can be caused by -
1. Pulmonary parenchyma diseases (intrinsic causes) - infiltrates from
inflammation, toxins, and mechanisms yet to be elucidated.
• Inflammation in the alveolar interstitium with possible involvement of
the peripheral bronchial structures, this condition is known as interstitial lung
diseases (ILDs).
• Other conditions originating from the alveolus (e.g., edema, hemorrhage) spread
to interstitial structures causing stiffening, inflammation and scarring of the lung
tissues results in alteration of the lung architecture and functional impairment.
Acronym "PAINT“ - pleural, alveolar, interstitial, neuromuscular, and thoracic
cage abnormalities.
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3. 2. Extrapulmonary diseases (extrinsic causes) - limitations in neuromuscular
function and chest wall movements.
• These may be due to neuromuscular diseases, pleural disorders, obesity,
costosternal or costovertebral fusion and a fusion or deviation of the T vertebra.
• Other etiologies that result in a physical impediment to inspiration, impaired
ventilatory function and respiratory failure.
TYPES OF DISEASES AND CONDITIONS INVOLVED IN
INTRINSIC RLD EXTRINSIC RLD
Idiopathic pulmonary fibrosis (IPF) Kyphoscoliosis
Non-specific interstitial pneumonia and
Acute interstitial pneumonia
Pleural conditions such as effusions,
trapped lung, pleural scarring, chronic
empyema
Inorganic dust exposure such as silicosis,
asbestosis
Neuromuscular disorders like muscular
dystrophy, ALS, polio
Sarcoidosis Obesity
Pulmonary vasculitis Asbestosis
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4. EPIDEMIOLOGY
• Restrictive syndromes account for about a fifth of pulmonary syndromes, while
obstructive syndromes are the majority (80%).
• Risk factors –
– Elderly - prevalence increases from individuals aged 35-44 years to in those
older than 75 years. Sarcoidosis : 20-40 years.
– Gender - women has an increased risk for a restrictive pattern and
sarcoidosis. IPF is more common in men than in women.
– Obese individuals - Restrictive patterns are related to an elevated BMI with
a decrease in LV.
– Smokers- IPF individuals are usually current or past smokers.
– Connective tissue and Autoimmune disease
– Occupational and Environmental - Inorganic dust, organic dust, gases and
fumes, radiation.
– Drug Induced - Chemotherapeutic agent, radiation therapy
– Infections - Viral and bacterial.
– Genetic/Inherited - Familial pulmonary fibrosis
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5. SYMPTOMS
• Shortness of breath or inability to catch their breath
• Unproductive cough that is persistent
• Fatigue and Muscular pain
• Clubbing in fingers and toes
• Wheezing or gasping breath
• Anxiety and depression
PATHOPHYSIOLOGY
• In RPD, alveolar epithelial cells appear to become damaged and die. Recurring
inflammation starts leading to immunopathology and tissue destruction.
• The initial or absent inflammatory stage is quickly followed by an uncontrolled
wound-healing by out of control release of fibroblasts causing scarring and
hardening (fibrosis) of the delicate tissues of the lungs.
• Impairment in gas exchange is a common finding –
– Increased alveolar-arterial oxygen gradient, which depends on the alteration
of the V/Q ratio and the diffusion capacity.
• Hypoxemia –
– Hypoxemia (PaO2<60 mmHg) can be a consequence of lung damage, and an
expression of respiratory failure. 5

6. • Compliance –
– is reduced as a consequence of increased lung elastic recoil related to the
extracellular matrix deposition.
– It results in greater pressure generation and energy requirements from the
respiratory muscles for any given tidal volume.
• All of them causes an increase in RR as a compensatory mechanism to minimize
the work of breathing, with higher than normal minute ventilation, with
hypercapnia developing only in the late disease stages.
FUNCTIONAL CHARACTERISTICS
• Functional characteristics of a restrictive pattern in PFTs
HISTORY AND PHYSICAL EXAMINATION
• A comprehensive history and thorough physical examination are required to
identify and classify these complex disorders appropriately.
• Most common characteristic is an insidious progression of dyspnea.
Decreased FVC and TLC
FEV1 is slightly decreased or stays normal
The ratio of FEV1 to FVC is usually preserved or increased.
Reduced DLCO
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7. • A careful history must include the severity of symptoms(by PFT), time
course/origin of symptoms, rate of worsening of symptoms, family history,
smoking and drug history, as well as occupational and environmental exposure
history.
• ILDs classically produce the "3Cs“- cough, clubbing of the nails, and coarse
crackles on auscultation.
• In patients with extrinsic restriction will include an increased BMI, spine
deviation or have a history of neuromuscular disease.
EVALUATION
1. Pulmonary function tests (PFTs) -
• Decreased TLC and FVC with a preserved FEV1/FVC ratio (greater than 70%).
• In intrinsic pulmonary restriction - DLCO is decreased
• In extrinsic pulmonary restriction - DLCO remains normal
• Additionally, the static pressure-volume curve is shifted downward and to the
right as a result of decreased lung compliance.
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9. 2. Chest Radiography and High resolution Computing tomography –
– Peripheral reticular opacities (netlike linear and curvilinear densities)
predominantly at the lung bases.
– Honeycombing (coarse reticular pattern)
– Lower lobe volume loss can also be seen.
3. 6MWT - early arterial oxyhemoglobin desaturation during exercise/ test.
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10. • According to the ATS, the TLC predicted (adjusted for gender, age, height) value
can be used for assessing the severity of the restrictive condition -
• Interpretation of % predicted by FVC -
TLC < 80% = Restrictive disease
70-80 % predicted: mild restrictive disease
60-70% predicted: moderate restrictive disease
50-60% predicted: moderately severe restrictive disease
< 50% predicted: severe restrictive disease
80-120% - Normal
70-79% - Mild reduction
50%-69% - Moderate reduction
<50% - Severe reduction
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11. TREATMENT
• Each therapy strategy is individualized according to the patient's history and
symptoms.
1. Pharmacologic treatment -
• Immunosuppressant drugs – steroids and cyclophosphamide depending on the
degree of disease.
• New anti-fibrotic drugs - pirfenidone and nintedanib to slow down disease
progression
• Patients with acute exacerbations are usually treated with steroids over several
days.
2. Oxygen therapy –
• Oxygen administration improves exercise tolerance for patients with resting or
exertional hypoxemia.
3. High-flow nasal cannula -
• HFNC work with an air oxygen blender allowing from 21% to 100% FIO2,
heating and humidifying the inspiratory gas and generating up to 60 L/min in
adults.
• HFNC may have a role in the management of end-stage ILD patients with severe
hypoxemia, as palliative treatment.
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12. 4. Non-invasive ventilation -
• NIV may play a role in the chronic setting regarding the impact on the quality
of life of patients with end-stage disease.
• Some studies suggest that chronic use of NIV in ILD patients with hypercapnic
CRF (chronic respiratory failure) might decrease daytime arterial partial
pressure of CO2 and increase arterial partial pressure of O2.
5. Palliative care –
• The main goal of palliative care for patients with ILDs is to improve and
maintain an acceptable quality of life during the advanced phases of the
disease.
6. Pulmonary rehabilitation -
• Comprehensive intervention based on a thorough patient assessment followed
by patient-tailored therapies that include, but are not limited to, exercise
training, educational and behavioral changes, designed to improve the physical
and psychological condition of people with chronic respiratory disease and to
promote the long-term adherence of health-enhancing behaviors.
• Pulmonary rehabilitation programs involve aerobic conditioning, strength and
flexibility training, educational lectures, nutritional interventions, and
psychosocial support.
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13. 7. Transplantation -
• Lung transplantation remains the only effective therapy in patients with
advanced ILDs refractory to medical treatment.
8. Managing comorbid conditions –
• Morbidly obese patients who fail to lose weight by traditional methods should
be referred for gastric bypass surgery evaluation as it has been shown effective
in achieving significant weight loss and improvement on PFTs.
• Impairment in pulmonary function in patients with severe scoliosis may be
controlled with surgical correction.
9. Patient Education -
• For patients with progressive disease, pulmonary rehabilitation centers aid in
counseling patients about their breathing, smoking cessation and oxygen needs
as well as teach exercises that can improve respiratory function and mitigate
symptoms.
• At times, exercise and weight loss can prove effective, especially when obesity
is the main factor leading to pulmonary restriction.
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14. THE MOST FREQUENT COMPLICATIONS AND
COMORBIDITIES ASSOCIATED WITH CRF -
• Pulmonary hypertension
• Chronic obstructive pulmonary disease (COPD)
• Emphysema
• Pulmonary embolism
• Congestive heart failure
• Lung cancer
• Obstructive sleep apnea syndrome (OSAS)
• Small airway disease.
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15. PT MANAGEMENT IN PULMONARY FIBROSIS
• Goals -
– Maximize the patient's quality of life, general health and wellbeing.
– Educate the patient about PF and self management
– Ceasing of smoking, prevention of infections.
– Optimize alveolar ventilation and reduce the work of breathing.
– Maximize aerobic capacity and Optimize endurance and strength
• The primary interventions for maximizing cardiopulmonary function and oxygen
transport in patients with PF include some combination of education, aerobic
exercise, strengthening exercises, chest wall mobility exercises, body
positioning, coughing, relaxation techniques and energy conservation.
• An ergonomic assessment of home and work environments may be indicated to
maximize function in these settings.
• During aerobic exercise, patients with PF are prone to arterial desaturation.
Patients who desaturate during sleep, require supplemental oxygen during
exercise
• Breathlessness is accessed using a modified version of the Borg scale of
perceived exertion.
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16. • Education includes information in preventative health practices, such as
removing from the causative environment, smoking reduction and cessation,
weight control, hydration, relaxation.
PROGNOSIS
• Patients with RPD from a pleural effusion should experience resolution of
symptoms upon drainage, and pregnant patients should also recover upon
delivery.
• Intrinsic pulmonary restriction from pneumonia is associated with excellent long
term outcomes when treated.
• Patients diagnosed with IPF have a median survival of 3-5 years after diagnosis.
CONCLUSION
• Despite the known ominous prognosis of some ILDs, differential diagnosis of
CRF should be properly assessed.
• In ILD end-stage patients management options such as rehabilitation programs
and palliative care remain largely underused thus a multidisciplinary approach
to CRF would be desirable and may improve the outcomes and the QOL.
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17. REFERENCES
• Martinez-Pitre, P. J., Sabbula, B. R., & Cascella, M. (2020). Restrictive
Lung Disease. In StatPearls. StatPearls Publishing.
• Paola Faverio1, Federica De Giacomi1, Giulia Bonaiti1, Anna Stainer1,
Luca Sardella1, Giulia Pellegrino2, Giuseppe Francesco Sferrazza Papa2,
Francesco Bini3, Bruno Dino Bodini4, Mauro Carone5, Sara Annoni6,
Grazia Messinesi1, Alberto Pesci1. Review Management of Chronic
Respiratory Failure in Interstitial Lung Diseases: Overview and Clinical
Insights
• Wahls S. A. (2012). Causes and evaluation of chronic dyspnea. American
family physician, 86(2), 173–182.
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