Interstitial lung disease (ILD) ppt slideshare

Interstitial lung disease
• Interstitial lung disease (ILD) or DPLD (diffuse parenchymal lung
disease) is a large group of diseases that cause scarring (fibrosis) of the
lungs.
• The scarring causes stiffness in the lungs which makes it difficult to
breathe and get oxygen to the bloodstream.
• It mainly affects the interstitium (the tissue and space around the alveoli)
of lungs.
• Lung damage from ILD’s is often irreversible and gets worse over time.

EPIDEMIOLOGY
• The exact incidence and prevalence of disease is not unknown.
• Earlier it was considered a rare disease in India but now it’s not rare
according to a number of studies.
• It is estimated that 10-15% of the patients coming to a pulmonary
physician are diagnosed with ILD.
• Incidence is higher in men due to occupational exposure.
• The average rate of survival for someone with this disease is
between three and five years.

Sarcoidosis
Growth of tiny collections of inflammatory cells in different parts of the
body.
Hypersensitivity pneumonitis (extrinsic allergic alveolitis):
inflammation of airspaces and small airways within the lungs, caused
by hypersensitivity to inhaled organic dusts and moulds.

• Idiopathic interstitial pneumonia : Represent the majority of cases of
interstitial lung diseases (up to two-thirds of cases).- Idiopathic pulmonary
fibrosis (IPF) is the most common subgroup.
• Nonspecific interstitial pneumonia (NSIP)
• Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)
• Desquamative interstitial pneumonia (DIP): characterised by mononuclear
cell infiltration of the airspaces
• Cryptogenic organizing pneumonia (COP)
• Acute interstitial pneumonia (AIP) (Hamman-rich syndrome)
• Lymphoid interstitial pneumonia (LIP): It is a syndrome of fever, cough and
dyspnea, with bibasilar pulmonary infiltrates consisting of dense interstitial
accumulations of lymphocytes and plasma cells

• Lymangioleiomyomatosis: abnormal muscle-like cells begin to grow out of control in
certain organs or tissues, especially the lungs, lymph nodes, and kidneys. Over time,
these LAM cells can destroy the healthy lung tissue.
• Pulmonary alveolar proteinases: rare lung disorder characterized by an abnormal
accumulation of surfactant derived lipoprotein compounds within the alveoli of the
lungs
• Langerhans cell histiocytosis: abnormal clonal proliferation of Langerhans cells,
abnormal cells deriving from bone marrow and capable of migrating from skin to
lymph nodes.
• Pleural parenchymal fibroelastosis: rare condition characterized by predominantly
upper lobe pleural and subjacent parenchymal fibrosis, the latter being intra alveolar
with accompanying elastosis of the alveolar walls

CAUSES
 Inhalation of inorganic dust (such as crystalline silica, asbestos, and
coal dust) or
 Inhalation of organic dust from organisms encountered in farming,
 Use of air conditioning and
 Animal husbandry
 Radiation damage, and
 Infectious agents

Exogenous or endogenous stimuli
Dust fumes, cigarette
smoke, autoimmune
conditions
Drugs, infection, radiation,
& others
Microscopic lung injury
Intact Wound healing aberrant
lung homeostasis Pulmonary fibrosis
Genetic predisposition
Auto immune conditions
Dyspnea
Dry cough
Fatigue
Pleuritic
chest pain

CLINICAL MANIFESTATION
o Breathlessness (most common)
o Non-productive cough
o Fatigue
o Pleuritic chest pain
o Hemoptysis – infrequent

DIAGNOSTIC INVESTIGATIONS
HISTORY
Occupational history
Personal history
Medical history and Surgical history
Prolonged medication therapy
Radiation exposure
Family history

Systemic symptoms
 Connective tissue disease is a frequent cause of ILD
 Nonspecific symptoms such as night sweats, fever, fatigue or weight
loss suggest an underlying inflammatory condition

Dermatologic symptoms
Heliotrope rash
 Periorbital erythema with or without edema of eyelids and periorbital
tissue
 Highly characteristic of DM

Dermatomyositis
 Gottron’s papules
 mechanic’s hands
 Systemic sclerosis: calcium
deposits, puffy fingers,
raynaud’s, digital ulcers and
scars
 Systemic lupus erythematosus:
malar rash, photosensitivity skin
reaction, hair loss

Gastrointestinal symptoms
 Esophageal motility problems
 Chronic, intermittent aspiration can lead to progressive fibrotic lung
disease
 Bloating and diarrhea

Musculoskeletal complaints
 Connective tissue disease
 Raynaud’s phenomenon –
scleroderma, SLE and
antisynthetase syndrome
 Swollen fingers (sausage
digits) may be observed in
systemic sclerosis and
polymyositis

Ophthalmologic symptoms
 Dry eyes – Sjogren syndrome
 Increasing edema, syncopal events, or
exertional chest discomfort may indicate
severe pulmonary hypertension
 Presence of palpitations or syncope in a
patient with sarcoidosis – cardiac
sarcoidosis
 Pleuritic chest pain, leg swelling and
increasing dyspnea – consideration of
acute pulmonary embolism

Past medical history
 Prior diagnosis of connective tissue disease
 Case of HIV disease – lymphocytic interstitial pneumonia (LIP) are
common
 H/o acute or chronic kidney disease might suggest underlying
vasculitis, pulmonary renal syndromes
 H/o liver disease could suggest sarcoidosis, primary biliary cirrhosis

Occupational history
 Inorganic exposure
 Organic exposure
Medication history
 Nitrofurantoin
 Amiodarone
 NSAID’s
 H/o recent chemotherapy
 H/o immune-modulating drug use

Physical examination
Pulmonary signs:
 tachypnea and tachycardia, even at rest
 Bilateral, basilar, Velcro like rales
 Signs of pulmonary hypertension
Extrapulmonary signs:
 Clubbing (e.g. IPF)
 Skin abnormalities,
 peripheral lymphadenopathy, hepatosplenomegaly (sarcoidosis)
 Subcutaneous nodules (rheumatoid arthritis)

 Muscle tenderness and proximal weakness (polymyositis)
 Inspiratory squeaks
 Clubbing
 Skin involvement-vasculitis, tuberous sclerosis
 Arthritis
 Eye changes (uveitis, conjunctivitis)
 Muscle weakness
 Neuropathy
 Lymphadenopathy

INVESTIGATIONS
 Chest radiography
Nodules, linear (reticular) infiltrates, or a combination of the two
(reticulonodular infiltrates)
Diffuse ground glass pattern- early
Cystic areas (honeycomb pattern) – late
The first indication of underlying ILD
 High resolution CT scan : More sensitive than chest radiograph

• Pulmonary function test:
reduced lung volumes, reduced
diffusing capacity, a normal or
supernormal ratio of FEV1 to
FVC, static lung compliance is
decreased
• Maximal transpulmonary
pressure is increased (a very
high negative pressure must be
generated to open the fibrotic
alveoli)

 Laboratory testing
 Elevated liver enzymes or hypercalcemia – sarcoidosis
 Renal insufficiency – pulmonary renal syndromes
 Peripheral eosinophilia – chronic eosinophilic pneumonia, churg-
strauss syndrome, drug reaction
 Bronchoscopy
 Useful in the diagnosis of DPLD
 Inspection of the upper and lower airways, bronchoalveolar lavage
(BAL), and the performance of transbronchial lung biopsy.

 BAL:
 Bloody lavage specimens – diffuse alveolar hemorrhage
 Milky white BAL fluid – pulmonary alveolar proteinosis
 BAL eosinophilia (>25%) – acute eosinophilic pneumonia
 BAL lymphocytosis- granulomatous ILD, suggestive of
hypersensitivity pneumonitis, drug reaction or cellular NSIP
 Positive lymphocyte proliferation assay in chronic beryllium disease
 Asbestos bodies in asbestosis
 CD1A positive cells on flow cytometry may lead to a diagnosis of
LCH
 In the immunocompromised host, BAL fluid is highly sensitive for
the diagnosis of bacterial, viral, fungal, and mycobacterial disease.

 Surgical lung biopsy
 Despite a high yield in certain forms of lung disease, the utility of
transbronchial biopsy for most of these is low and surgical biopsy is
often required for accurate diagnosis
 The usual technique is video assisted thoracoscopic surgery (VATS)
that has a low morbidity and mortality in selected populations.

TREATMENT
 Provide symptom- relief
 Improve quality of life
 Prevent complications
 End of life care and palliative treatment
 Removal from exposures
 Treatment of comorbidities
 Palliative care
 Lung transplantation

• Removal from exposures
o Drug reaction is suspected
o Mold growth, removal of birds from the home, extensive cleaning of
upholstery, window coverings, and ventilation systems
o Occupational exposures- avoided
• Antifibrotic drugs
o Useful in progressive fibrotic lung disease
o Pirfenidone
o Stabilize lung function

• Corticosteroids:
o Mainstay of therapy
o Prednisone, 1 mg/kg for 1 month, followed by 40 mg/day given for 2
months
o Gradually tapered (5 mg/week) over several months to a
maintenance dose of 15 to 20 mg/day
o Corticosteroids are continued until pulmonary function is stable for 1
year
o Relapses require returning to high dose steroids

• Immunosuppressive therapy
o Some forms of ILD, including COP, CTD – associated ILD,
and sarcoidosis, shows favorable response to steroids and
other immunosuppressive agents
o When a more prolonged course of therapy is anticipated,
azathioprine or cyclophosphamide, permit low dose of
steroids
o If no clinical improvement is seen after 3 to 6 months of
therapy, discontinuation of immunosuppressive therapy
should be strongly considered

Lung transplant
o Most patients with ILD referred for lung transplantation have IPF –
advanced stage.
o A severely impaired DLCO (<39%) as well as survival and are
considered to be triggers for active listing.
o Early referral to a lung transplant center is useful.

PROGNOSIS
• Some forms of ILD resolve completely, while others lead to long term
and irreversible scarring and lung damage with accompanying
respiratory failure.
• Pulmonary hypertension can develop in cases of long standing ILD and
can lead to cor pulmonale.
• The prognosis is dependent upon the type and severity of ILD as well
as the underlying health status of the patient.

PREVENTION
• Avoid or limit exposure to toxins or treatments that can lead to ILD.
• Proper diet and exercise reduces chances of developing ILD.
• Quitting smoking and avoiding exposure to substances known to
cause ILD can prevent the disorder from developing or worsening.
• People who are employed in jobs where they may be heavily exposed
to known causes of lung disease in the workplace typically should
undergo routine screening for lung disease.

NURSING MANAGEMENT
 Ineffective breathing pattern related to decreased lung compliance, decreased
energy as characterized by dyspnea, abnormal ABG, cyanosis and use of
accessory muscles
 Monitor respiratory rate, depth, pattern, pulse oximetry, and arterial blood gas.
 Elevate the head of the bed to at least 30 degrees.
 Encourage deep breathing exercise, coughing, and the use of incentive spirometry.
 Administer medications(sedatives) with caution that may lead to respiratory failure
 Observe for evidence of coughing, increasing dyspnea or pulse rate.
 Maintain patent airway through suction and ventilatory support.

 Risk for decreased cardiac output related to positive pressure ventilation
 Assess heart rate, rhythm, level of consciousness, and hemodynamic
parameters
 Provide bed rest to the patient to relieve symptoms
 Monitor ECG changes.
 Auscultate breath sounds and heart sounds, listen for abnormal heart sounds,
murmurs, gallop sounds.
 Assess for stiffness of chest and consolidation.

 Risk for impaired skin integrity related to prolonged bed rest,
prolonged intubation and immobility
 Assess integrity of skin, risk of pressure ulcers by braden’s scale
 Change the position of the patient every two hourly
 Provide nutritious diet to the patient(high calorie, high protein diet).
 Provide TPN in case the patient is not able to take orally or is advised npo
 Provide bed bath and keep the skin dry
 Apply emollients and reassess the skin integrity

 Knowledge deficit related to health condition, new equipment and
hospitalization as characterized by increased frequency of questions posed by
patient and significant others.
 Assess the knowledge level of the patient and relatives about the disease condition,
the equipment used in treatment and the medication and hospitalization need of the
patient.
 Clarify the doubts of the family members and allow them to ventilate their feelings
 Provide therapeutic environment and counsel the family members
 Encourage the family members to stay positive and ask if any query persists.
 Provide information on how to promote recovery with healthy diet and exercise.
 Teach the patient deep breathing exercise and coughing etiquettes and range of
motion exercises

Nursing alert: nursing assessment is essential to minimize the
complications related to neuromuscular blockade. The patient may
have discomfort or pain but cannot communicate these sensations. In
addition, frequent oral care and suctioning may be needed.

CONCLUSION
• Interstitial lung disease is a group of disease that causes progressive
scarring of lung tissues. People with interstitial lung disease may
experience chronic or dry cough, fatigue or inability to exercise,
shortness of breath or weight loss, etc. And the treatment of patients
with interstitial lung disease depends on severity and underlying
cause but often includes steroids, oxygen therapy, and pulmonary
rehabilitation.

REFERENCES
 Brunner and suddharths. Textbook of medical and surgical nursing. 13th edition vol. I. .New delhi: reed elsevier india
pvt. Ltd.; 2014. Pg. No. 360- 395
 Lewis. Medical surgical nursing. Assessment and management of clinical problems. 2015. New delhi. Elsevier vol. I. Pg.
No. 461-493
 Joyce M. Black and jane hokanson; medical surgical nursing; volume 2, 8th edition, reed elsevier, india pvt. Pg. No. 1630
 Kasper, fauci, hauser, lingo, harrison’s principles of internal medicine, 19th edition, 2015. Noida, mcgraw hill india pvt ltd.
• Research hyperlinks:
 Suzuki, atsushi et al. “The impact of high-flow nasal cannula oxygen therapy on exercise capacity in fibrotic interstitial
lung disease: a proof-of-concept randomized controlled crossover trial.” BMC pulmonary medicine vol. 20,1 51. 24 feb.
2020, doi:10.1186/s12890-020-1093-2
 Kreuter, michael et al. “Health-related quality of life and symptoms in patients with IPF treated with nintedanib: analyses
of patient-reported outcomes from the INPULSIS® trials.” Respiratory research vol. 21,1 36. 30 jan. 2020,
doi:10.1186/s12931-020-1298-1

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