Interstitial Lung Disease Flashcards
What is the best diagnosis for ILD?
Surgical biopsy with histological exam for precise diagnosis.
What is interstitial lung disease
heterogenous group of diffuse parenchymal lung disease with a variety of causes, known and unknown
How many diseases does ILD have
extremely diverse group of acute and chronic disorders
How is ILD categorized
By varying degrees of inflammation and fibrosis of the lung parenchyma
What is the definition of ILD
ILD/DPLD are diseases affect the tissue and space around the alveoli, causing progressive scarring of the lung tissue through inflammation and fibrosis
How many types of ILD are there
200 different subtypes
How is ILD diagnosed
By clinical, pathophysiological, immunological, and imaging
Clinical history of ILD
Slow progression of dyspnea on exertion or at rest
nonproductive cough
Duratón can last months to years
What are the classifications of ILD?
Major subgroups of ILD are now broadly defined as:
Idiopathic interstitial pneumonias (IIPs)
Granulomatous ILD (e.g., sarcoidosis, hypersensitivity pneumonitis)
What are the classifications of ILD? #2
ILD with known associations (e.g., occupational and drug exposures, connective tissue diseases)
Miscellaneous ILD (e.g., pulmonary Langerhans cell histiocytosis [PLCH])
Clinical history of ILD #1
Fever, chills, and night sweats
Clinical history of ILD #2
Prior medications used and irradiation
Clinical history of ILD #3
Patient/ Family history- most critical step in evaluation
Clinical history of ILD #4
Detail work history- occupational exposures
Clinical history of ILD #5
Travel history
Clinical history of ILD#6
Enviromental exposures- due to hobbies
Clinical history of ILD #7
Smoking history
Clinical history of ILD #8
Past systemic conditions
Clues from initial eval to suggest ILD
Dyspnea/ all ILD
Clues from initial eval to suggest ILD
Nonproductive cough/ most ILD, productive cough is unusual
Clues from initial eval to suggest ILD
Fatigue/ most ILD and present with dyspnea
Fever, chills, weight loss- interstitial pulmonary infections, collagen vascular disorder
Clues from initial eval to suggest ILD
Inspiratory crackles/ most ILD
Wheezing- uncommon mostly with hypersensitivity pneumonia
Pleural rub- rheumatoid arthritis, systemic lupus
Digital cubbing- interstitial pulmonary fibrosis
Clues from initial eval to suggest ILD
Tachypnea- 15% of all interstitial pulmonary lung diseases
Chest pain- uncommon, pleuritic chest pain/ systemic lupus, rheumatoid arthritis, and pneumothorax
Clues from initial eval to suggest ILD
Arthralgias, arthritis- CTD’s sarcoidosis
Clues from initial eval to suggest ILD
Hemoptysis- diffuse alveolar hemorrhage syndrome, LAM
Clues from initial eval to suggest ILD
Eye symptoms- sarcoidosis, CTD, polyangiitis, and granulomatosis
Salivary gland enlargement- sarcoidosis, Sjogren’s disease
Clues from initial eval to suggest ILD
Abnormal gastroesophageal reflux, dysphagia- CTD (especially SCL), IPF
Clues from initial eval to suggest ILD
Peripheral lymph adenopathy- Sarcoidosis, lymphoid interstitial pneumonitis, ILD with connective tissue disorders.
Clues from initial eval to suggest ILD
Hepatosplenomegaly- Sarcoidosis, eosinophilic granuloma, chronic cor pulmonale
Clues from initial eval to suggest ILD
Neurologic manifestations- Tuberous sclerosis, systemic vasculitis, sarcoidosis, eosinophilic granuloma
Clues from initial eval to suggest ILD
Rapid onset or worsening- Acute interstitial pneumonia, acute hypersensitivity pneumonitis, cryptogenic organizing pneumonia, CTD, diffuse alveolar hemorrhage
Etiology (cause) of ILD
Drugs: ACE inhibitors, amiodarone, and nonsteroidal inflammatory drugs
Etiology (cause) of ILD
Illicit drugs- cocaine heroin
Etiology (cause) of ILD
Inorganic material - asbestos, coffee, farming material hot tub, bird droppings
Etiology (cause) of ILD
Infections- aspergillosis, histoplasmosis, parasite infection, mycobacterial and viral infection
Etiology (cause) of ILD
Connective tissue disorders- system sclerosis, autoimmune myosis, Goodpasture syndrome, and systemic lupus erythematosus
Etiology (cause) of ILD
Vasculitis- eosinophilic granulomatosis with polyangiitis,
Etiology (cause) of ILD
Miscellaneous causes- amyloidosis, chronic aspiration, sarcoidosis, tuberous, sclerosis, lipoid pneumonia
Etiology (cause) of ILD
0% of persons with ILD have no clear-cut cause that is easily identifiable. This affects the diagnosis a precise etiology for ILD,
Etiology (cause) of ILD
30% of persons with ILD have no clear-cut cause that is easily identifiable. This affects the diagnosis a precise etiology for ILD,
Epidemiology
ILD is PREDOMINIANTELY AN ADULT disease; HOWEVER, IT DOES occur in CHILDREN. Certain ILDS such as sarcoidosis, PLHC, and autoimmune-associated lung disease develop in adults.
Epidemiology
The prevalence is 10 times greater in African Americans 34 cases per 100,000
Epidemiology
Occurs in middle age toolder adults
Epidemiology
Occurs in middle age to older adults
Pathogenesis (manner of development)
ILD dominated by inflammation:
Organizing pneumonia
Nonspecific interstitial pneumonitis
ILD dominated by fibrosis:
Characterized by fibroblastic foci
Pathogenesis (manner of development)
Lung fibrotic disorders
Characterized by accumulation of fibroblasts, myofibroblasts, and extracellular matrix leading to chronic respiratory failure.
Origins and activation of fibroblasts unknown.
Etiology (cause) of ILD
Major subgroups of ILD are now broadly defined as:
Idiopathic interstitial pneumonias (IIPs)
Etiology (cause) of ILD
Granulomatous ILD (e.g., sarcoidosis, hypersensitivity pneumonitis)
Etiology (cause) of ILD
ILD with known associations (e.g., occupational and drug exposures, connective tissue diseases)
Etiology (cause) of ILD
Miscellaneous ILD (e.g., pulmonary Langerhans cell histiocytosis [PLCH])
Epidemiology
31.5 new cases diagnosed per 100,000 men/yr and 26.1 new cases diagnosed per 100,000 women/yr
Pathogenesis
ILD dominated by inflammation:
Organizing pneumonia
Nonspecific interstitial pneumonitis
Pathogenesis
ILD dominated by fibrosis:
Characterized by fibroblastic foci
Pathogenesis
Lung fibrotic disorders
Characterized by accumulation of fibroblasts, myofibroblasts, and extracellular matrix leading to chronic respiratory failure.
Origins and activation of fibroblasts unknown
What are some risk factors to ILD?
Smoking
Bronchiolitis associated ILD
Desquamative interstitial pneumonia
Pulmonary Langerhans cell histiocytosis
What are some risk factors to ILD?
COPD
Higher risk for development of ILD
What are some risk factors to ILD?
Rheumatoid arthritis
Older age and male gender increases risk
What are some risk factors to ILD?
Exposure to radiation therapy
What type of laboratory test are done for ILD?
CBC, calcium, creatine, BNP. Liver function test, and urine sample.
Additional blood tests include those to detect the presence of autoantibodies, precipitating immunoglobulins against organic antigens, and serum angiotensin converting enzyme.
Lung function tests
usually show restrictive disease, useful but not diagnostic
What results would LFT show for ILD
Reduced values, Normal or elevated FEV1/FVC, Reduced diffusing capacity (DLCO)
Due to V/Q mismatch
What will ABG result show?
Early ILD
Normal or mild hypoxemia/respiratory alkalosis at rest
What will ABG result show?
Late ILD
Hypercapnea
What will ABG result show?
Exercise test
Six-minute walk (6MWT) with pulse oximetry
Cardiopulmonary exercise test
Overnight pulse oximetry
Why is cardiac assessment done?
Need to rule out heart failure as differential diagnosis.
If HF or PH is suspected, BNP is measured
What will x-ray reveal?
CXR will show changes when symptoms are present.
Can be normal with some cases when lung involvement is mild.
Which is more accurate CT or x-ray?
High-resolution CT
Provides greater accuracy.
Most appropriate protocol for ILD
What typical patterns will be seen?
Reticulation
Honeycombing
Ground glass appearance
Consolidation
Micronodules
Is tissue sampling (biopsy) effective?
Most likely method of definitive diagnosis is surgical lung biopsy.
What treatment should provide a plan for each of the following?
What pharmacological agent(s) are appropriate, if any?
How will disease progression be monitored?
Should a lung transplant referral be made?
Is the disease end stage?
Is the disease likely to respond to therapy?
Is supportive, palliative care for the patient the best approach?
What treatment or management is needed?
Dependent on underlying disease and primary histologic classification
May consist of immunosuppressive drugs
What treatment or management is needed?
Corticosteroids
Nintedanib
Intracellular inhibitor
Pirfenidone
Antifibrotic, anti-inflammatory, and antioxidant
Does treatment include O2 therapy?
Oxygen
Hypoxemia is a common finding with ILD
Pulmonary rehab
Not well studied
Can improve quality of life
How can it be prevented?
Vaccinations/avoidance of infection
For long term survival what is a permanent treatment?
Lung transplant
May be required for ongoing survival
Potentially curative option
Timely referral needed
Rate of decline in FVC and DLCO predictor of requirement
What is the prognosis?
Depends on type of ILD and response to therapy
Better chance of survival if:
Female
Younger
Have less dyspnea
More significant response with lavage at the onset of symptoms
Overall prognosis poor chance of survival is less
