ILD

Question 1

What is ILD

Answer 1

◦ Heterogeneous conditions (>200)
◦ Lung parenchyma involvement
◦ Varying degrees of inflammation/fibrosis
◦ Remodeling of interstitial space
◦ Region between epithelium & endothelium
◦ Usual dominant site of involvement
High rates of morbidity & mortality
Prognosis dependent
◦ Disease extent & specificity
Classification System
◦ ILD with known vs unknown causes
◦ Limitations with approach

Question 2

S/s of PAH

Answer 2

Progressive exertional dyspnea or persistent dry cough
• Progressive dyspnea most common complaint
• Dry cough prominent symptom in IPF
Fatigue
• Common to all ILDs
Cough with hemoptysis
• ILD associated diffuse alveolar hemorrhage
• Goodpasture’s Syndrome
• Other etiology
Chest pain
• Rare in most cases
• Exception of sarcoidosis
Non-respiratory symptomology related to multisystem disorder

Question 3

Physical findings of ILD

Answer 3

Physical Exam
◦ End inspiratory fine crackles or rales
◦ Lung bases
◦ Most patients with IPF
◦ Earliest signs of disease
◦ Found many forms of ILD

Question 4

Signs of advanced ILD

Answer 4

Signs of advanced disease
◦ Cyanosis
◦ Digital clubbing
◦ Cor pulmonale

Question 5

Describe usual interstital pneumonia

Answer 5

Patchy interstitial fibrosis
◦ Alternating areas of normal lung
II. Temporal heterogeneity of fibrosis
◦ Scattered fibroblastic foci in the background of dense acellular collagen
III. Architectural alteration
◦ Chronic scarring or honeycomb change
Acute presentations
◦ Considered days to weeks
◦ Commonly misdiagnosed as more common diseases
◦ Types of ILD that can present
◦ Eosinophilic pneumonia
◦ Acute interstitial pneumonia (AIP)
◦ Granulomatosis with polyangiitis

Question 6

What imaging and procedures should be ordered for ILD

Answer 6

Chest Imaging
◦ CXR can be the first clinical indication of ILD
◦ Enlarged hilar lymph nodes
◦ Central nodular opacities in mid to upper lung zones
High Resolution CT (HRCT)
◦ Standard of care as initial evaluation
◦ Define the extent of ILD
◦ Features suggestive of advanced disease
◦ Identify coexisting diseases
◦ Provides viable biopsy locations.
Lung Biopsy
◦ Bronchoscopy
◦ Examination of serial lavage fluid & cellular examination
◦ Surgical lung biopsy
◦ VATS procedures

Question 7

What is idiopathic pulmonary fibrosis

Answer 7

Most common ILD of unknown cause
◦ Associated Hx of smoking or other environmental exposures
◦ Commonly diagnosed in 5th or 6th decade of life
◦ Affects men more than women
◦ Progressive disease with poor prognosi

Question 8

CT results of idiopathic pulmonary fibrosis

Answer 8

Chest CT
◦ Subpleural reticulation
◦ Honeycombing and traction bronchiectasis
◦ Usual interstitial pneumonia (UIP) pattern

Question 9

Treatment plan for idiopathic pulmonary fibrosis

Answer 9

Treatment Plan
◦ Antifibrotic therapy (i.e. Perfinidone)
◦ Physical therapy & supplemental 02
◦ Lung transplantation

Question 10

Describe non-specific interstitial pneumonia

Answer 10

Distinct clinical entity with characteristic features
◦ Commonly observed with connective tissue diseases
◦ Non-smoking females
◦ 5th decade of life

Question 11

S/s of non-specific interstital pneumonia

Answer 11

Clinical Manifestations
◦ High-resolution CT (HRCT) scan
◦ Diffuse subpleural, symmetric, ground glass & reticular opacities
◦ Volume loss & traction bronchiectasis
◦ Diagnostic lung biopsy
◦ Interstitial inflammation & fibrosis with a uniform appearance

Question 12

Treatment of non-specific interstitial penumonia

Answer 12

Treatment Plan
◦ Oral steroids
◦ Cytotoxic & biologic agents
◦ Oxygen therapy & pulmonary rehabilitation
◦ Lung transplant Credit: https://www.merckmanuals.com/

Question 13

What is cryptogenic organizing pneumonia; include s/s and treatment

Answer 13

Occurs in 5th & 6th decade of life
◦ Presents w/ subacute flu like illness, cough, dyspnea, fever & fatigue
◦ Commonly mistaken for pneumonia
◦ Possible underlying causes
◦ Connective tissue disorder, medications or malignancy
Clinical Findings
◦ Inspiratory rales
◦ Restrictive lung deficits with hypoxemia
◦ HRCT scan
◦ Patchy subpleural consolidative opacities
◦ Associated ground glass opacities
◦ Surgical lung biopsy
◦ Patchy regions of organizing pneumonia with granulation tissue
Treatment Plan
◦ Corticosteroids

Question 14

Connective tissues ILDs, s/s, and treatment

Answer 14

Common associated connective tissue diseases
◦ Scleroderma, rheumatoid arthritis, polymyositis, Sjogren’s syndrome, lupus
Clinical Manifestations
◦ Pulmonary hypertension
◦ Separately or concomitantly
◦ Imaging findings comparable to NSIP & IPF
◦ Additional findings
◦ Dilated esophagus
◦ Pulmonary artery enlargement
Treatment Plan
◦ Mycophenolate
◦ Minimizing acid reflux
◦ Lung transplant

Question 15

What are the s/s of sarcoidosis lung disease

Answer 15

verview
◦ Systemic disease of unclear etiology
◦ Granulomatosis inflammation of lung
◦ Onset of disease ~3rd or 4th decade of life
Clinical Manifestations
◦ Insidious onset
◦ Malaise, fever, dyspnea
◦ Erythema nodosum
◦ Lupus pernio
◦ Iritis
◦ Cardiomyopathy S/S
◦ Parotid gland enlargement
◦ Hepatosplenomegaly
◦ Lymphadenopathy

Question 16

Lab and imaging findings in sarcoidosis lung disease

Answer 16

Leukopenia
◦ Elevated ESR
◦ Hypercalcemia or hypercalciuria.
◦ Elevated angioconverting enzyme
◦ Pulmonary function testing
◦ Restrictive changes w/ decreased lung volumes & diffusing capacity
◦ Air flow obstruction
◦ EKG w/ conduction disturbances & dysrhythmias
◦ Chest imaging
◦ Diffuse reticular infiltrates, focal infiltrates, nodules & cavitations
◦ Fibrotic changes in upper lobes

Question 17

Diagnostics and treatment plan for sarcoidosis ILD

Answer 17

iagnosis
◦ Multiple organ involvement
◦ Biopsy
◦ Noncaseating granuloma
Treatment Plan
◦ Referral to Ophthalmology
◦ Oral corticosteroids (Prednisone 0.5 -1.0 mg/kg/day)
◦ Initiate for disabling constitutional symptoms
◦ Immunosuppressive agents
◦ Corticosteroid intolerance
◦ Corticosteroid refractory disease