INTERNAL MEDICINE EXIMIUS

INTERSTITIAL LUNG DISEASE 2021

JENA LYNN M. ALAN Nov, 2019

IPF occurs most commonly in patients aged >60 and is

INTERSTITIAL LUNG DISEASE quite rare among patients aged <50
 Diffuse parenchymal lung diseases include a large number sarcoidosis, CTD associated ILD, and less common ILDs
(>200) of heterogeneous conditions that affect the lung such as LAM, pulmonary Langerhans cell histiocytosis
parenchyma with varying degrees of inflammation and (PLCH) tend to present between the ages of 20 and 40
fibrosis c. Duration of Symptoms
 difficult to diagnose ◦ Acute presentations (days to weeks) – unusual,
 combined weight of clinical data, laboratory studies, eosinophilic pneumonia, acute interstitial pneumonia
pulmonary function testing, imaging findings, and (AIP), HP, and granulomatosis with polyangiitis (GPA)
histopathology (if obtained) are jointly required to make a ◦ subacute presentations (weeks to months)-
confident diagnosis sarcoidosis, CTD associated ILD, drug-induced ILD, or
 recommend a multidisciplinary approach to the diagnosis COP
NOTE: ◦ chronic indolent presentation (months to years)- IPF
- Owing to a variety of clinical presentations, as well as overlapping d. Respiratory Symptoms
imaging and histopathologic findings (Table 287-1), ILDs can be ◦ Progressive dyspnea with exertion- MC
- No single piece of data confers a diagnosis alone ◦ Dry cough, fatigue
- An example of a multidisciplinary approach might include a NOTE: and are commonly misdiagnosed as more
conference attended by pulmonologists, rheumatologists, common diseases such as pneumonia, a COPD
radiologists, and pathologists where all of the data generated on a exacerbation, or heart failure.
patient can be discussed and reviewed jointly by those with unique
sets of expertise in the care of patients with ILD. e. Past Medical History
Classification of ILD ◦ personal history of a CTD or a history of symptoms
commonly associated with a CTD
◦ history of malignancy- dermatomyositis associated
COP and sarcoid-like reactions
◦ history of asthma and allergic rhinitis- eosinophilic
GPA
f. Medications
◦ methotrexate, azathioprine, rituximab, and the tumor-
necrosis factor-alpha blocking agents
◦ antibiotics (e.g., nitrofurantoin), anti-arrhythmics (e.g.,
amiodarone) and anti-neoplastic agents (e.g.,
bleomycin
g. Family History
◦ presence of a close relative with an IIP is among the
strongest risk factors for IPF
◦ mucin gene (MUC5B), telomerase reverse
transcriptase gene (TERT) - associated with both
familial and idiopathic forms of pulmonary fibrosis
h. Social History
Note: ◦ smoking - respiratory bronchiolitis and desquamative
- Unknown causes of ILD include groups of rare disorders often with interstitial pneumonia (DIP)
classic presentations (e.g., a spontaneous pneumothorax in a young NOTE:
female with diffuse cystic changes on a chest CT might suggest - The most well replicated genetic factors for pulmonary fibrosis (a
lymphangioleiomyomatosis [LAM]) and the most common group of promoter variant of a mucin gene [MUC5B]) and various genetic
ILDs, the idiopathic interstitial pneumonias (IIPs). determinants known to influence telomere length (e.g., variants in
the telomerase reverse transcriptase gene [TERT]) appear to be
Diagnostic Approach associated with both familial and idiopathic forms of pulmonary
History fibrosis similarly.
a. Age - Sometimes referred by pathologists jointly as smoking related—
◦ 60 y/o- IPF ILD)
◦ 20 – 40 y/0- sarcoidosis, CTD associated ILD, and less
common ILDs such as LAM, pulmonary Langerhans Physical Examination
cell histiocytosis (PLCH)  End-inspiratory fine crackles, or rales at the lung bases
b. Sex  Signs of advanced disease include cyanosis, digital clubbing,
◦ LAM- young women and cor pulmonale
◦ CTD-associated ILDs are more common among NOTE: are found in most patients with IPF and may be one of the
women, except RA associated ILD which is more earliest signs of the disease
common among men
◦ IPF and occupational/exposure-related ILDs – men

Pulmonary Function Test

NOTE: • restrictive

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 INTERNAL MEDICINE EXIMIUS
INTERSTITIAL LUNG DISEASE 2021
JENA LYNN M. ALAN Nov, 2019

• reduced TLC, FEV1 and FVC • UIP pattern

• A reduction in the diffusing capacity of the lung for carbon Treatment:
monoxide (DLCO) • lung transplant, supplemental oxygen, physical therapy
• rarely diagnostic
• characterize the extent of disease, decline in repeated CRYPTOGENIC ORGANIZING PNEUMONIA(COP)
measures are correlated with an elevated rate of mortality Clinical Manifestations:
• 50–60 y/o
Chest Imaging • subacute flu-like illness, with cough, dyspnea, fever, and
Chest Xray fatigue
• can be the first clinical indication that an ILD might be present • Inspiratory rales are often present
• enlarged hilar lymph nodes and a pattern of central nodular • restrictive lung deficits on PFT
opacities in the mid to upper lung zones suggest sarcoidosis • commonly mistaken for pneumonia
• A basilar reticular pattern, with small cystic spaces, in the • can occur in isolation or can be secondary to an underlying
absence of clinical evidence for heart failure- IPF connective tissue disease (e.g., polymyositis), medications, or
can result from an underlying malignancy
High resolution chest CT (HRCT) HRCT Image Findings
• standard of care in the initial evaluation • patchy, sometimes migratory, subpleural consolidative
• define the extent of the ILD, the presence of more concerning opacities often with associated ground-glass opacities
features suggestive of advanced disease (e.g., honeycombing), • rim of subpleural sparing (reversed halo or atoll sign)
can provide information on coexisting diseases (e.g Treatment
emphysema and lung cancer) • Corticosteroids
NOTE: With a few exceptions, CXR alone rarely leads to a specific
diagnosis ACUTE INTERSTITIAL PNEUMONIA (HAMMAN-RICH SYNDROME)
Clinical Manifestation
Lung Biopsy • rare and often fatal
• Fiberoptic Bronchoscopy • acute onset of respiratory distress and hypoxemia
- helpful in establishing a specific ILD diagnosis • A prodromal period of symptoms consistent with an acute
• Surgical Lung Biopsy upper respiratory infection is common
• VATS • difficult to distinguish from acute respiratory distress
(see table) syndrome (ARDS)
IDIOPATHIC INTERSTITIAL PNEUMONIA HRCT Image Findings
IDIOPATHIC PULMONARY FIBROSIS • patchy bilateral ground-glass opacities
Clinical Manifestations: Histopathology
• most common ILD of unknown cause • diffuse alveolar damage (DAD)
• diagnosed in the 5th or 6th decade of life Treatment
• affects men more than women • supportive and often includes mechanical ventilation
• frequently associated with a history of smoking NOTE: Similar to ARDS and acute exacerbations of underlying
• progressive disease with poor prognosis pulmonary fibrosis, AIP presents histopathologically as
HRCT Image Findings
• honeycombing

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