Intro to DPLD Flashcards
What are the four main classifications of ILD?
1) diseases of known cause or association
2) idiopathic interstitial pneumonias
3) granulomatous diseases
4) miscellaneous interstitial diseases
Interstitial lung diseases (ILDs) also go by what name?
diffuse parenchymal lung diseases
What is the defining feature of ILD’s histologically speaking?
Damage to the alveolar wall and interstitium
What is pneumoconiosis?
DPLD of known causes from occupational exposure to inorganic dusts
What is hypersensitivity pneumonitis?
DPLD of known causes from an immunologic response to inhaled organic dusts
DPLDs caused by reactions to chemicals, drugs, radiation and toxic gases or associated with connective tissue diseas fall under what category of DPLD?
known cause
Idiopathic pulmonary fibrosis (IPF) and
Idiopathic nonspecific interstitial pneumonia (NSIP) are what category of idiopathic interstitial pneumonias (IIPs)?
chronic fibrosing
What are the 2 smoking related IIPs
Respiratory bronchiolitis-interstitial lung disease (RB-ILD)
Desquamative interstitial pneumonia (DIP)
What are the 2 acute/subacute IIPs?
Cryptogenic organizing pneumonia (COP)
Acute interstitial pneumonia (AIP)
What is the most common class of idiopathic interstitial pneumonias?
chronic fibrosing IIPs
Sarcoidosis and hypersensitivity pneumonitis are what kind of DPLD?
granulomatous
hypersensitivity is also a DPLD of known cause
What is the most frequent presenting symptom of DPLD?
dyspnea
followed by non productive cough
What is the most common finding on pulmonary exam in DPLD?
inspiratory crackles (especially IPF)
Most ILD’s have what pattern on Xray?
reticular or reticulonodular, typically at the bases
What CXR finding is common in sarcoidosis?
hilar lymphadenopathy
What imaging technique is used in diagnosing DPLD?
high resolution CT
What are characteristic IPF patterns in HRCT?
lower lobe-predominant peripheral reticular opacities, traction bronchiectasis and honeycombing
ground glass opacities on HRCT are common in what form of DPLD?
NSIP
upper- or mid-lung-zone-predominant peribronchovascular nodules with hilar and mediastinal lymphadenopathy are common HRCT findings in what form of DPLD?
sarcoidosis
What kind of testing is useful in diagnosing connective tissue disease associated ILD?
auto-antibody serology
What elevated enzyme is associated with sarcoidosis?
ACE
not sensitive or specific
What kind of defect is found in PFT’s for ILDS?
restrictive with reduced diffusion capacity
An obstructive defect or mixed restrictive/obstructive defect may also be seen in sarcoidosis or hypersensitivity pneumonitis
When is bronchoscopy with trasnbronchial biopsy and bronchoalveolar lavage useful in diagnosing ILD?
good for sarcoidosis or lymphangitic carcinoma
Video-assisted thoracoscopic surgery (VATS) is used in what process?
surgical lung biopsy for diagnosis of many ILDs
Other than removing exposure, what is used in hypersensitivity pneumonitis?
Corticosteroids and other anti-inflammatory agents are often used; they are typically beneficial in sarcoidosis and hypersensitivity pneumonitis, and sometimes in connective tissue disease-associated ILD
Pirfenidone and nintedanib are both used in what disease? (anti-fibrotics)
IPF
What ILD does not generally respond to steroids?
IPF
What is the most common organ involved in sarcoidosis?
lungs
What disease commonly presents with dyspnea and cough; fatigue, fever, night sweats, weight loss and specific symptoms referable to involvement of other organs, as well as hilar/mediastinal lymphadenopathy and reticulonodular opacities, usually in a mid- and upper-lung-zone-predominant distribution and a restrictive defect?
sarcoidosis
If sarcoidosis does not remit wihin 2-5 years and granulomatous inflammation persists, what is the long term effect?
fibrosis and impaired function
bird proteins (“bird fancier’s disease), thermophylic actinomycetes in moldy hay (“farmer’s lung”), and Cladosporium or Mycobacterial species in hot tubs (“hot tub lung”) are all examples of what?
organic antigens that trigger HP
What immune responses play a role in HP?
T cell and humeral
Acute dyspnea, often accompanied by fever, chills and malaise, within hours after handling birds, hay, or being in a hot tub . This is followed by resolution of symptoms within one to a few days.. What disease does this desribe?
hP
Is chronic HP often preceded by acute symtpoms?
no
HRCT findings include centrilobular ground-glass nodular opacities, often with an upper-lobe predominant distribution, and signs of air trapping. Chest imaging show reticular opacities, traction bronchiectasis and honeycombing indicative of fibrosis. On pulmonary function testing, restrictive, obstructive and mixed defects are found, as are decreases in diffusing capacity. Bronchoscopy with bronchoalveolar lavage reveals a cell profile with increased lymphocytes. Lung biopsy findings include airway and alveolar inflammation with large numbers of lymphocytes and poorly formed non-caseating granulomas peribronchially. What disease does this describe?
chronic HP
What is the difference between HP granulomas and sarcoidosis?
The loose granulomas in HP contrast with the tightly packed, organized epithelioid granulomas seen in sarcoidosis.
What is the most important part of treating HP?
antigen avoidance
can also use corticosteroids and immunosuppression in progressive disease
Which pneumocytes are most susceptible to injury?
type I
