SM 168 IIPs Flashcards
Where do all Interstitial Lung Diseases tend to effect the body?
Interstitial Lung Diseases primarily effect the interstitial space
Why are diffuse pulmonary metastases not considered Diffuse Pulmonary Lung Disease?
DPLD implies an unknown etiology, and cancer is a known etiology
What considerations help you make sure a suspected IIP is idiopathic?
Detailed history, physical exam, and connective tissue labs
What considerations rule out sarcoidosis in a suspected IIP?
Lack of: lymphadenopathy, upper-lobe nodules/fibroses, non-caseating granulomas, and multi-organ involvement
What considerations help you rule out eosinophilic disease in a suspected IIP?
Use a BAL to determine Eosinophil count
Does a prior history of DLPD support or rule out a diagnosis of IIP?
Support, and narrows down to AIP or COP
Does active smoking support or rule out a diagnosis of IIP?
Rule out?
What details are important in a history when considering a suspected IIP?
Pets, mold exposure, occupational exposures
What is the treatment for an elevated eosinophil count on a BAL?
Corticosteroid
What is AIP?
Acute Interstitial Pneumonia, an Idiopathic Interstitial Pneumonia (IIP)
How does AIP present?
AIP presents like ARDS, with acute hypoxemia, bilateral alveolar infiltrates and hyaline membranes, without a clear trigger
How is AIP differentiated from ARDS?
ARDS is due to a lack of surfactant, and is therefore not an IIP because it has a clear cause; AIP mimics ARDS but does not have a clear cause and is therefore an IIP
How does AIP show up on histology?
Like ARDS, it presents as Diffuse Alveolar Damage
How is AIP treated?
Supportive care primarily
What is COP?
Cryptogenic Organizing Pneumonia, an Idiopathic Interstitial Pneumonia (IIP), that presents with organizing pneumonia on histology
How does COP show up on histology?
COP presents as an organizing pneumonia
Why is a medication review important for IIP differential dx?
Medications like Amiodarone can cause lung damage = not an IIP
What basic lab work should be done to rule out IIP?
Connective tissue disease labs and hypersensitivity pneumonitis panel - if positive, not an IIP
What connective diseases are frequently screened for?
ANA = Lupus
Rheumatoid Arthritis
Myositis
What is the drawback of the hypersensitivity pneumonitis panel?
The major drawback is that because it screens for the presence of antibodies against inhaled antigens, it may not be specific in diagnosing Hypersensitivity Pneumonitis because the antibodies may circulate naturally
What is sarcoidosis?
A multisystem idiopathic granulomatous disease
Who is frequently effected by sarcoidosis?
Younger patients
Which disease causes bilateral hilar adenopathy and upper lobe parenchymal disease/nodules?
Sarcoidosis
How does Sarcoidosis present on CXR?
Bilateral hilar adenopathy and upper lobe nodules
What types of granulomas does Sarcoidosis form?
Well formed non-caseating granulomas
What non-lung symptoms might a Sarcoid patient have?
Nerve damage, cardiac arrhythmias, liver damage
How is management of COP and AIP different?
COP responds to steroids, AIP does not
How is the presentation of COP and AIP different?
Both are ILD’s, but AIP is much more severe while COP is subacute
Do ground glass opacities support or rule out IPF?
Rule out, and suggest smoking-induced disease
Why is a diagnosis of IPF important?
Low survival, and the only form of lung disease that is worsened by immunosuppression
What is the treatment regiment for IPF?
Antifibrotic therapy = Pirfenidone and Nintedanib, do not immunosuppress
Should corticosteroids be used in IPF?
NO, immunosuppression worsens IPF
What clinical features are consistent with IPF?
Cough and dyspnea without an obvious trigger, with a history of smoking
Is a history of smoking supportive of a diagnosis of IPF?
Surprisingly, yes
What radiographic features are consistent with IPF?
A UIP pattern on HRCT
What is a UIP pattern?
A pattern of HRCT that includes basal fibrosis and honeycombing, as well as reticulation
What is traction bronchiectasis and does it support IPF?
Traction bronchiectasis is dilation of an airway due to inflammation, and supports IPF
Does ground glass support IPF?
No, wrong radiographic pattern (UIP supports)
Does upper lobe fibrosis support IPF?
No, wrong radiographic pattern (lower lobe supports)
Does consolidation support IPF?
No, wrong radiographic patterns (consolidation =/= fibrosis)
How is consolidation different from ground glass?
Ground glass is semi-transparent on radiography while consolidation is thick white
Does peribronchovascular fibrosis support IPF?
No, wrong radiographic pattern (IPF effects the periphery of the lung)
Do diffuse micronodules support IPF?
No, wrong radiographic pattern (IPF is concentrated at the periphery of the lung)
What should be done if a patient seems to have inconclusive features of IPF?
Order a lung biopsy
What are the key features of the UIP pattern on Histology?
Spatial and Temporal Heterogeneity, Fibroblastic Foci, and Microscopic Honeycombing
How does a UIP pattern swing an IPF diagnosis?
If you see UIP in an otherwise inconclusive patient, diagnose IPF
Does the pharmacological treatment for IPF improve mortality?
Pirfenidone and Nintedanib do not improve mortality, but may improve lung function
What is the non-pharmacological treatment for IPF?
Supplemental O2 to treat hypoxia, positive pressure ventilation, flu vaccines, palliative care
