168: IIPs (Idiopathic Interstitial Pneumonias) Flashcards
Which IIP has a clinical presentation similar to infectious pneumonia?
COP: Cryptogenic Organizing Pneumonia
- Subacute fever
- Malaise
- Cough
- Dyspnea
- Migratory, multifocal peripheral opacities
Often, patients are treated with antibiotics without improvement
What are the two major IIPs that are most common in current smokers?
Respiratory Bronchiolitis ILD (RB-ILD)
Desquamative Interstitial Pneumonia (DIP)
Would have ground glass opacities/nodules.
Let go of the details
You suspect that your patient has an IIP due to their cough and dyspnea without an apparent cause.
Their HRCT shows bilateral, basilar-predominant, sub-pleural reticulation and traction bronchiectasis
Is this consistent with any of the IIPs? If not, what would you suspect?
This is consistent with IPF (Idiopathic Pulmonary Fibrosis)
The HRCT shows a definitive UIP pattern, and if the clinical history is consistent you can diagnose IPF without lung biopsy
(if the HRCT is not definitive, lung biopsy is required)
What is required to diagnose IPF?
- Definitive
- HRCT showing fibrosis in a UIP (Usual Interstitial Pneumonia) pattern + Clinical history
- Bilateral, basilar-predominant, sub-pleural
- Reticulation, traction bronchiectasis and/or honeycombing
- No biopsy necessary
- HRCT showing fibrosis in a UIP (Usual Interstitial Pneumonia) pattern + Clinical history
- If HRCT does not provide definitive evidence for or against IPF–> lung biopsy
- Lung biopsy showing UIP pattern
- Fibrosis with temoral and spatial heterogeneity
- Sub-pleural involvment
- Fibroblastic foci
- Microscopic honeycombing
- Lung biopsy showing UIP pattern
Describe the clinical presentation of IPF (Interstitial Pulmonary Fibrosis)
- Symptoms/Physical exam:
- Dyspnea, cough, crackles, restrictive physiology with no obvious trigger
- History:
- Adults >65 y/o
- History of cigarette smoking
- Family history
A patient presents to your clinic with cough, dyspnea, and fatige for the last several months.
What additonal symptoms would make you suspicious for an underlying connective tissue disease?
- Rash (malar rash with lupus)
- Joint pain or deformities (with RA)
- Mechanics hands with antisynthetase syndrome
- Grottons papules with dermatomyositis
- Other symptoms
- Muscle aches
- Unexplained organ dysfunction
- Skin changes
- Synovitis (joint inflammation)
What are the two major Acute/Subacute IIPs?
Acute Interstitial Pneumonia (AIP) - very sick
Cryptogenic Organizing Pneumonia (COP)
Let go of the details
What are the two major chronic, fibrosing IIPs?
Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic Nonspecific Interstitial Pneumonia (NSIP)
You suspect that your patient has an IIP due to their cough and dyspnea without an apparent cause.
Their HRCT shows predominant ground glass opacities and uniform fibrosis.
Is this consistent with any of the IIPs? If not, what would you suspect?
This is consistent with a chronic fibrosing IIP, and not consistent with IPF.
If a surgical lung biopsy is also not consistent with IPF, you might suspect NSIP (non-specific interstital pneumonia) which is basically just a chronic, fibrosing IIP that is not consistent with IPF
However, an NSIP pattern is commonly seen in CTD and drug-induced pneumonitis. Re-evaluate underlying causes before diagnosing idopathic NSIP
You suspect that your patient has an IIP due to their cough and dyspnea without an apparent cause.
They are a current smoker with a 10 pack/year history of smoking. Their HRCT shows ground glass opacities and nodules
Is this consistent with any of the IIPs? If not, what would you suspect?
This is consistent with respiratory bronchiolitis-associated ILD (RB-ILD) or desquamative interstitial pneumonia (DIP), which is are both smoking-related IIPs.
- HRCT findings:
- Ground glass opacities
- Centrilobular nodules
- Clinical history: Smoking
You suspect that your patient has an IIP due to their cough and dyspnea without an apparent cause.
They do not have a history of lung disease, but they are acutely ill.
Is this consistent with any of the IIPs? If not, what would you suspect?
This is consistent with Acute Interstitial Pneumonia (AIP) (very seriously sick) or Cryptogenic Organizing Pneumonia (COP)
Let go of the details.
You suspect that your patient has an IIP due to their cough and dyspnea without an apparent cause.
Their chest radiograph shows lilateral hilar adenopathy and a reticular pattern in the upper lobes of the lung. You can also see areas of late-stage fibrosis.
Is this consistent with any of the IIPs? If not, what would you suspect?
This is not consistent with IIP
This pattern would lead you to suspect sarcoidosis, especially if the patient is younger and has multisystem involvement
Confirmed by tighly-packed, well-formed granulomas on biopsy
What questions should you be asking yourself if you suspect that a patient has IIP?
What answers are constent with IIP?
- Is there an apparent cause of your patient’s illness (No)
- Is this sarcoidosis (No)
- Are there eosinophils (No)
***If 1-3 = No , you are probably dealing with an IIP***
4. Is the patient acutely ill without prior history of lung disease?
If yes, either AIP or COP
If no, move on to Q5
5. Is the patient an active smoker with predominantly ground glass opacities and/or nodules on chest CT?
If yes, most likely RB-ILD or DIP
If no, move on to Q6
6. Is this idopathic pulmonary fibrosis?
If there is UIP pattern on HRCT, this is likely
7. Is this nonspecific interstitial pneumonia?
If HRCT findings to not fit a definite UIP pattern, this is likely NSIP
What is the difference between IIP and IPF?
IPF is a type of IIP
Specifically, IFP is a chronic, fibrosing IIP
IPF has worse outcomes. And you DO NOT treat with immunosuppression
Describe the clinical presentation of COP
Similar to infectious pneumonia
- Subacute fever
- Malaise
- Cough
- Dyspnea
- Migratory, multifocal peripheral opacities
