287 - Interstitial Lung Disease Flashcards
ILD is a group of diseases involving the ___ of the lung.
Parenchyma
Typical presentation of ILD includes: (5)
- Dyspnea
- Dry stubborn cough
- Hemoptysis
- Wheezing
- Chest pain
In order to achieve diagnosis of ILD we need the combination of the following: ___ signs, ___ results, PFT (___), ___ (HRCT etc..), ___.
signs results pulmonary function tests Imaging Histopathology
What are the 4 etiological groups of ILD?
- Exposure (occupational/treatment related)
- Systemic disease (connective tissue/granulomatous)
- Idiopathic interstitial pneumonias (IPF/nonspecific)
- Other
IPF is more common when >___ years of age. Nevertheless, ___ and ___ are more common between the ages of ___
60
Sarcoidosis
CTD (Connective tissue disease)
20-40
LAM (lymph-angio-leio-myo-matosis) is more common in young ___. Most CTD (___) are in female (beside ___- more common in male).
women
connective tissue disease
RA
In ILD diseases, acute clinical presentation (days-weeks)- is more characteristic to ___, and __. Otherwise, ILD has an ___ presentation (months-years)
HP (hypersensitivity pneumonitis)
GPA (granulomatosis with polyangiitis)
Indolent
The most common complaints in ILD are ___ and ___, but they do not always present.
Progressive exertional dyspnea
Dry cough
Many drugs may cause ILD. mention 5:
- MTX
- Rituximab
- Amiodarone
- Bleomycin
- Nitrofurantoin
End inspiratory ____ or ___ noted at the ___ are found in most patients with IPF and may be one of the earliest signs of the disease.
Fine crackles
Rales
Lung bases
Most ILD will lead to a restrictive disturbance with decreased ___, ___, ___, ___.
TLC (total lung capacity)
FEV1 (forced expiratory volume)
FVC (forced vital capacity)
DLCO (Diffusing capacity carbon monoxide)
The common imaging test for suspected ILD is ___. It can help assessing the severity of the disease (___) and rule out comorbidities (___, ___). It also helps directing biopsy.
HRCT
Honeycombing
Emphysema
Malignancy
Biopsy can diagnose ___ diseases.
Granulomatous
Surgical biopsy with video guidance may __ the length of hospitalization. It is more beneficial when performed ___ the initiating ___
reduce
Before
Treatment
IPF becomes more common with age, usually diagnose in the ___ decade of life, more in ___, usually with a history of smoking or occupational exposure. The prognosis is bad, ___ survival for ___ years
5-6
Men
50%
3-5
The treatment for IPF includes anti fibrotic agents such as ___, ____ (slows down the disease), respiratory ___, ___ and ___ transplantation.
Nintedanib Pirfenidone physiotherapy Oxygen Lung
What does NSIP stands for>
Non specific interstitial pneumonia
NSIP is more common in non smoking ___ in the ___ decade of life. It has a good prognosis (>___ survival in 5 years), and even better in patients with cellular NSIP pattern.
Women
5th
80%
When performing HRCT in NSIP (nonspecific interstitial pneumonia ) we may find diffused subpleural reticular opacities (____). Decreased ___ and traction bronchiectasis ___.
Ground glass
TLC
bronchiectasis
Histopathology of NSIP includes ____ / uniform ____. The look of it is classified as ___ or __
Inflammation
fibrosis
Cellular
Fibrotic
Treating NSIP is based on Immunosuppression with: (3)
Steroids
Cytotoxic drugs
rituximab
What is the syndrome associated with acute IIP (Idiopathic interstitial pneumonia)?
Hamman-Rich syndrome
IIP (idiopathic interstitial pneumonia) is rare but usually leads to ___. It present itself with respiratory __ and blood ___. Usually there is a ___ like prodrome.
death
distress
Hypoxia
UTRI
HRCT of IIP will show: ___ and ___ in dependent areas. Treatment will be with ___ ventilation
Patchy ground glass
Consolidations
Mechanical
In systemic sclerosis, ILD will present in __% of patients, sometimes with pulmonary ___.
50
HTN
ILD due to rheumatoid arthritis is more common in ___ and after ___ exposure.
Men
Tabaco
ILD due to dermatomyositis/polymyositis appear in up to ___ of patients with __ Ab.
45%
Anti synthetase
The most common granulomatous ILD is ____.
Sarcoidosis
GPA (Wegener’s disease)- we will see the involvement of the: lungs, ___, ___ ,___, ___.
Ears
Nose
Throat
Kidney
Eosinophilic GPA (Churg-Strauss syndrome): the blood vessels infiltrations are ____. It is common to see chronic ____, ____. ___ is found in peripheral blood
Eosinophilic
Sinusitis
Asthma
Eosinophilia
In ILD __, ___, ___ will be decreased
TLC
FEV
FVC
Fibrotic bronchoscopy- the fluid can diagnose __ and cytology can suggest ___ or ___
DAH (Diffuse alveolar hemorrhage)
Eosinophilic pneumonia
Lipoid pneumonia
Hemoptysis may suggest DAH (__), GPA (__) or LAM (__).
diffuse alveolar hemorrhage
Eosinophilic granulomatosis with polyangiitis (Churg Strauss)
lymphangioleiomyomatosis
Fatigue is common in all ILD patients, while chest discomfort is common mostly in ___
sarcoidosis
ILD causes ___ and ___, mainly between the ___ and the ___ layers.
inflammation
Fibrosis
Epithelial
Endothelial
Treatment for systemic sclerosis will include __ and ___. In scleroderma patients- consider ___ / surgery to reduce reflux
Cyclophosphamide
Mycophenolate
PPI
