Flashcards in Diffuse Parenchymal Lung Disease Deck (36)
1
What can effect the interstitium
- edema
- malignancy
- infections
- inflammation and fibrosis (INTERSTITIAL LUNG DISEASE)
2
Mechanism of interstitial lung disease
1. lung injury: environment, autoimmune, unknown (can lead to 2 or 3)
2. inflammation: this can lead to possible resolve or 3
3. lung fibrosis
3
Causes of primary interstitial lung disease (ILD)
- idiopathic interstitial pneumonias
- sarcoidosis
4
Causes of secondary ILD
- drugs
- exposures/occupations
- radiation
- aspiration
- connective tissue disease related ILD (autoimmune)
- smoking-related
5
Important aspect to diagnosing ILD
- taking a good history
6
Types of diffuse parenchymal lung disease
- smoking related
- environmental exposure
- granulomatous
- autoimmune
- idiopathic interstitial pneumonias
7
Signs of sarcoidosis
- uveitis
- erythema nodosum (bruises on shins)
- noncaseating granulomas
8
Person to first diagnose someone with sarcoidosis & when
J. Hutchinson - 1878
9
Systems effected by sarcoidosis
- multisystem disease: every system can be effected
- most common: lung (95%), eyes, lymph nodes, liver, skin
10
Who gets sarcoidosis
- all ages, gender, races
- familial
11
Incidence/Prevalence of sarcoidosis
- highest in african american women
- norther european african americans (sweden)
12
Clinical manifestations of sarcoidosis by country
- US/Europe: respiratory disease
- Japanese: cardiac & eye disease
13
What defines sarcoidosis
- granuloma with no identifiable bug
14
What is a granuloma
- macrophages, epitheliod cells, multinucleated giant cells
- surrounded by CD4 T cells (Th1) w/ few CD8 and B cells
15
How do patients present w/ sarcoidosis
- skin abnormality
- fevers, night sweats, fatigue, weight loss
- cough, shortness of breath
- chest pain, palpitations, arrhythmia
- NO symptoms at all**
16
Aspects needed to diagnose sarcoidosis
- clinical symptoms
- radiograph
- biopsy w/ pathology
****MUST RULE OUT ANY OTHER CAUSE: infections, cancer, foreign bodies, inhalations****
17
Meaning behind staging of sarcoidosis
- doesn't mean you have a worse prognosis
- deals with how you will treat the patient
18
Clinical course of sarcoidosis
- spontaneous remission: 50-70%
- wax and wane-chronic and persistent: 30-50%
- progressive: 1-5%
19
Treatment of sarcoidosis
- lacking evidence
- vital organ involvement such as eye, cardiac, neuro: corticosteroids
- other immunosuppressants
20
Clinical case of fibrosis
- old age
- chronic
21
Physical exam findings of fibrosis
- low O2 when walking
- VELCRO crackles w/ NO wheezing
- mild clubbing
22
Percentage of idiopathic pulmonary fibrosis (IPF) patients that have crackles
80%
23
Percentage of IPF patients that have clubbing
50%
24
Pulmonary function test results of IPF patients
- low FVC, FEV, total lung capacity, diffusing capacity
25
Type of lung disease in IPF
- restrictive lung disease w/ severely reduced diffusing capacity
26
Radiograph findings of IPF patient
- reduced lung volume
- honeycombing
- lower lobe predominance
27
Diagnosing idiopathic pulmonary fibrosis (IPF)
- exclude other known causes of pulmonary fibrosis
- abnormal pulmonary function tests
- features of pulmonary fibrosis on CT scan
- usual interstitial pneumonia an open lung biopsy
28
Other causes of pulmonary fibrosis
- medications: amiodarone, nitrofurantoin, cocaine/heroin, mineral oil, chemo, anti TNF
- medical history: autoimmune disease
29
Social history showing possible causes of IPF
- smoking, occupation, hobbies, inhalations, drugs, mold exposure, pets (BIRDS ARE BAD), hot tubs (mycobateria)
30
Mechanism of injury in IPF
insult to epithelium and basement membrane--aberrant wound healing or vascular injury--fibrotic foci (fibroblast proliferation, collagen deposition)--Fibrosis (circulating fibroblasts also contribute)
***INFLAMMATION does NOT play a roel***
31
Does inflammation play a role in IPF
NO INFLAMMATION DOES NOT PLAY A ROLE
32
Another name for idiopathic pulmonary fibrosis
usual interstitial pneumonia
33
Median survival of patient w/ IPF
3-5 years
34
Treatment for IPF
- anti fibrotics: prifenidone, nintedanib
- pulmonary rehab
- oxygen
- lung transplant
35
Diffuse parenchymal lung disease types and treatments
- smoking related: stop smoking
- environmental exposure: remove exposure
- granulomatous: steroids/immunosuppressants
- idiopathic interstitial pneumonias: steroids/immuno/ANTIFIBROTIC
- autoimmune/collagen vascular disease: steroids/immuno
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