Diffuse Parenchymal Lung Disease Flashcards
What can effect the interstitium
- edema
- malignancy
- infections
- inflammation and fibrosis (INTERSTITIAL LUNG DISEASE)
Mechanism of interstitial lung disease
- lung injury: environment, autoimmune, unknown (can lead to 2 or 3)
- inflammation: this can lead to possible resolve or 3
- lung fibrosis
Causes of primary interstitial lung disease (ILD)
- idiopathic interstitial pneumonias
- sarcoidosis
Causes of secondary ILD
- drugs
- exposures/occupations
- radiation
- aspiration
- connective tissue disease related ILD (autoimmune)
- smoking-related
Important aspect to diagnosing ILD
- taking a good history
Types of diffuse parenchymal lung disease
- smoking related
- environmental exposure
- granulomatous
- autoimmune
- idiopathic interstitial pneumonias
Signs of sarcoidosis
- uveitis
- erythema nodosum (bruises on shins)
- noncaseating granulomas
Person to first diagnose someone with sarcoidosis & when
J. Hutchinson - 1878
Systems effected by sarcoidosis
- multisystem disease: every system can be effected
- most common: lung (95%), eyes, lymph nodes, liver, skin
Who gets sarcoidosis
- all ages, gender, races
- familial
Incidence/Prevalence of sarcoidosis
- highest in african american women
- norther european african americans (sweden)
Clinical manifestations of sarcoidosis by country
- US/Europe: respiratory disease
- Japanese: cardiac & eye disease
What defines sarcoidosis
- granuloma with no identifiable bug
What is a granuloma
- macrophages, epitheliod cells, multinucleated giant cells
- surrounded by CD4 T cells (Th1) w/ few CD8 and B cells
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**
Aspects needed to diagnose sarcoidosis
- clinical symptoms
- radiograph
- biopsy w/ pathology
- MUST RULE OUT ANY OTHER CAUSE: infections, cancer, foreign bodies, inhalations*
Meaning behind staging of sarcoidosis
- doesn’t mean you have a worse prognosis
- deals with how you will treat the patient
Clinical course of sarcoidosis
- spontaneous remission: 50-70%
- wax and wane-chronic and persistent: 30-50%
- progressive: 1-5%
Treatment of sarcoidosis
- lacking evidence
- vital organ involvement such as eye, cardiac, neuro: corticosteroids
- other immunosuppressants
Clinical case of fibrosis
- old age
- chronic
Physical exam findings of fibrosis
- low O2 when walking
- VELCRO crackles w/ NO wheezing
- mild clubbing
Percentage of idiopathic pulmonary fibrosis (IPF) patients that have crackles
80%
Percentage of IPF patients that have clubbing
50%
Pulmonary function test results of IPF patients
- low FVC, FEV, total lung capacity, diffusing capacity
Type of lung disease in IPF
- restrictive lung disease w/ severely reduced diffusing capacity
Radiograph findings of IPF patient
- reduced lung volume
- honeycombing
- lower lobe predominance
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
Other causes of pulmonary fibrosis
- medications: amiodarone, nitrofurantoin, cocaine/heroin, mineral oil, chemo, anti TNF
- medical history: autoimmune disease
Social history showing possible causes of IPF
- smoking, occupation, hobbies, inhalations, drugs, mold exposure, pets (BIRDS ARE BAD), hot tubs (mycobateria)
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
Does inflammation play a role in IPF
NO INFLAMMATION DOES NOT PLAY A ROLE
Another name for idiopathic pulmonary fibrosis
usual interstitial pneumonia
Median survival of patient w/ IPF
3-5 years
Treatment for IPF
- anti fibrotics: prifenidone, nintedanib
- pulmonary rehab
- oxygen
- lung transplant
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
How is the type of interstitial lung disease determined
- by the type, amount, location of cells
