- uveitis - erythema nodosum (bruises on shins) - noncaseating granulomas

- all ages, gender, races - familial

- macrophages, epitheliod cells, multinucleated giant cells - surrounded by CD4 T cells (Th1) w/ few CD8 and B cells

Diffuse Parenchymal Lung Disease Flashcards by Ben Nigg

What can effect the interstitium

edema
malignancy
infections
inflammation and fibrosis (INTERSTITIAL LUNG DISEASE)

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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

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Causes of primary interstitial lung disease (ILD)

idiopathic interstitial pneumonias

- sarcoidosis

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Causes of secondary ILD

drugs
exposures/occupations
radiation
aspiration
connective tissue disease related ILD (autoimmune)
smoking-related

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Important aspect to diagnosing ILD

taking a good history

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Types of diffuse parenchymal lung disease

smoking related
environmental exposure
granulomatous
autoimmune
idiopathic interstitial pneumonias

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Signs of sarcoidosis

uveitis
erythema nodosum (bruises on shins)
noncaseating granulomas

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Person to first diagnose someone with sarcoidosis & when

J. Hutchinson - 1878

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Systems effected by sarcoidosis

multisystem disease: every system can be effected

- most common: lung (95%), eyes, lymph nodes, liver, skin

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Who gets sarcoidosis

all ages, gender, races

- familial

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Incidence/Prevalence of sarcoidosis

highest in african american women

- norther european african americans (sweden)

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Clinical manifestations of sarcoidosis by country

US/Europe: respiratory disease

- Japanese: cardiac & eye disease

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What defines sarcoidosis

granuloma with no identifiable bug

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What is a granuloma

macrophages, epitheliod cells, multinucleated giant cells

- surrounded by CD4 T cells (Th1) w/ few CD8 and B cells

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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**

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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