Lung fibrosis

(1)

Interstitial lung diseases

Restrictive lung diseases

Lung fibrosis

”small” lungs

(2)

Restriktive lung function

Also seen

without

problems in

the lung

parenkyma

(3)

In principle there

is a ”acute”

restriktive

disorder

Pleuraexsudate,

pneumonia

Atelektasis etc..

(4)

Disease localized in the paremkyma

What is lost is forever lost …..

…if treatment is not started in time…

(5)

Symptoms

• Slowly progession

– But attracts is prevalent

• Breathlessness

– At first at activity – Later all the time

• cough

– Non- productive • Signs

– cyanosis

– Low saturation • low lung function • Dromstikfingers

(6)

However the initial most often mistake diagnosis that

mis-interpretated as lung fibrosis is ???

COPD !!!!

Why??

(7)

COPD

FVC is falsely

decreased

“Air trapping”

“solved” by Slow vital capasity FVC<SVC

(8)

One clue is that the pt is obstruktive

Then wounder !!

(9)

(10)

Myths !

• Smoking does NOT normally cause lung

fibrosis

• However nothing without exceptions …..

Very rare lung diseases ……

(11)

(12)

(13)

(14)

(15)

Causes:

Restictive lung diseases

1. Changes in the lung parenkyma

2. Diseases in the pleura, chest wall, muscles and nerves

(16)

What happens in

lung fibrosis ??

(17)

(18)

(19)

(20)

Lung fibrosis

decreased ability

of oxygen optake

(21)

Findings

• Decrease in saturation under activity

– Later also at rest

• A-gas shows hypoxia not hypercapnia

– Only in the Terminal fase is hypercapnia seen.

• So no problem giving oxygen (in contrast

to COPD)

(22)

What do we have to measure in

patients with lung fibosis??

(23)

(24)

There is a slight difference in diagnosing

and monitoring the disease

• Lung function – Forced volumen – TLC, RV and DLCO • Anatomic changes – Bronkoscopy – HRCT scan – X-RTG Thorax – Ekko/hjertekat – Lungebiopsy – Dexa scanning • Serological changes – Blood tests • Activity

(25)

(26)

TLC: How big a the lungs ?

TLCO: How ”good” are the lungs to oxygen optake ?

(27)

6-min Walking

test

– How far?

– Desaturation?

– Symptoms severe ?

– The test accesses the physical ability? – Degree of severeness

– Disease development

– Guidance to when transplantation should be considered

(28)

HRCT- scan

• Changes

• distribution

• Pattern

• development

– Effect of treatment

– Changes in disease

Which disease ? Who bad ? Diffential diagnosis ? Further work-up ?

(29)

(30)

CT versus HRCT

(31)

(32)

(33)

Bloodtests

• Diagnosis

• Prognosis

(34)

Bloodtests

• Common bloodtests…..

• Extra Interstitial blood tests:

– ACE: Sarcoidose

– ANA: Relaterede to ex - (SLE,RA)

– ANCA: Wegeners granulomatosis

– Anti-CCP Reumatoid artritis

– IgM-RF Reumatoid artritis

– GBA Goodpasteurs disease

– SLC-70 Sclerodermi

(35)

(36)

Bronchoscopy

– BAL: Cell count and distribution of the

lavage can give an idea of which disease is

involved, activity and prognosis

• Biopsy: which disease?

(37)

And then!

Think!

Dignosis !

Treatment!’

Remenber ONLY

For lungspecialist!!!!

(38)

(39)

usual interstitial pneumonia (UIP), fibrotic nonspecific interstitial pneumonia (NSIP), and desquamative interstitial pneumonia (DIP)/respiratory bronchiolitis-associated interstitial lung disease (RBILD)/cellular NSIP

Survival is

dependent on

the diagnosis

(40)

Restrictive diseases Intrinsic lung diseases

– Interstitial lung diseases

» Arthritis related (SLE, RA, scleroderma) » “Ideopatic” (ex UIP)

- “smoke related” (ex Histeocytosis X) – Asbestosis/silicosis

– Allergic (allergic alveolitis)

– Pleura (debris-exsudat)

– Medicine (nitrofurantoin, amiodarone, bleomycin).

– Pneumonia

– radiation

Extrinsic diseases (extra-parenchymale diseases) – Non-neuromuskular

• Deformities

• Heart disease • ARDS

– Neuromuscular

• Poliomyelitis, Guillain-Barre syndrome, ALS, myasthenia gravis, muscular dystrophies

Inflammation and/or scarring of lung tissue

Fill airspaces exudat/debris (pneumonnitis)

(41)

Treatment

• Immunosupresiva

– Prednisolon • One time • Continuos – Others

• Azatioprime; metrotrexate, cyclosporine many others

• Anti-inflammatory

– acetylcysteine

• Removal of cause

– Allergic alveolitis

(42)

(43)

(44)

CM-44 0 200 400 600 800 1000 1200 1400 1600 1800 85 86 87 88 89 90 91 92 93 94 95 96 97 98 99 00 01 02 03 Year Double lung Single lung

Worldwide Lung Transplantation Numbers

Source: International Society of Heart and Lung Transplantation (ISHLT); UNOS

Lung transplants performed worldwide, by year

Emphysema/COPD

Idiopathic pulmonary fibrosis Cystic fibrosis

Alpha-1 antitrypsin deficiency Primary pulmonary hypertension Sarcoidosis Retransplant/graft failure Other 1.8% 2.6% 4.2% 39.0% 10.4% 17.0% 16.0% 9.0% Primary diagnosis, 01/1995 - 06/2003

(45)

(46)

(47)

CM-47

Comparative Transplantation

Survival Rates

0 25 50 75 100 0 1 2 3 4 5 6 7 8 9 10 Time, years S u rv iv a l, % Alpha-1 antitrypsin Cystic fibrosis Emphysema/COPD IPF PPH 0 25 50 75 100 0 1 2 3 4 5 6 7 8 9 10 Time, years Kidney—living donor Liver—cadaveric donor Heart

Primary lung transplant by underlying diagnosis

Primary kidney, liver, and heart transplant

*Kidney, liver, and heart data extrapolated from OPTN Annual Report, 2003.

(48)

CM-48

Causes of Death Following

Lung Transplantation

0% 10% 20% 30% 40% 50% 60% 70% 80% 90% 100% 0 - 30 days 31 days -1 year > 1 - 3 years > 3 - 5 years > 5 years Bronchiolitis Infection, non-CMV Graft failure Other Cardiovascular Acute rejection Malignancy Technical CMV Lymphoma

(49)

Status for

Lung transplantation



Survivel —50% died after 5 years



Bronchiolitis obliterans main reason for a bad

survivel rate



Main aim to treat and prevent bronchiolitis

obliterans

(50)