Interstital Lung DiseaseNicholas Ashley SHO

Definition – Interstital Fibrosis• Chronic inflammatory condition of the lung parenchyma that

has multiple aetiologies but ultimately results in fibrosis of the alveoli and interstitium through fibroblast activation

• Doesn’t affect the airways, affects the part involved in gaseous exchange

Aims• Learn causes of pulmonary fibrosis• Main investigations in pulmonary fibrosis• Treatment strategies

Causes

Idiopathic

Organic(EAA)

Inorganic(Pneumoconiosis)

CTD

Drugs

Sarcoidosis

Idiopathic“Idiopathic Interstitial

Pneumonitis”

Drugs Organic

Idiopathic Pulmonary Fibrosis

Methotrexate Pigeon Fancier

Desquamative Interstitial Pneumonia

Nitrofurantoin Farmers

Acute Interstitial pneumonitis

Amiodarone Hot Tub or Malt Workers

Inorganic CTD Sarcoidosis

Coal Minors Rheumatoid Arthritis OWN CATEGORY

Asbestosis Systemic Sclerosis

Silicosis Vasculitidies

Clinical Features•HISTORY

Onset?Timeframe? Progressive?Order of Sx?

PMHxSmokingPetsExposure in Job/HobbyAsk specific meds!If confident ask CTD Sx

Cough (No Sputum)SOB (Progressive)

Ex ToleranceWeight LossFatigue

After HPCx:

Differentials to Consider

CCF

BRONCHIECTASIS

COPD

• Pink frothy sputum• Pillows at night• PMHx HTN/Valve/IHD• PND and Orthopnoea• Ankle swelling• Palpitations

• Thin copious sputum• ?Severe lung insult in PMHx

• Routine questioning

LUNG CANCER • Weight loss• Smoking/exposure

• EXAMINATION

Bibasal CracklesClubbing

Cor PulmonaleRespiratory Effort Reduced ExpansionCyanosis

Investigations

BLOODS

FBC, U&E, LFT, Ca, ACE

RF, antiCCP, ANA

T-Spot

ABG

IMAGING

CXR

Echo

HRCT

SPECIAL

Spirometry

BAL

Biopsy

MICRO

Sputum MCS

TB Culture

Treating Idiopathic Pulmonary Fibrosis

Difference of Opinion

• Corticosteroids• Azathioprine• N-Acetyl Cysteine

Important

SurgeryLTOT

PalliatePulm

Rehab

MDTSmoking Cessation

If the cause isnt idiopathic then treatment varies – as a rule of thumb it often involves starting steroids and/or removing causative agent!

Prognosis

• IDIOPATHIC - Generally poor as there is not much

that can be done to slow progression

• OTHERS - Very variable. Can often slow disease

progression but cannot reverse fibrotic

changes already present

Case Vignette• 70 year old gentleman presents to you with SOB gradually

worsening over the last 8 months• His exercise tolerance has decreased from being able to walk 3

miles to now managing 200 yards before stopping due to SOB• He has no PMHx of note and has smoked 20/day for 40 years• He appears SOB on examination with clubbed fingers and a

saturation of 91% OA. Examination reveals fine fixed crackles and no wheeze

Vignette Questions• What is your differential list?• Any specific symptoms or signs to rule in fibrotic lung disease /

rule out differentials?• How would you fully investigate this patient – justify your use

of each Ix• What would the management plan entail and what would this

gentleman’s prognosis be?