Restrictive Lung Disease - Management Flashcards
What are the signs and symptoms of IPF?
Signs - failed Rx for LVF or infection, finger clubbing, crackles, traction bronchiectasis (bronchioles pulled open by adjacent fibrosis).
Symptoms - chronic SOB and cough.
What are typical presentations of IPF?
60-70yrs old.
M>F.
A fibroblastic focus - produces collagen.
What is the treatment of IPF?
MS = 3 years (tends to worsen progressively).
Refer to the ILD clinic.
Oral Anti-Fibrotics (OAFs - pirfenidone, nintedanib, palliative care).
Surgery - transplant.
What is sarcoidosis?
A multi-system granulomatous disease of unknown cause.
Non-caseating granuloma - histological hallmark.
What are the symptoms of sarcoidosis?
Erythema nodosum.
Can be vision-threatening.
Skin lesions with granulomas (usually in areas of skin trauma - scars, tattoos).
What is lucas lupus perino?
A rarer manifestation of chronic sarcoidosis.
Causes a disfiguring nasal lesion.
Common in Puerto Rico.
Treatment - traditional steroid injections.
What is the diagnosis of sarcoidosis?
History and examination.
CXR - allows for staging.
Stage 1 - hilum lymphadenopathy and paratracheal enlargement.
Stage 2 - spotty lungs, lung involvements together with enlarged glands.
Stage 3 - interstitium change.
Stage 4 - fibrotic stage (very advanced and irreversible pulmonary fibrosis).
What are the investigations of sarcoidosis?
PFTs.
Blood tests.
Urinalysis.
ECG.
TB skin test.
Eye examination.
Bronchoscopy (including transbronchial biopsies and EBUS).
CT (less common).
What are the surgical biopsies of sarcoidosis?
Mediastinoscopy.
VATS.
What are the remission rates of sarcoidosis?
Stage 1 - 55-90% remission.
Stage 2 - 40-70% remission.
Stage 3 - 10-20% remission.
Stage 4 - 0% remission.
Sarcoidosis tends to be more a nuisance than life-threatening.
What is the treatment for sarcoidosis?
No Rx - mild disease, normal lung function, few symptoms, no vital organ involvement.
NSAIDs - erythema nodosum, arthralgia.
Topical steroids - skin lesions, cough.
Systemic steroids - cardiac, neurological, or eye disease not responding to topical steroids.
What is the prognosis of sarcoidosis?
10-20% of patients sustain permanent (extra-)pulmonary complications.
Examples - progressive respiratory failure, bronchiectasis, aspergilloma, haemoptysis, pneumothorax.
What causes HP?
Caused by birds, barley, whiskey, or even exposures at home.
What are interstitial lung diseases?
200+ diseases that cause thickening of the interstitium and can result in pulmonary fibrosis.
What is the prevalence of sarcoidosis?
World-wide - <40yrs old, M<F.
White European - skin/eye/LNs.
Japanese - cardiac sarcoidosis.
African-American - progressive.
