Chronic ILDs (interstitial lung disease) Flashcards
what are some ILD examples?
Granulomatous
Inhalational
Idiopathic pneumonias
Connective tissue
Drug induced
Other
ILD:
Granulomatous =
Inhalational =
Idiopathic pneumonias =
Connective tissue =
Drug induced =
Other =
Granulomatous = Sarcoidosis
Inhalational = Hypersensitivity pneumonitis, pneumoconiosis (asbestos/silicosis)
Idiopathic pneumonias = Pulmonary fibrosis (MC ILD) + Non pul fibrosis
Connective tissue = scleroderma, RA
Drug induced = Biomycin, amiodarone
Other = good pastures, vasculitis
Idiopathic Pulmonary Fibrosis
is it MC?
Seen in which Px?
Cause?
Commonest ILD
Seen in older men 60+, who SMOKE
idiopathic (unknown cause)
Idiopathic Pulmonary Fibrosis
RF?
Smoking
occupational (eg. dust)
Drugs (methotrexate)
Viruses (EBV, CMV)
Idiopathic Pulmonary Fibrosis
pathology?
Pul fibrosis (progressive scarring) –> type 1 resp failure
Idiopathic Pulmonary Fibrosis
Sx?
exertion dyspnoea
dry unproductive cough
Idiopathic Pulmonary Fibrosis
Dx?
Spirometry = Restriction; FEV1:FVC >0.7 BUT LOW FVC (<0.8 normal)
GS = High res CT (chest) = GROUND GLASS LUNGS + traction bronchiectasis
Tx for Idiopathic Pulmonary Fibrosis
Smoking cessation + vaccines
Pirfenidone, nintendinir
Surgery (lung transplant)
Pneumoconiosis
How is it acquired?
2 types?
Occupationally acquired ILD
Silicosis
Asbestosis
Pneumoconiosis
How is silicosis caused?
Inhalation of silicon dioxide, egg shall calcification at hilar lymph nodes
Pneumoconiosis
How is asbestosis caused?
Inhalation of asbestos, affects pleura, MESOTHELIOMA = T1 RESP FAILURE
Sarcoidosis
what is it?
most common in what type of population?
Idiopathic granulomatous disease
women, 20-40, afrocarribean
Sarcoidosis
Sx?
Fever, fatigue
Resp = dry cough, dyspnoea
Other = eye lesions (uveitis), lupus pemio (blue-red nodules on nose/cheeks)
Sarcoidosis
Dx?
Chest x ray (staging 1-4)
shows BILATERAL HILAR ADENOPATHY + pulmonary infiltrates
Diagnostic = Biopsy showing non caveating granuloma
Also high serum Ca2+ and ACE (seen in granulomas)
Sarcoidosis
Tx?
Early stages = self resolving but Symptomatic Px = corticosteroids
(Sx 2+3, any stage 4)
What is Lofgren’s syndrome?
Triad of?
Subset of sarcoidosis
Triad of:
Bilateral hilar lymph node infiltration
Erythema Nuclosum
Acute polyarthritis (MC = ankles)
Hypersensitivity Pneumonitis
What is it?
T3 hypersensitivity; immune (Ab-Ag) complex deposition at lung tissues + causes immune hyper response
Hypersensitivity Pneumonitis
RF?
Types?
Tx?
Occupation (eg. farming), bird keeping
Farmers lung (MC) - mouldy hay
Pigeon fancier’s lung (a non protein bird dropping)
Malt workers lung
Cheeseworkers lung
Humidifier fever
Tx = remove allergy
Goodpastures
What is it?
Pathology?
T2 autoimmune hypersensitivity response
AutoAb, Anti GBM attack LUNGS + KIDNEYS causing lung fibrosis and glomerulonephritis
Goodpastures
Dx?
Tx?
Lung + kidney biopsy = damage and Ig deposition
Serology = anti GBM +ve
Tx = Supportive, corticosteroids, plasma exchange to get rid of Anti-GBM
Wegener’s granulomatosis
What is it?
What is it associated with?
Granulomatosis with polyangitis
Granulomatous vasculitis affecting small + medium vessels
Typically causes ENT, lung, kidney Sx (ELK)
C-ANCA associated vasculitis
Wegener’s granulomatosis
Sx? ELK
ENT = Saddle shaped nose, ear infection
Lungs = diffuse alveolar haemorrhage therefore haemophysis
Kidney = GlomerulonephrItis therefore haematuria
Wegener’s granulomatosis
Dx?
Tx?
cANCA +VE
(MPA + EGPA = mostly pANCA +ve)
Tx = corticosteroids, immunosuppression (eg. nituximab)
