Intrinsic Restrictive Lung Disease - Pulmonary fibrosis, Sarcoidosis, Asbestosis, Pneumonia Flashcards
IPF
-presentation
45+
Persistent SOB on exertion, dry cough
Bilateral inspiratory crackles
Clubbing
Restrictive spirometry
IPF
-causes
Idiopathic
Drug induced
- chemo
- methotrexate
- amiodarone
- nitrofurantoin
Environmental
- asbestos, orgnaic dust
- hay, straw, grain
- bird poo
AI
-RA, SLE, Sjogrens, scleroderma, dermatomyositis, polymyositis
IPF
-diagnosis, investigations
Clinical diagnosis with the support of lung function tests and imaging
LFTs - spirometry, gas transfer => restrictive intrinsic lung problem
Scans
-CXR => interstitial lung markings
Key Ix - CT => reticular honeycombing, traction bronchiectasis
Procedures done if still unsure - broncheolar lavage, lung biopsy
IPF
-management (conservative, medical, surgical
MAINLY SUPPORTIVE
- Pulmonary rehabilitation
- Oxygen therapy
- Smoking cessation
Nintedanib
Pirfenidone
Lung transplantation/palliative care
Upper lung fibrosis causes
Lower lung fibrosis causes
Upper - CHARTS
- Coal worker
- Histiocytosis/hypersensitivity pneumonitis
- AS
- Radiation
- TB
- Silicosis (rocks, soil)/sarcoidosis
Lower -ACID
-Asbestosis
-Connective tissue AI minus AS
-IPF
-Drugs - amiodarone, bleomycin, methotrexate
Sarcoidosis
-epidemiology
-etiology
-pathophysiology
Women
Young adults
Mix of genetic and environmental
AI => non caseating granulomas on lungs and skin
Can be acute or chronic
Sarcoidosis
-presentation
Loefgren syndrome - acute, mild, self limiting
Bilateral hilar lymphadenopathy - SOB, dry cough, fatigue, weight loss
Erythema nodosum
Arthritis
Uveitis
Lupus pernio
Tender swollen LN
Sarcoidosis
-diagnosis, investigations
Clinical diagnosis of exclusion
- Restrictive spirometry
- High Ca, ACE, CRP
- CXR - bilar hilar lymphadenopathy
- Lung biopsy - non caseating granulomas
Sarcoidosis
-management
If asymptomatic - no treatment needed
CS
-highCa
-eye/neuro/heart involvement
-symptomatic and CXR changes
-may also use methotrexate, azathiopurine, HCQ = LFTs needed to assess impacts
If end stage lung disease - lung transplants considered
Asbestos exposure
-presentation of 4 lung problems
-management
Pleural plaques - benign (20-40 years latent)
Asbestosis - severity linked to length of exposure
- lower lung fibrosis => SOB, low exerecise tolerance
- conservatively managed
Mesothelioma - limited exposure can cause disease
- SOB, chest pain, pleural effusions
- v aggressive => palliative chemo
Lung cancer
Pneumonia
-epidemiology, spread
Young children/elderly
IC
Droplet inhalation
Haematogenous
Pneumonia
-presentation, symptoms
Dyspnoea
Purulent/clear sputum
Cough
Fever
High RR,
Hypotension, pyrexia (systemic inflammation)
Crackles, increased VR (exudate and consolidation)
Central cyanosis/confusion (hypoxemia)
Pneumonia
-diagnosis, investigations
DEFINITIVE - CXR
IDENTIFY ORGANISM
-Blood, sputum culture
-Viral PCR
-Atypical serology
-Urine AG (legionella, pneumococcal)
ABG - PO2
FBC
U&E, LFT, CRP
CURB65
-how to use
-interpretation
Confusion AMTS<8
Urea >7
RR >30
BP systolic<90 diastolic<60
65
0-1 home
2 admission considered
3-5 urgent admission, maybe ITU
Pneumonia
-management based on CURB65
-HAP
Empirical broad spec ABx in 4hrs
0-amox
-doxy, clari, erythro (pregnant)
1-2-amox+clarithromycin
3-5-coamox+clarythromycin
Coamox