Restrictive lung disease Flashcards
What is the pathology behind restrictive lung disease
Pathological material enters
Acute inflammation = diffuse alveolar damage (DAD)
Leads to massive pulmonary oedema
Cells are damaged
Vessels become more leaky
Macrophages and proteins form hyaline membranes
Interstitial inflammation and fibrosis = rapid
Can be fatal
What can DAD also be
ARDS
What are causes - ask in HX
Occupational exposure Drugs - amiadarone Hypersensitivity to allergens - EAA Infection - TB / fungi / pneumonia GORD associated Systemic causes
What are systemic causes - ask in Hx
Sarcoidosis SLE Systemic sclerosis RA UC Vasculitis Autoimmune thyroid
What if unknown cause
Known as idiopathic pulmonary fibrosis
What are symptoms of restrictive lung disease and what does it lead too
SOB Dry cough Crackles - end of inspiration Abnormal breath sounds Shallow breath as reduced chest size Abnormal CXR / CT Clubbing Type 1 resp failure Pulmonary hypertension -> Cor pulmonale HF
What are RF for developing
Major trauma Chemical injury Circulatory shock Drugs Infection Autoimmune Radiation
What is investigation of choice for DADs
CXR - show shaggy heart / diaphragm (diff oedema)
Always
HRCT
What does histology show
Protein rich oedema Hyaline membrane Loss of fibrin Epithelial fibroblast proliferation Scarring Fibrosis + remodelling of interstitium Chronic inflammation
Is there reduced lung compliance
Yes as stiff
What does spirometry show
Low FEV! Low FVC Ratio the same Reduced gas transfer as diffusion abnormality V/Q imbalance
What causes chronic
Sarcoidosis
UIP - usual interstitial pneumonia
Granulomatous
Hypersensitivity pneumonitis
What is sarcoidosis
Multisystem granulomatous disorder
Granuloma = inflammatory cluster filled with mostly macrophages
Non-ceasaing = no necrosis and is buzzword for sarcoidosis
What is histology in sarcoidosis
Epithelioid and giant cell granuloma's Filled with MO Little lymphoid infiltrate Variable fibrosis Alveoli surrounding granuloma = normal
What causes sarcoidosis
Unknown cause
Immune reaction to unknown antigen?
Can affect any organ
What does acute sarcoid present like
Bilateral hilar lymphadenopathy - seen on CXR or CT
Swinging fever
Erythema nodosum
Polyarthralgia
When is bilateral hilariously lymphadenopathy also common
TB Malignancy - lymphoma Fungi HIV Mycoplasma Hypersensitivty pneumonitis Histiocytosis
What are insidious symptoms
SOB Dry cough Crackles Chest pain Malaise Weight loss Pink lesions containing granuloma - over scar tissue Clubbing Mediastinal LN
What are skin symptoms
Lupus pernio
Symptoms of non-pulmonary disease
Systemic - fever, fatigue, weight loss Hypercalcaemia - macrophages in granuloma increase conversion from vitae's D Lymphopenia LN Liver cirrhosis / cholestasis HSM Uveitis Conjunctivitis Optic neuritis Bells Palsy Neuropathy Cardiomyopathy Heart block Renal
Who is affected by sarcoid
Young females
How do you Dx
What is diagnostic
Blood - Elevated serum Ig / ACE / Ca / LFT / ESR
CXR
Spirometry = restrictive
Transbronchial biopsy / bronchoscopy = diagnostic
Mediastinoscopy + biopsy of hilar LN
Other organ involvement U+E / urine dip LFT ECHO - if cardiac ECG- BBB/. arrhythmia PET CT to see what lights up TB test USS abdo Examine eye for occular
What does CXR show
Trachea Nodes Chunky light hilar Pulmonary infitrates Fibrosis
What does biopsy show
Non-caesating granuloma
How do you treat mild disease, normal PFT, no important organ
No treatment
What do you do if erythema nodosum / arthralgia
NSAID
What do you do if skin lesions / uveitis / cough
Topical Steroid
Cardia / neuro / hypercalcaemia / symptomatic stage 2/3 disease
Systemic steroid
Biphosphonates for hypercalcaemia
2nd line = immunosuppression
What is stage 1 on CXR
Bilateral hilar lymphadenopathy
Stage 2
+ pulmonary infiltrate
Stage 3
Pulmonary infiltrates ONLY
Stage 4
Fibrosis Causes pulmonary hypertension Loss of lung function Impairment to lung function / gas exchange Benefit from lung transplant
What are complications of sarcoidosis
Respiratory failure Bronchiectasis Aspergilloma Haemoptysis Pneumothorax
DDX of sarcoidosis
Infection
Autoimmune
Vasculitis
EAA
What is hypersensitivity pneumonitis also known as
Extrinsic allergic alveolitis
What is hypersensitivity pneumonitis
Group of diseases caused by inhalation of allergen
Immune mediated
What type of hypersensitivity and histology
Type 3 = immune complex Granuloma's - centracinar (type 4) Foamy histiocytes Intersitital pneumoinitis Bronchiolitis obliterans can occur
What happens in acute
Alveoli infiltrated by inflammatory cells
If remove allergen process can be halted
What happens in chronic
Granulomas + lymphocytes = chronic inflammation
Fibrosis
Bronchiectasis
What are acute symptoms 4-8 hours after exposure
Recurrent SOB Dry cough Pyrexia Myalgia Bibasal crackles Wheeze Tachypnoea Flu symptoms Reduction in lung volume and diffusion
What are chronic symptoms
Fibrosis Increasing SOB Weight loss Cough Crackles Wheeze Malaise / lethargy Clubbing Type 1 resp failure Cor pulmonale
How do you investigate
CXR
BAL
Biopsy
FBC - increased WCC, neutrophil, ESR
What does CXR show
Fibrosis of upper and middle lobe
Diffuse shadowing
What does biopsy show
Fibroses
Alveolitis
Granuloma
What does BAL show
Lymphocytosis and mast cells
How do you treat
Remove allergen
Steroids in severe
Oxygen may be used in acute attacks
Often misDx as
Pneumonia
What is Ddx of fibrosis
Infection
Lymphoma
Carcinoma
Haemorrhage
What is idiopathic pulmonary fibrosis
Most common cause of fibrosis in the lung
Pathology of IPF
Patchy chronic inflammation Intra-alveolar fibrosis Obliteration of bronchioles Smooth muscle and vascular proliferation Turns into collagen
What causes IPF
Connective tissue - ANCA / RF +Ve
Drugs
Asbestos
After viral infection
Symptoms of IPF
Progressive exertional dyspnoea
Dry cough
Basal fine crackles at end of inspiration
Clubbing
Malaise Fever Weight loss Arthralgia Elevated ESR Cyanosis Failed Rx of LVF / pneumonia
How do you investigate
ABG - decreased O2 Bloods - increased CRP, Ig, ANCS, RF CT = diagnostic CXR Spirometry - restrictive BAL PFT - reduced gas transfer
What does CXR show
Fleeting shadows Small irregular opacities Peripheral opacities -> honeycomb late Diaphragm and fuzzy heart border Smaller lung volume Often misdiagnosed as pneumonia
What does BAL show
Lymphocytosis
Neutrophils
Eosinophilia suggest worse prognsosi
How do you treat
Often poor response to Rx Pulmonary rehab Oral anti fibrotic Supplementary O2 Opiates for SOB Palliative Surgical transplant
What are the complications
Lung cancer Fibrosis Honeycomb End stage lung disease Cor pulmonale and res failure = death
What are most common restrictive lung disease
Idiopathic
Drugs
LL tend to be affected
What affects upper lobe
CHARTS Coal work - hypersnesitivity Histiocytosis / hypersnesitivity / EAA AS Radiation TB SArcoidosis
What affects LL
Idiopathic Connective tissue Drugs Asbestos a!A!
What is the advanced stage of fibrosis
Honeycomb lung
What drugs cause fibrosis
Amiadarone
Cyclophosphomdie
Methotrexate
Nitrafuratoin
What is hypoxia at rest an indication for
Oxygen therapy
What can be given to slow down progression
Anti-fibrinolytic
To get drug what is required
Non-smoker
FVC <80%
What lifestyle
Smoking cessation
Pulmonary rehab
What do yo follow up patients with
CXR
Spirometry and PFT
What is important in history
Childhood - TB / pneumonia Occupational exposure Dust / asbestos Immune - scleroderma / RA / sarcoid Smoking Drugs Pets Feather duvet FH
When should you think if ILD
Desaturations
Chronic SOB / chronic cough
What is general management of ILD
Remove cause Home O2 if hypoxic at rest Stop smoking Physio and pulmonary rehab Pneumococcal and flu Lung transplant is an option ACP and palliation
What type of crackles
Fine end inspiratory
In pneumonia
Coarse inspiratory
