Restrictive Lung Diseases Flashcards
Pectus excavatum (funnel chest) [2]
Associations [2]
Complications [2]
Congenital abnormality of connective tissue. Depressed sternum
Associated with Ehlers-Danos syndrome, Marfan syndrome
Complications:
- Iatrogenic pneumothorax
- Haemothorax
Pectus excavatum (funnel chest)
Investigations [5]
Management [2]
Ix:
- CXR
- Clinical examination (Marfan features)
- Spirometry
- Cardiopulmonary function testing
- CT/MRI thorax
Mx:
- Conservative if mild
- If functional disability then to Ratvitch operation or Nuss technique
Pectus carinatum (pigeon chest)
Association [3]
Clinical features [3]
Symptoms [2]
Associations:
- VSD
- Marfan syndrome
- FHx
- Keel chest with depressed lower and middle sternum
- Prominent upper sternum
- Large forces on inspiration cause in-drawing of lower ribs giving a Harrison’s sulcus
- Usually asymptomatic
- but restrictive defect can cause reduced VC > SOB
Pectus carinatum (pigeon chest)
Investigations [4]
Management [4]
Investigation:
- CXR
- Spirometry
- ECG, Echo
- Scoliosis
Management:
- Conservative if mild
- Casting, bracing, chest compressors
- Endoscopic resection of costal cartilage and sternal osteotomy (cosmetic)
- Open surgical repair for significant pulmonary dysfunction
Restrictive lung diseases that will be covered:
Sarcoidosis
Interstitial lung disease
- Extrinsic allergic alveolitis
- Idiopathic pulmonary fibrosis
Other restrictive lung diseases (in other decks):
- Occupational lung disease
- Pleural disease
Sarcoidosis
Epidemiology [4]
Definition
Micropathology [2]
Epidemiology:
- 20-40 yo, F>M
- Afro-Caribbean, temperate climates
Definition: idiopathic multi-system granulomatous disorder
Micropathology:
- CD4+ T cell alveolitis >
- Non-caseating granulomas with multi-nucleated giant cells
- An association has been noted between sarcoidosis and Class 2 MHC region chromosome 6p and 3p.
Sarcoidosis presentation:
- Asymptomatic
- Acute sarcoidosis [2]
- Pulmonary disease [3]
Acute sarcoidosis: erythema nodosum +/- polyarthalgia
Pulmonary disease:
- dry cough
- progressive dyspnoea, reduced exercise tolerance
- chest pain (all associated with progressive lung function deterioration)
Non-pulmonary manifestations of sarcoidosis:
- Metabolic [4]
- Inflammatory [4]
- ‘Organomegaly’ [4]
- Neuro [3]
- Cardio [2]
- Special syndromes [3]
Hypercalcemia, hypercalciuria
Renal stones
Terminal phalangeal bone cysts
Constitutional upset
Erythema nodosum, Subcutaneous nodules
Uveitis, conjunctivitis, kerato-conjunctivitis sicca
Lymphadenopathy
Hepatomegaly, Splenomegaly
Lacrimal and parotid gland enlargement
Bell’s palsy, Neuropathy
Meningitis, brainstem and spinal syndromes
Space occupying lesions, pituitary dysfx
Cardiomyopathy, arrhythmias
Lupus pernio
Heerfordt’s syndrome
Lofgrens syndrome
Lupus pernio [3]
Raised purple plaque, indurated
Affecting nose tip and around nostril
Not itchy or painful
Heerfordt’s syndrome [4]
Maxillary swelling
Parotid swelling
Facial palsy
Uveitis
Sarcoidosis Investigation
Bloods [4]
PFTs [3]
Bloods:
- FBC (reduced WCC)
- U&E (hypercalcemia)
- ACE (elevated) although limited role in diagnosis
- Ig (elevated)
Pulmonary function tests
- Restrictive pattern
- Reduced lung volumes
- Impaired gas transfer
Sarcoidosis Investigation
- CXR [3]
- CXR findings can be divided into 4 stages:
CXR
- Bilateral hilar lymphadenopathy
- Pulmonary infiltrates
- +/- fibrosis
o Stage 0: normal
o Stage 1: BHL
o Stage 2: BHL + peripheral pulmonary infiltrates
o Stage 3: peripheral pulmonary infiltrates alone
o Stage 4: progressive pulmonary fibrosis, bulla formation (honeycombing), pleural involvement
Sarcoidosis Investigation
Diagnostic tissue biopsy [5]
Other investigations [7]
Diagnostic tissue biopsy showing non-caseating granuloma in:
- lymph node biospy
- transbronchial biopsy
- EBUS (mediastinal lymph node samplying)
24h urine (Ca up), red cell casts
ECG (arrhythmias)
USS (nephrocalcinosis, HSM)
Bone XR (punched out lesions of terminal phalanges)
CT/MRI (neurosarcoidosis)
HRCT - shows nodularity in peri-lymphatic distribution.
PET scan (active areas of inflammation)
Sarcoidosis mx Active [2] BHL [1] Chronic [2] Refractory disease [3]
- Active sarcoidosis: bed rest, NSAIDs
- BHL alone: no mx, often resolves spontaneously, asymptomatic = no treatment required.
Chronic mx:
- Corticosteroids: oral PREDNISOLONE 40mg for 4-8w
- then reduce dose over 1y according to clinical status
Refractory disease:
- IV METHYLPREDNISOLONE, immunosuppressants (e.g. METHOTREXATE)
- anti-TNF alpha therapy
- lung transplantation
Sarcoidosis indications for CCS [5]
Prognosis
- parenchymal lung disease (symptomatic or progressive ie worsening changes on CXR). Increasing symptoms and deteriorating lung function
Extrapulmonary manifestations are absolute indictions for CCS.
Pro: 60% w/ thoracic sarcoidosis recover within 2yrs, 20% respond to steroid therapy, little improvement in others
Causes of BHL [6]
Sarcoidosis
Infection: TB, mycoplasma
Malignancy: lymphoma, carcinoma, mediastinal tumours
Organic dust disease: silicosis, beryliosis
EAA
Histiocytosis X
Extrinsic allergic alveolitis
Aetiology [4] (state the allergen)
Pathophysiology [5]
- Bird-fancier’s/pigeon fancier’s lung: bird-dropping protein
- Farmer’s/mushroom worker’s lung: micropolyspora faeni, theroactinomuces vulgaris
- Malt worker’s lung: aspergillus clavatus
- Sugar-worker’s lung (bagasossis): thermoactinomyces sacchari
Pathophys:
- TIII or TIV Hypersensitivity
- Acute phase of lymphocytic alveoli’s
- Alveoli are infiltrated with inflammatory cells
- Chronic exposure > non-caseating granuloma formation
- Bronchiolitis obliterans
EAA presentation
- 4-6h post exposure [4]
- Chronic [5]
- 4-6h post-exposure
- Fever, rigors
- Myalgia
- Dry cough, SOB
- Crackles but no wheeze - Chronic
- Increasing SOB
- Weight loss
- Exertional SOB
- T1 RF
- Cor pulmonale
Investigations for:
Acute presentation
Chronic presentation
Acute:
- Bloods: FBC, ESR, ABG, serum precipitins
- CXR
- PFT (reversible restrictive)
Chronic:
- Bloods
- CXR
- PFT
- BAL (increased lymphocytosis, mast cells)
What does positive precipitin test indicate?
What result would you expect of a chronic patient?
Indicates exposure only
Chronic patient would have a positive precipitin test
What can be seen on CXR of acute [2] vs chronic [2]
EAA
Acute:
- Upper zone mottling or consolidation
- Hilar lymphadenopathy (rare)
Chronic:
- Upper zone fibrosis
- Honeycomb appearance
CXR fibrotic shadowing causes
Upper zone [7]
Lower zone [4]
Upper zone:
- TB
- EAA
- AS
- RT silicosis
- CWP
- Sarcoidosis
- Histoplasmosis
Lower zone:
- Idiopathic pulmonary fibrosis
- Amiodarone
- Asbestosis
- RA
Management EAA
Acute [4]
Chronic [3]
Acute:
- Remove allergen
- Give 25-60% O2
- Then give oral prednisolone 40mg
- Gradually reduce
Chronic
- Avoid allergen exposure eg wear face-mask
- Long term prednisolone
- Compensation from employers
Spirometry of restrictive lung disease [5]
Low FEV1 Low FVC Ratio the same Reduced gas transfer as diffusion abnormality V/Q imbalance
Idiopathic pulmonary fibrosis
Symptoms [4]
Signs [3]
Symptoms:
- Dry cough
- Exertional dyspnoea
- Malaise, weight loss
- Arthralgia
Signs:
- Cyanosis
- Finger clubbing
- Fine ended crackles
IPF
Investigations: bloods [5], PFTs
Bloods:
- ABG (type 1 or type 2 RF)
- CRP (up)
- Ig (up)
- ANA (30% positive)
- RF (10% positive)
PFT: restrictive pattern, reduced gas transfer
IPF
Radiological imaging
- bilateral interstitial shadowing (typically small, irregular, peripheral opacities - ‘ground-glass’
- later progressing to ‘honeycombing’
- high-resolution CT scanning is the investigation of choice and required to make a diagnosis of IPF
sub-pleural reticular opacities that increase from the apex > lung base
IPF Mx [6]
Prognosis?
Pulmonary rehabilitation
Anti-fibrotic Rx
Smoking cessation
Best supportive care
PPI
High dose steroids are not recommended
Lung transplantation
Prognosis is poor, average life expectancy 3-4y from diagnosis.
IPF
Anti fibrotic Rx [2]
Pirfenidone
Nintedanib
How does sarcoidosis cause hypercalcemia
macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
Interstitial lung disease
definition, categorisation
Interstitial lung disease (ILD) describes any process that results in diffuse inflammatory change or fibrosis within the interstitium (). It can be classified by the aetiology
What is Lofgren’s syndrome
Lofgren’s syndrome is an acute form of the disease characterised by
* bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia.
ILD Aetiology
- Inorganic dusts
- Organic materials
- Drug induced
- Connective tissue disease
- Sarcoidosis
ILD Aetiology
Distinguish between inorganic dusts and organic materials causing aetiology
Inorganic dusts
* Coal, silica, beryllium, asbestos cause disease by inhalation > pneumoconiosis
Organic materials cause Th 4 hypersensitivity pneumonitis > granuloma formulation > inhalation of organic antigen in previously sensitised individual.
* Antigens - thermophilic actinomycetes (farmer’s lung), Aspergillus clavatus (malt worker’s lung) and bird proteins (bird fancier’s lung).
What malignancies can cause ILD?
What is association between ILD and lung cancer?
- Rarely malignancies can cause ILD, e.g. lymphangitic carcinomatosis.
- Patients with ILD have an increased risk of lung cancer.
Drug induced ILD
- Amiodarone
- Chemotherapy agents - bleomycin, methotrexate
- Sulfasalazine
- Nitrofurantoin
- Radiation
ARDS causes
Pulmonary [4]
Extra-pulmonary [6]
Pulmonary
- pneumonia
- gastric aspiration
- direct lung injury
- vasculitis
Extra-pulmonary causes:
- Shock, hemorrhage, trauma, head injury
- Sepsis, DIC
- Multiple transfusions
- Pancreatitis, liver failure
- Malaria
- Obstetric events
ARDS Pathophysiology [3]
- Direct injury or severe systemic illness causes diffuse alveolar damage
- Inflammatory mediators increase capillary permeability
- Protein rich pulmonary edema
ARDS presentation [5]
Cyanosis Tachypnoea Tachycardia Peripheral vasodilation Lateral fine inspiratory crackles
ARDS investigation
Bloods [8]
CXR [1]
- Bloods: FBC, U&E, LFT, amylase, clotting profiles, CRP, blood cultures, ABG
- CXR: bilateral infiltrates
ARDS another investigation to order
What does it measure?
Pulmonary artery catheter
Pulmonary capillary wedge pressure (PCWP)
ARDS diagnostic criteria [5]
- All the symptoms of acute onset
- CXR showing bilateral infiltrates
- PCWP <19mmHg
- Lack of signs of CHF
- Refractory hypoxemia with Pa02:FiO2 <200
ARDS Mx modalities [5]
Prognosis
ITU admission Respiratory support Circulatory support Treat underlying problem Nutritional support
Prog: 50-75% mortality
ARDS
Respiratory support
Cardiac [4]
Respiratory: Mechanical ventilation CPAP with 30-60% O2
Cardiac:
- Arterial line to monitor PCWP and cardiac output
- Conservative fluid management
- Maintain CO with inotropes, vasodilators and transfusion
- Manage pulmonary HTN with NO
