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Granulomatous Lung Disease And Interstitial Lung Disease - Coggle Diagram
Granulomatous Lung Disease
And
Interstitial Lung Disease
Necrotising
Tuberculosis
Types
Post Primary / Reactivation TB
Types
Endogenous (Reactivation)
Exogenous (Reinfection)
Associations
Alcoholism
Diabetes
Silicosis
Immunosuppression (HIV / Anti-TNF drugs)
TNF inactivated - Bound TNF barrels means TB can reactivate
Pulmonary Aspects
Predominantly apical
Top lobes have bullet holes
Caseous / cheesy necrosis and pneumonia
Cavities - may be complicated by fungus balls, carcinoma, bronchopleural fistulas with pneumothorax
Pleural and pulmonary fibrosis and calcification
Obliterative endarteritis but also possibility of erosion into pulmonary arteries - haemoptysis - Rasmussen's aneurysm
Extrapulmonary Complications
Haematogenous / miliary spread to other organs
Amyloid
Extrapulmonary TB
Potts Disease
Tuberculous vertebral osteomyelitis
Lupus Vulgaris
Cutaneous tuberculosis
Renal Tuberculosis
Tuberculous Epididymitis
Tuberculous Meningitis
Primary TB
Primary TB occurs when a person is initially exposed to the Mycobacterium tuberculosis bacteria
Features
Ghon Focus and Ghon Complex
The initial site of infection seen as a small lesion
When combined with associated lymph node involvement it forms a Ghon complex
Gun complex -Ghon complex
Cactus seeds - hilar nodes
A pulmonary lesion, known as Ghon lesion or Ghon focus, along with ipsilateral hilar lymph node involvement
Parenchymal subplural lesion usually at the subpleural fissure between upper and lower lobes
Enlarged hilar / mediastina caseous lymph nodes draining the parenchymal focus
Pathogenesis
Inhaled tubercle bacilli ingested by alveolar macarophages
Macrophages with bacilli aggregate, froming microscopic nodules that deform architecture
Development of T-cell mediated immunity CD4 and Cd8
CD4 - Infereron - Secretory changes to macrophages resulting in appearance of epithelioid histiocytes
CD8 - Kill macrophages resulting in caseous / cheesy necrosis
Fusion of macrophages form Langerhan's type giant cells
Mantle of B lymphocytes
Prognosis
Resolution (90-95%)
Development of fibrous capsule
Calcified scar
Fibrous sac
Progressive Primary TB
Erosion into bronchus - dissemination withing bronchial tree (galloping consumption
Pleural spread - TB empyema
Compression by caseous nodes of bronchus or trachea causing lobar collapse
Haematogenous dissemitaion - Miliary TB
Millet = Miliary
Seeds seep into bloodstream causing bacteraemia and spread
Potentially fatal
Extrapulmonary TB
Epidemiology
Fall in incidence and mortality in developed countries
Elderly
Recrudescence of quiecent infection aquired in youth
AIDs pts
Urban poor
Immunosuppressed
Smokers
HIV Co-Infections
6 mil pts with dual infection
Majority in Sub-Saharan Africa
Particularly aggressive TB with widespread dissemination and poor host response
HIV infx promotes infx with opportunistic mycobacteria
Multidrug resistant strains of TB have emerged
Mycobacteria
Types
Mycobacterium bovis
Drinking milk from infected cows -
MOTT (Mycobacteria other than tuberculosis)
Mycobacterium tuberculosis
Droplet infection
Microbiology
Aerobic
Difficult to stain
waxy cell wall
Scanty in tissue
Slow growth in culture - PCR helpful
No toxins or histolytic enzymes
Escape macrophage killing
Macrophage cage
Inhibit phagosome-lysosome fusion
Stirrups - Sulfatides
Prevent the fusion of lysosomes and phagosomes (clouds)
Induce delayed hypersensitivity
Prognosis
Skin Tests and Vaccinations
Vulnerable Groups
Elderly
Disseminated miliary TB (non-reactive TB)
May be little granulomatous response, necrosis, DAD, PUO
Immunocompromised
Poorly formed granulomas
Opportunistic MAI from environment can cause spindle cell pseudotumours that mimic sarcoma
Spindle cells are histiocytes
Other Causes
Fungi
Aspergillus
Histoplasma
Mucor
Coccidiodes
Cryptococcus
Bastomyces
PCP
Worms
Non-Necrotising
Sarcoidosis :soccer:
Multisystem
Disease of unknown cause characterised by non-necrotising granulomas in many tissues and organs
Non caseastng granulomas
Collection of macrophages without an area of central necrosis
Effected organs
Frequent
Lungs
Lymph nodes
Spleen
Liver
Bone
Marrow
Skin
Eye
Salivary glands
Less frequent
Heart
Kidneys
CNS
Endocrine glands - pituitary
Epidemiology
Higher latitudes
Scandinavia
Northern Europe
North America
Black population
Young black female
Female >male
Rare in American indians, Inuits
Pathogenesis
Enhanced cellular hypersensitivity at involved sites but depressed elsewhere
Increased CD4 lymphocytes in lung
Helper T squires
CD4+ activated in sarcoidosis
Presentation
Mild non-specific chest complaints
Erythema nodosum 33%
Increased serum Ca2+
Raised glass of milk
Kidney stones
Sunny Vit D lights
Increased serum ACE
Increased serum gammaglobulins
Antibody keys
Polycloncal hypergammaglobulinemia due to CD4+ dysregulation
Anergy to common skin antigens
No reaction to feather
The Helper T cells are in the granulomas !
Gradual onset of dypnoea on exertion
Dry cough
Constitutional symptoms
Skinny goalie with flame headband
Malaise
Fever
Anorexia and weight loss
Gravel nodules
Subcutaneous nodules - non-painful and contain abuntant granulomas
Lupus pernio
Violaceous rash on nose and cheels
Anterior uveitis
Blurry red rimmed glasses
Redness
Blurry vision
Glaucoma
Retinal and optic nerve involvement - vision loss
Retina street light with broken wires
Lacrimal and salivary gland involvement
Dry eye and mouth
Dry water bottle
Granulomatous hepatitis
Liver spot cow
Restrictive cardiomyopathy
Restrictive net
Radiographic Staging
I Hilar adenopathy alone
Best outcome
II Hilar adenopathy and parenchymal infiltrates
III Parenchymal infiltrates alone
Worst outcome
Pulmonary Sarcoid
Non-necrotising granulomas
Along lymphatics
Balls at midline
Hilar and paratracheal lymph nodes - hilar lymphadenopathy
Tight Clusters of epethelioid histocytes
Multinucleate giant cells
Multiple purple panels
Multinucleated giant cells formed by fusion of activated macrophages
Tight rim of concentric fibroblasts, scattered lymphocytes
Schaumann and Asteroid bodies
Ball with star panels
Giant cells may contain asteroid bodies (stellate inclusions)
Show-man with purple cleat drinking Milk
Clacified leather cleat
Schaumann bodies contain laminated calcium and protein
Granulomatous vasculitis
Link Title
Investigations
Bronchoalveolar lavage
Shows elevated CD4+ to Cd8+ ratio (2:1)
BAL bottle
Endobronchial transbronchial biopsies
Chest X-ray
Hilar soccar balls in lung tree
Enlarged bilateral hilar and mediastinal lymph nodes on CXR
Differential Diagnosis
Infection
Brylliosis
HP
IVDU
Tumour / Lymphoma
Hypersensitivity Penumonitis
AKA Extrinsic Allergic Alveolitis
Immunological reaction to inhaled organic antigens
Diffuse inerstitial granulomatous lung disease
Triggers
Farmer's lung
Bacterium found in moldy hay
Pigeon breeder's lung
Bird feathers or excreta
Humidifier lung / Hot tub lung
Thermophilic bacteria
Talc Granulomatosis
Interstitial and perivascular granulomatous and giant cell reaction to foreign particulate matter
Granulomatous
Specific type of inflammation involving the aggregation of epithelioid histiocytes and macrophages forming a granuloma
Types
Necrotising
Dead cells in the centre of the granuloma
Most are infectious
Non-necrotising
No dead cells inside the granuloma
Causes
Infection
Necrotising GLD - TB
Autoimmune disease
Sarcoidosis
Environmental exposures
Certain dust particles - beryllium
Presentation
Respiratory Symptoms
Cough
Shortness of breath
Chest pain
Systemic Symptoms
Depending on cause
Fever
Fatigue
Interstitial Lung Disease
Hetergenous group of non-neoplastic disorders resulting from damage to the lung parenchyma by varying patterns of inflammation anf fibrosis
Aetiology / Associations
Collagen vascular disease
Drugs and toxins
Environmental
Idiopathic
Interstitium is the primary site of injury
Also affects airspaces, airways and vessels
Diagnosis
Clinical-Radioloy-Pathology correlation
Disease of Known Aetiology
Sarcoidosis / berylliosis
Organising pneumonia
Hypersensitivity diseases related to drugs
Amyloidosis
Eosinophilic pneumonia
Smokers ILD
Diffuse alveolar damage
Pneumoconioses
Vasculitis / DAH
Contrictive bronchiolitis
Lymphangitic tumour
Not finished
