Diffuse lung disease/Pulm HTN Flashcards
Imaging findings of 3 distinct phases.
- acute
- subacute (classic sign)
- Chronic (classic sign)
Hypersensitivity pneumonitis - reaction due to inhaled organic antigens like bird proteins, thermophilic actinomycetes.
- Acute (rarely seen) - inflammatory exudate filling alvuli - nonspecific GGO
- subacute; centrilobular ground glass; mosaic attenuation. Head cheese sign; patchy GGO and areas of lucency due to mosaic attenuation
- upper lobe predominant pulm fibrosis. (head cheese sign)
Classic imaging findings
- Demographics
- Histologic appearance
- Prognosis and response to steroids
- Associated with what disease?
- 2 types
NSIP - Ground glass, subpleural sparing. Effects posterior and peripheral lobes, like UIP
- Younger patients (40s-50s) compared to IPF
- Thickened alveolar septa from chronic inflammation (less fibrotic change compared to IPF)
- Better prognosis than IPF, responds to steroids
- Scleroderma (Can show dilated esophagus to suggest scleroderma)
- Fibrotic NSIP. Cellular NSIP
- Classic imaging
- Pathologic corresponding name
IPF - Most common ILD, bad prognosis
- Honeycombing, traction bronchiectasis, basilar predominant (Apical to basal gradient)
- Usual interstitial Pneumonia
- Definition of Pulmonary ARTERIAL hypertension
- Definition of pulmonary VENOUS hypertension
- >25mmHG Pulm arterial systolic pressure
- >18mm Pulm cap wedge pressure (an approximagion of pulm venous pressure)
How are Precapillary and postcapillary causes of pulm hypertension described?
- Precapillary: primary abnormality is pulm arterial system or pulm parenchyma leading to alveolar hypoxia
- Postcapillary causes: abnormality of pulmonary veins or elevation of pulm venous pressure
WHO 5 groups of pulm HTN etiologies
- Pulm Arterial HTN - PPH (primary pulm HTN), congenital left to right shunts, Pulmonary veno-occlusive disease and pulm capillary hemangiomatosis
- Pulm venous HTN - Left sided heart disease
- Pum hpertension a/w chronic hypoxemia - COPD, Interstitial lung disease, sleep apnea,
- CTEPH
- Pulm HTN due to misc disorders
Pulm HTN findings
Describe hilum convergence sign
Main PA diameter > 3 cm
Pulm artery calcifications (pathognomonic)
CTEPH - mosaic attenuation
Ground glass centro-lobular nodules (especially in pulm veno-occlusive disease)
Hilum Convergence sign: hilar pulmnoary artery branches converging into an enlarged PA
Describe the 3 WHO group 1 Pulm HTN etiologies
Primary Pulmonary Hypertension (PPH), precapillary: Enlargement of main PA w/ tapering of vessels
Cardiac shunts, Precapillary: (ASD, VSD, PAPVR)
Pulm veno-cclusive disease, precapillary: fibrotic obliteration of pulm veins/venules. Imaging features include pulm arterial enlargement, pulm edema, and ground glass/centrilobular nodules.
- What is this WHO5 pulm hypertension disease?
- Most common causes
- Who5: fibrosing mediastinitis - progressive proliferation of fibrous tissue w/in the mediastium. May lead to encasement and compression of mediastinal structure
- . Histoplasmosis, tb
3 plain film signs of PE
- Fleischner sign - widening of PA due to clot (picture)
- Hampton’s Hump - peripheral wedge shaped opacity - infarct
- Westmark sign: focal peripheral hyperlucency secondary to oligaemia in lung distal to PA thrombus
- CT findings
- Treatment
Cryptogenic organizing pneumonia
granulation tissue polyps that fill the distal airways and alveoli
- mixed consolidatino and GGO in peripheral and peribornochovascular distribution. Atoll sign (reverse halo)
- Tx. Steroids
Respiratory bronchiolitis - Interstitial lung disease (RB-ILD)
Smoking related disease
- Describe RB and RB-ILD
- CT appearance
- Histologic appearance
- RB: pigmented macrophages are found in respiratory bronchioles (very common in smokers)
RB-ILD is when people have RB + symptoms
- Centrilobular nodules and patchy GGO.
- Histologically - sheets of macrophages filling the terminal airways.
Desquamative interstitial pneumonia (DIP)
RB-ILD and DIP are a spectrum (both smoking related)
- Describe DIP
- Imaging features
- DIP: The macrophages have now made it to the alveoli
- Imaging: diffuse-basal predominant, patchy or subpleural GGO. Cysts may also be present.
What is this?
- Commonly a/w what?
- imaging
- histologic landmark?
Lymphoid interstitial pneumonia - rare
- Sjorden
- Diffuse lower lobe predominant GGO. Scattered cysts (due to air trapping). Pneumothorax may occur in advanced disease.
- diffuse infiltration of interstitium by lymphocytes
Acute interstitial pneumonia (AIP) - AKA DAD
Acute onset, terrible prognosis.
- What is primary cause
- What are 2 phases?
- Primary cause is surfactant destruction
- Early exudative phase: hyaline membranes, diffuse alveolar infiltration by immunce cells, noncardiogenic edema
Chronic phase: alveolar wall thickening (1 week after inhalational injury).
Name the interstital lung diseases. Which is the worst? which are smoking related?
IPF: (2nd worst. UIP is pathologic name)
NSIP (smoking)
COP
RB-ILD, DIP (both smoking)
LIP
AIP (acute, worst prognosis)
Hypersensitivity pneumonitis - Organic antigens
- acute phase
- Subacute phase? Classic imaging sign?
- Chronic
Acute: inflammatory exudate filling the alveoli - GGO and centrilobular nodules
subacute: centrilobular ground glass nodules, mosaid attenuation. Head cheese sign
Chronic HSP: upper lobe predominant pulm fibrosis. Superimposed mosaic attenuation, centrilobular nodules, ggo
Pneumonoconiosis - INORGANIC dust inhalation
- name 2 most common pneumoconioses
- Imaging (classic calcs?)
- Pneumoconiosis = increased risk of what?
- Caplan syndrome
- Inhaled disease that effects LOWER Lobes
- Silicosis, Coal workers pneumoconiosis (CWP)
- Uncomplicated disease: multiple upper lobe centrilobular and subpleural nodules
eggshel lymph node calcs (silicosis)
complicated: Progressive massive fibrosis
3. TB
4. RA + CWP or silicosis.
5. Asbestosis (particles are too large to be removed by macrophages/lymphatics)
Eosinophilic lung disease
- Simple pulmonary eosinophilia - AKA what?
Imaging
- Chronic eosinophilic pneumonia - imaging
- treatment?
- Loffler sndrome
Migratory areas of focal consolidation
- extensive alveolar filling and interstitial infiltration w/ inflammatory eosinophils.
Pathcy peripheral consolidation w/ upper lobe preference
- Steroids
Pulmonary vasculitis (name 3)
Churg-Strauss, microscopic polyangiitis, Wegner
Churg-Strauss - small vessel vasculitis
- Imaging
- Which titer is positive?
- periphearl consolidation or ground glass
- P-Anca
microscopic polyangiitis
- Describe clinical findings
- Imaging
- Most common cause of pulm hemorrhage w/ renal failure
- Diffuse central predominant ground glass
Wegner’s granulomatosis - Systemic small vessel vasculitis
- Classic clinical triad
- Titer?
- 3 places it effects
- Sinusitis, lung involvement, renal insufficiency
- C-Anca is positive
- Upper airways - nasopharyngeal/eustachian tube obstruction. involvement of larynx/bronchi
Lungs; Multiple cavitary nodules - don’t respond to abx
Kidneys
Lung Drug toxicity. What reaction patterns can occur?
Pulm Edema, ARDS, organizing pneumonia, eosinophillic pneumonia, bronchiolitis obliterans, pulm hemorrhage, NSIP, UIP.
Radiation lung injury
- Early radiation pneumonitis
- Radiation fibrosis
- Occurs w/in 1 month - most severe at 3-4 months after treatment. Ground glass cenetered at radiation port
- 6-12 months after therapy.
Key finding is distribution of fibrosis and traction bronchiectasis.
Sarcoidosis
- Stages of fibrosis (0-4)
- Most common CT finding of sarcoidosis
- Bronchial involvement may cause what lung pattern?
- Stage 0: normal
Stage 1: Hilar or mediastinal adenopathy only
Stage 2: Adenopathy WITH lung changes
Stage 3: Diffuse lung disease WITHOUT adenopathy
Stage 4: End stage fibrosis
- Perilymphatic nodules of variable sizes.
- Mosaid attenuation due to air trapping.
Pulmonary langerhans cell histiocytosis - smoking related lung disease
- Disease is most often isolated to lungs, but where else can it effect?
- Progressgion of disease
- radiographic findings
- treatment
- Lucent bone lesions, diabetes insipidus, hypophysitis, skin involvement.
- Nodules –> Cavitary nodules –> irregular cysts. It can sometimes present as pneumothorax
- upper lobe predominant cysts, and irregular peribronchovascular
- Responds to steroids and quitting smoking
Ddx for disease affecting lungs, and bones?
PLCH, malignancy, tb, fungal disease, sarcoidosis, gaucher disease
Pulmonary alveolar Proteinosis - idiopathic disease causing filling of the alveoli w/ proteinaceous lipid rich material
- Radiographic findings
- CT hallmark
- Susceptible to superimposed infection, particularly whar?
- Treatment?
- May have perihilar opacification like pulm edema, but heart size is normal and there are no pleural effusions.
- Crazy paving.
- Nocardia (presents as consolidation)
- Bronchioalveolar lavage.
Lymphangioleiomatosis - thin regular shaped cysts involveing all 5 lobes
- Describe pathogenesis
- Epidemiology
- Treatment?
- Associated with what complications
- Bronchiolar obstruction and lung destruction due to proliferation of muscle cells in small vessels, lymphatics, and bronchioles
- Women w/ child bearing age. 1% of patiets w/ tuberous sclerosis have LAM.
- Some cases respond to anti-estrogen therapy
- Chylothorax, pneumothorax
