Diseases of Unknown Origin/Miscellaneous Disorders Flashcards
What % of patients with Sarcoidosis have pulmonary involvement?
90%
What are the stages of sarcoidosis?
Stage 0: no radiographic findings
Stage 1: hilar adenopathy only
Stage 2: hilar adenopathy with parenchymal involvement
Stage 3: parenchymal involvement only
Stage 4: Fibrotic changes – pulmonary insufficiency
What is the distribution of sarcoidosis?
Apical
What syndrome is associated with sarcoidosis when erythema nodosum is also present and what is the triad?
Lofgren’s syndrome
Triad:
- erythema nodosum
- fever
- hilar adenopathy
Which stages have the best prognosis?
Stage 0 & 1
Sarcoidosis affects which organs/body systems?
- Pulmonary
- Cardiovascular
- Ocular
- Cutaneous
- Liver/Spleen
- Renal
- Nervous system
What are the radiographic findings associated with usual interstitial pneumonia (UIP)?
- reticular opacities
- honeycombing
- traction bronchiectasis
- irregular fibrosis
- ground-glass opacity
What is the pathology with desquamative interstitial pneumonia?
Alveoli that fill with macrophages. There is a strong association with smoking.
This pneumonia is thought to represent the end stage of respiratory bronchiolitis interstitial lung disease.
Lymphocytic interstitial pneumonia (LIP) most commonly occurs in patients with which pre-existing disease?
Autoimmune (particularly Sjogrens syndrome and AIDS)
Bronchiolitis obliterans organizing pneumonia is now referred by what name?
Cryptogenic Organizing pneumonia (COP)
What characterizes cryptogenic organizing pneumonia from other pneumonia?
Presence of granulation tissue in the alveolar ducts, alveoli AND bronchiolar lumen.
What is the preferred distribution of BOOP and what is its appearance?
Peripheral, subpleural and peribronchovascular.
Patchy consolidation or ill-defined nodules.
What is a rare, yet pathognomonic radiographic sign for BOOP?
Reverse halo sign (atoll sign) – ground glass opacity surrounded by denser ring of consolidation.
Luminal occlusion/stenosis of the bronchioles as a result of submucosal and peribronchiolar inflammation & fibrosis (WITHOUT granulation tissue or polyps/polyposis) is the pathology for which condition?
Obliterative bronchiolitis (not to be confused with BOOP which does have granulation tissue).
What is the M/C/C for cryptogenic organizing pneumonia?
Idiopathic mostly (but pulmonary infection and collagen vascular diseases are also causes).
Which syndrome is an aggressive form of idiopathic pulmonary pneumonitis and fibrosis and has a poor prognosis (death within 1 year)?
Hamman-Rich syndrome (Acute interstitial pneumonitis)
Which condition is almost exclusively found in women of child-bearing age and can present with chylous pleural effusions and pneumothorax from ruptured cysts?
Lymphangioleiomyomatosis
What is the pathogenesis of lymphangioleiomyomatosis and which other condition can the pulmonary findings mimic?
Progressive proliferation of spindle cells, resembling immature smooth muscle. Proliferation of the cells along the bronchioles causes air trapping –> cysts.
Radiographically, can present very similar to pulmonary tuberous sclerosis.
Pulmonary histiocytosis X:
a) How does it present?
b) Who does it present in?
c) Where does it present?
a) Granulomatous nodules along peribronchial tracts –> reticular/nodular/cystic pattern
b) Young or middle-aged; 90% are smokers
c) Mid to upper lobes
Which idiopathic condition presents with diffuse microliths?
What is its clinical presentation and what is the component of the microliths?
Pulmonary alveolar microlithiasis.
Usually an incidental finding (until late stage where there may be respiratory failure).
Microliths = calcium phosphate.
What is the aka for Riley-Day syndrome?
Familial dysautonomia
What is the pathogenesis of familial dysautonomia?
Where is its common distribution?
Malfunction of the autonomic nervous system resulting in hypersecretion of mucous glands - causing repeated bouts of pneumonia. Seen exclusively in Jewish descent.
M/C seen in right upper lobe.
What is the M/C complication of blunt chest trauma?
Parenchymal contusion
What is the name of the condition where the whole lung/lobe is twisted 180 degrees?
What are 3 causes for this?
Lung torsion
3 Causes:
a) Spontaneous – have other pulmonary/diaphragmatic abnormalities
b) Post-traumatic – severe compressive trauma to thoracic cage
c) After thoracic surgery
Where is the M/C location for a fracture of the trachea/bronchi?
Right side – main stem bronchi; 1-2cm distal to carina; parallel to cartilage rings
Fractures of the trachea and bronchi are usually accompanied by what other finding (other than pneumomediastinum)?
Fracture upper 3 ribs.
What is the main source of mediastinal hemorrhage?
Bleeidng from aorta
Where is the M/C location for an aortic rupture?
Aortic isthmus (90%) – immediately distal to the origin of the left subclavian artery.
What is the non-traumatic mechanism for a ruptured diaphragm?
Sudden increase in intra-thoracic or intra-abdominal pressure against a fixed diaphragm.
What side is a ruptured diaphragm M/C?
Left (will see stomach and colon in thorax)
What is the M/C/C for acute mediastinitus?
Esophageal rupture
Where is the M/C location for an esophageal rupture?
Below the cricopharyngeal muscle.
What is the pathomechanism with alpha-1-antitrypsin?
Alpha-1-antitrypsin inhibits the action of neutrophil elastase which degrades normal lung tissue. Deficiency of this will cause BASAL emphysema.
