L8- Pulmonary Pathology IV Flashcards
(46 cards)
NSIP:
(1) etiology
(2) prognosis in comparison to IPF/UIP
(3) age group affected
(4) Tx
(nonspecific insterstitial pneumonia) 1- unknown 2- better prognosis 3- younger people 4- steroids or immunomodulatory therapy
Describe the histology of NSIP
(nonspecific insterstitial pneumonia)
-uniform fibrosing process (chicken-wire): i) cellular variant (lymphocyte infiltration), ii) fibrosing variant, iii) both
-lacks honeycomb change, fibroblast foci (in comparison to IPF/UIP)
Cryptogenic Organizing Pneumonia:
(1) alternate names
(2) etiology
(3) Sxs
(4) Radiographic findings
(5) Tx
1- BOOP (bronchiolitis obliterans organizing pneumonia)
2- unknown
3- cough, dyspnea
4- patchy peribronchial or subpleural consolidation
5- spontaneous recovery or with 6 mos steroids
Describe the histology Cryptogenic Organizing Pneumonia
-Masson Bodies: polyploid plugs of loose fibroblastic tissue filling alveolar spaces
- normal underlying parenchyma / alveolar architecture not destroyed
- no interstitial fibrosis or honey combing
(1) cause of Organizing Pneumonia
(2) cause of Cyptogenic Organizing Pneumonia
1- viral/bacterial pneumonia, inhaled chemicals/toxins, transplantation (lung, bone marrow), collagen vascular disease
2- unknown (or obscure)
Describe the histology of Organizing Pneumonia
(not cryptogenic)
- interstitial fibrosis
- honeycombing
AIP = (1)
- (2) affected age group
- (aggressive/benign) disease
- follows (4) event or is apart of (5) event
- (6) prognosis
(acute interstitial pneumonia)
1- Hamman-Rich Syndrome
2- older Pts, ~59 y/o
3- very aggressive
4- 2 wks post-URI
5- acute phase of IPF (interstitial pulmonary fibrosis)
6- significant mortality rate - survivors with recurrences and chronic conditions
AIP presents with (1) and (2) histologically, very similar to people who have (3)
(acute interstitial pneumonia)
1- diffuse alveolar damage
2- hyaline membranes
3- ARDS / DAD
LIP = (1)
- (2) interstitial changes
- (3) associated conditions
- (4) etiology / pathogenesis
- (5) possible complication
1- lymphoid interstial pneumonia
2- interstitial expansion by groups/sheets of lymphoid cells
3- CT disease, autoimmune disease, HIV infection
4- idiopathic (possibly)
5- progression to lymphoma in small number of cases
Majority of interstitial lung diseases are related to (1), three examples include: (2)
1- smoking
2:
-DIP, desquamative interstitial pneumonia
-respiratory bronchiolitis associated interstitial lung disease
-Pulmonary Langerhans cell histiocytosis
DIP = (1)
- (2) affected group, age/sex
- characterized by the presence of (3) in airspaces, seen in (4) patients
- may also present with (5)
1- desquamative interstitial pneumonia 2- 50-60s, M=F 3- macrophages (filled alveolar space) 4- current/former smokers 5- emphysema
DIP = (1)
- (2) clinical presentation
- (3) pulmonary function test
- (4) Tx
1- desquamative interstitial pneumonia
2- insidious onset of dry cough, dyspnea, clubbing of fingers
3- mild restrictive pattern (reduced volumes, inc/normal FEV1:FVC), moderately dec diffusion capacity
4- steroids, smoking cessaion
DIP = (1)
- (2) histological results, hint- 3
- (3) possible progression
1- desquamative interstitial pneumonia
2- alveolar macrophages (filled alveolar spaces), septal thickening, mild interstitial fibrosis
3- interstitial fibrosis (severe)
Respiratory bronchiolitis associated interstitial lung disease:
- (1) are the 2 hallmark features
- may also be present with (2)
- (3) affected age group / smoker status
1- chronic inflammation, peribronchiolar fibrosis
2- centrilobar emphysema
3- 50-60 y/o /// >30 pack years
Respiratory bronchiolitis associated insterstitial lung disease:
- (1) hallmark histological feature
- (2) clinical presentation
- (3) Tx
1- intraluminal pigmented macrophage (intracellular carbon) accumulation in 1st/2nd order respiratory bronchioles
2- mild disease, gradual onset of dyspnea, cough
3- steroids, smoking cessation
PAP = (1)
-(2) is the defect => (3) dysfunction and issue
Causes: (4), (5), (6)
1- pulmonary alveolar proteinosis (rare disease)
2- GM-CSF defect
3- pulmonary macrophage dysfunction => surfactant accumulation in alveolar/bronchiolar spaces
4- Autoimmune (90% cases): adults
5- Secondary: many disorders impairing macrophage maturation / activity
6- Congenital/hereditary: rare, GM-CSF related mutations
PAP = (1)
- (2) clinical presentation
- (3) Tx (hint- 2)
1- pulmonary alveolar proteinosis (rare disease)
2- very productive (gelatinous) cough, progressive dyspnea / cyanosis / respiratory failure [or a benign course with resolution]
3- pulmonary lavage (therapeutic) + GM-CSF replacement therapy
Sarcoidosis:
- (1) affected organs
- (2) is the hallmark feature seen in affected organs
- (3) etiology / pathogenesis
- highest prevalence in (4) patients
1- multiple organs / tissues
2- non-caseating / non-necrotizing granulomatous inflammation
3- unknown- evidence it involves Immune Response via Th cells to specific Ag (possible genetic pre-disposition)
4- young adults (<40 y/o) and smokers
Sarcoidosis results from a (1) hypersensitivity reaction to (2), driven by (3) cells
1- type IV hypersensitivity reaction
2- unknown Ag
3- Th cells (CD4+)
Sarcoidosis clinical presentation:
- (1) is an important feature related to its presentation
- (2) lung Sxs
- (3) constitutional Sxs
- (4) other organs involved –> (5) organs may have severe impairment
1- variable presentation, asymptomatic to serious presentation
2- (90% cases involve lungs) gradual respiratory Sx development: dry cough, SOB/dyspnea, vague discomfort)
3- fever, weight loss, fatigue, night sweats
4- skin (erythema nodosum), eyes (lacrimal glands - sicca syndrome), parotid glands (bilateral parotitis), spleen, liver, bone marrow
5- CNS, cardiac system, ocular system, skin
Sarcoidosis:
1) lab results
(2) CXR results (note hallmark feature
1- hypercalcemia + its resulting Sxs; elevated ACE (60% cases)
2- bilateral hilar lymadenopathy, +/- parenchymal infiltrates or fibrosis
Sarcoidosis Histology:
- (1) hallmark feature
- (2) progressive feature
- (3) rare feature
- (4)/(5) are the special histological and other hallmark features
1- non-necrotizing epithelioid granuloma w/ lymphangitic distribution
2- hyalinized scar (via collagen replacing granuloma)
3- (5-15%) progression to interstitial fibrosis and honeycombing
4- Schaumann bodies (Ca-protein concretions)
5- Astroid bodies (giant cell stellate inclusions)
define:
(1) Schaumann bodies
(2) Asteroid bodies
(for Sarcoidosis Histo)
1- laminated concretions of Ca and protein
2- stellate inclusions in giant cells
Sarcoidosis Tx
- 65-70% result in (1)
- 20% result in (2)
- 5-15% result in (3)
-(4) can occur post-lung transplantation
(Note- remission is spontaneous or with therapy)
1- recovery
2- permanent lung dysfunction
3- progressive fibrosis and cor pulmonale
4- sarcoidosis can reocur in 35% of Pts
