ILD (Raf) Flashcards
(161 cards)
1
Q
16 year old girl with SOB, inspiratory stridor, hoarsness, nasal congestion. Multiple ED visits. Labelled as asthma and VCD. (case was eventually diagnosed by findings on bronchoscopy)
A
GPA
(important to include GPA on differential for stridor). Pulmonary involvement is present in 80% of cases. May or may not have more obvious symptoms like pulmonary renal syndrome.
2
Q
Antibody involved with GPA?
A
C-anca (PR3). is the primary antibody.
Some patients are MPO (p-anca)
3
Q
Antibody invovled with microscopic polyangitis?
A
MPO (p-anca)
4
Q
Pulmonary manifestations of GPA?
A
Upper airway: tracheal stenosis, subglottic stenosis, epistaxis, nasal septal ulceration, sinusitis, oral ulcers
Nodules (which can be caseating), look like nodules on chest imaging
Diffuse alveolar hemorrhage
5
Q
Down syndrome patient with wheeze. What is your differential diagnosis?
A
Atopy is NOT more common in patients with Down’s compared to general population. In fact, DS patients have LOWER rates of atopy.
You should think of other things first:
- Airway malacia - could be intrathoracic or upper airway. Hypotonia could make this worse
- Vascular malformation (I don’t think they necessarily have a predisposition to vascular malformation, but need to think about broader differential for wheeze)
6
Q
Why are LRTIs a big deal in patients with down syndrome?
A
- Can be predisposed to LRTIs because of: upper airway abnormalities, GERD (which affect airway clearance and soil the airway) + immune system abnormalities (problems with innate and adaptive immunity - lower function of neutrophil, lower antibody response to vaccines, including respiratory pathogens)
- LRTIs can be more severe since they low respiratory reserve (lower surface of lung) b/c of acinar hypoplasia, alveolar hypoplasia
- Higher chance of hospitalization, ICU, longer length of stay
7
Q
2 year old DS patient with pleural effusion. Differential?
A
DS patients can be prone to chylous effusion inherently b/c of DS, though that’s more common in neonatal period. In an older DS child, would be improtant to think about other causes:
- infectious
- malignancy (since they are also prone to malignancy)
- pericardial effusion can be associated with hypothyroidism
For a DS neonate with chylothorax, you would definitely do full work up, but interestingly, congenital pulmonary lymphangiectasia is are in DS. (That being said, I think we had a patient with this)
8
Q
Why are DS patients prone to pulmonary hypertension?
A
Intrinsic abnormalities of vascular bed: persistence of fetal double capillary layer, thickened pulmonary arterioles, reduced size of vascular bed (along with alveolar hypoplasia) + exacerbating factors such as OSA (also infection, aspiration)
9
Q
Lower respiratory abnormalities in DS patient?
A
- Acinar and alveolar hypoplasia
- Subpleural cyst
- Increased prevalence of CHILD
- chylothorax
- increased frequency and severity of LRTI
- pulmonary hypertension
10
Q
Upper respiratory abnormalities in DS patient?
A
- Narrowing of whole airway, both above and below the cords
- Above vocal cords:
* Macroglossia - I think this is relative to skeletal size * Midface hypoplasia * Narrow nasopharynx * choanal stenosis * Enlarged tonsils and adenoids - relative skeletal * Short palate
* Below the vocal cords: * Trachea is narrower by 2 mm compared to non-DS—>high incidence of post intubation stridor * Tracheomalacia:
* Tracheobronchomalacia is common * Laryngomalacia is common * Tracheal bronchus is slightly more common * Subglottic stenosis is more common—commonly related to previous intubation, though not always * Tracheal stenosis:
* Associated with vascular rings and hypoplasia of aortic arch * Tracheal versus sublottic stenosis: just differ by location of stenosis. Sublottic is right below the cords. Tracheal is a bit further down. * Symptoms of upper respiratory:
* Snoring—>important to specifically ask about this, since parents may not volunteer this info * Noisy breathing * Cough
11
Q
For NEHI, what is the appearance on biopsy and staining pattern?
A
bombesin positive neuroendocrine cells are seen in the distal airways (respiratory bronchiole) and they can also be in clusters in alveolar duct
12
Q
Imaging findings in NEHI?
A
- ground glass opacities centrally, in RML and lingula
- Inspiratory/expiratory CT: air-trapping
- CT is very specific (about 100%), but imperfect sensitivity at 80%
- classic CT findings are enough to make diagnosis and don’t need to go on to biopsy
13
Q
what is a hydrocarbon?
A
What are hydrocarbons?
* Petroleum solvents * Household cleaning products * Kerosene * Liquid polishes and waxes * Furniture polish * Typical age group for accidental ingestion: * Toddlers * Age <5 years * Often these products are stored in containers that look like beverage containers
14
Q
How do hydrocarbons cause lung damage?
A
- Lung injury is through DIRECT aspiration
- Hypoxia is the MAIN issue, as opposed to hypercapnia. This is due to:
- Surfactant inactivation—>atelectasis and V/Q mismatch
- Bronchospasm
- Hydrocarbon vapours will displace alveolar gas
15
Q
What type of lung damage do hydrocarbons cause?
A
- Autopsy samples in humans have shown:
- Necrosis of bronchi, bronchioles and alveolar tissue
- Hemorrhagic pulmonary edema
- Interstitial inflammation
- Thromboses
16
Q
What is a non-infectious cause of pneumatoceles?
A
Hydrocarbon aspiration
17
Q
Long term complication of hydrocarbon aspiration?
A
Peripheral small airway disease–>obstruction with low FEV1, air trapping so high RV/TLC
No increased bronchial reactivity
18
Q
What features of hydrocarbons enable them to cause lung damage?
A
Hydrocarbons are vicious b/c:
- Low surface tension—>spread through tracheobronchial tree
- Low viscosity—>can go deep into lung
- High volatility—>high level of blood stream absorption, which causes CNS effects
19
Q
Which surfactant disorders can have a delayed presentation?
A
- ABCA3
- Surfactant protein C
20
Q
Lamellar body in SP-B?
A
Disorganized with large whirls and vaculoar inclusions.
Looks like a hole with inclusions, as opposed to a tree trunk
21
Q
Lamellar body in ABCA3?
A
-small and dense
- eccentrically placed inclusions
Fried egg (yolk and surrounding white of the egg)
22
Q
Treatment for ABCA3 and SP-C?
A
Streoids, hydroxychloroquine, azithromyci
23
Q
Which diseases cause non-caseating granulomas?
A
- Hypersensitivity pneumonitis
- Sarcoid
- Kendig’s also mentions: CGD, fungal, ulcerative colitis, GPA, berylliosis–>so I think some of these causes can give both caseating and non-caseating
24
Q
Which disease cause caseating granulomas?
A
- TB
- Granuomatosis with polyangitis
- IBD (necrobiotic nodules)
25
Pathology findings in HP?
- Alveolitis
- Giant cells
- Foamy alveolar macrophages
- Non-caseating granulomas
26
4 airway manifestations of SLE
- pleuritis with pleural effusion**
- pulmonary embolus**
- acute pneumonitis**
- infection such as pseudomonas, PJP, CMV-->very important to rule out infection for pulmonary presentations of lupus **
- shrinking lung syndrome
- chronic ILD
- pulmonary hemorrhage - possible, but less common
(when practically seeing a lupus patient, also need to think about: drug toxicity, cardiac disease and renal disease which may be affecting pulmonary status)
27
Is routine CT for asymptomatic patients with lupus recommended?
No
28
Risk factors for acute chest syndrome
- Age, in particular 2-4 years of age, though pediatrics>adults
- Pulmonary infection, such as atypicals like mycoplasma
- lower HbF
- HbSS has more ACS than HbSC
- Asian haplotype
- Fat embolism
- Post op atelectasis
- Bronchospasm
- Higher WBC count
29
What is the biggest risk factor for sickle cell chronic lung disease?
- ACS
30
What is the relationship between asthma and sickle cell disease complications?
- Asthma and wheezing are independently associated with SCD complications
- ONLY wheezing is associated with ACS
31
What is ACS?
```
It's not a disease, but it's a syndrome of respiratory symptoms (fever, cough, dyspnea, hemoptysis) + new radiographic infiltrate in a patient with sickle cell.
Variety of causes:
- infectious - eg. mycoplasma
- atelectasis - eg. post op
- fat embolism
```
32
What type of lung disease is sickle cell chronic lung disease?
- fibrotic (interstitial fibrosis), difuse
| - restrictive lung disease
33
Why are sickle cell disease at risk for OSA?
* functional asplenia—>compensatory T+A hypertrophy and extra medullary hematopoiesis
34
Sickle cell disease patient with obstruction, but no BD response. Etiology?
Consider causes for fixed airway obstruction, but can also have obstruction in sickle cell due to increased pulmonary capillary blood volume.
35
In sickle cell patients with suspected hypoxemia, how should this be confirmed?
Poor correlation between SpO2 and arterial blood gas, so should get PaO2 (blood gas)
36
What are signs of severe acute chest syndrome?
- Multilobar disease
- increased hypoxia
- increased RR
- low platelets
37
Which drug is strongly not recommended for PH management in sickle cell disease patient?
- phosphodiesterase inhibitors
38
Sickle cell patient with wheezing. Approach to investigation?
- PFT with pre/post bronchodilator testing
- Airway hyper-reactivity challenge tests, such as with exercise or cold air
- Methacholine challenge is NOT advised since this can cause ACS
- Key point: wheezing in SCD patient should NOT be labelled as asthma right away since there "other causes" (Kendig's doesn't go into details, but presumably the SCD itself with changes in pulmonary blood volume). That being said, may have lower threshold for treating for possible asthma since wheezing is a risk factor for ACS and both wheezing and asthma are independently risk factors for VOC
39
Sickle cell patient with dyspnea during exercise. DDx?
- Pulmonary hypertension
- Sickle cell chronic lung disease
- (Sleep disordered breathing is a co-morbidity that may be a role, but wouldn't be top on the differential)
- Obstructive disease--asthma or wheezing (which seems to be intrinsically related to SCD)
40
Why is oximetry not reliable for SCD?
* increased carboxyhemoglobin in patients with sickle cell disease since faster Hb turnover. Most older oximeters in the hospital can’t differentiate between carboxy and oxygenated hemoglobin
* HbS absorbs light at a different frequency than HbA
41
Appearance of lamellar body in SP-B and ABCA3?
SP-B: abnormally formed lamellar body with large whorls and vacuolar inclusions
ABCA3: small and dense lamellar body, with eccentrically placed inclusions-->appearance of fried egg
42
BAL findings in HP?
Lymphocytosis
Decreased CD4/CD8 ratio (since there is predominance of CD8 cells)
increased NK cells
43
Immune pathogenesis of HP?
Sensitization to inhaled antigen-->alveolitis, with accumulation of neutrophils in the alveoli in first 48 hours-->then, increased lymphocytes and macrophages with prodominance of CD8 cells
- Both type 3 and type 4 cell mediated immune responses are involved, but there is no immune complex deposition in the lung
44
Patient with lupus presents with pleural effusion. Differential?
Infection is high on the differential! Patients with lupus are at high risk for pulmonary infection, even in the absence of drug side effects
- Inflammatory: pleuritis/Serositis
- Transudative due to renal failure or cardiac involvement (renal involvement would be more common)
45
Lupus patient who presents with fever, cough, chest pain, new infiltrate on CXR. DDx?
- Infection!!- they are at risk for opportunisitc infections like aspergillus, PJP, pseudomonas , CMV
- In general for acute pulmonary symptoms in lupus patient, think about PE, though that wouldn't fit with fever
Other less common:
- DAH, which is more likely if associated nephritis, drop in Hb or hemoptysis
- Acute pneumonitis (but this would pretty much be a diagnosis of exclusion)
46
Lupus patient with chronic dyspnea with exercise, chronic cough. DDx?
- chronic ILD
| - pulmonary hypertension
47
Lupus patient with PFT showing restriction, DDx?
- chronic ILD
- Shrinking lung syndrome - I think normal DLCO corrected for VA, since there's no parenchymal disease
- Pleural effusion
- ?DAH, but may expect increased DLCO if acute hemorrhage
48
What stain is used to identify hemosiderin laden macrophages?
Prussian blue stain, which will stain for iron in macrophages
49
What % of hemosiderin laden macrophages corresponds to diagnosis of DAH?
>20% of 200 macrophages. There's normal to have up to 30% of hemosiderin laden macrophages.
50
Describe the approach to hemoptysis
See evernote on Chapter 61
51
Describe the approach to diffuse alveolar hemorrhage
Evernote on chapter 61
52
What is the work up for hemoptysis?
| What is the work up for DAH?
- CT chest with contrast (+/- sinus CT if suspecting GPA)
- Bronchoscopy - confirm pulmonary hemorrhage and get samples for infection
- Coagulation - INR, PTT, vWF, fibrinogen
- Infectious - sputum and blood culture, TB skin test
- echo to look for PH and cardiac disease
- CRP, ESR
- DAH: echo, ANA, ANCA, anti-GBM, antiphospholipid antibody, lupus anticoagulant, ESR/CRP, D dimer, anti-dsDNA
- CBC, iron studies
- Kidney: U/A, urine protein:creatinine ratio
Based on Dr. Dell:
- immune work up, including immunoglobulins
53
Causes of hypoxemia in a patient with sickle cell disease?
Hypoventilation:
- OSA (functional asplenia causes adenotonsillar hypertrophy and extra-medullary hematopoeisis)
- Depression of respiratory rate from use of narcotic in context of pain crisis
- Diffusion: sickle cell chronic lung disease, pulmonary hypertension
- VQ mismatch:
- Pain leading to splinting and atelectasis
- Obstructive lung disease (even in the absence of asthma)
- pulmonary thrombosis
54
Older patient (>2 years of age) with ILD symptoms (non-productive cough, dyspnea, poor activity tolerance)
Differential:
- Vascular: diffuse alveolar hemorrhage, cardiac: pulmonary hypertension, PVOD
- Infection - if immunocompromised-->chronic infection
- Bronchiolitis obliterans - related to post-infectious, rejection or GVHD
- aspiration
- ongoing disorder of infancy - NEHI, surfactant deficiency
- Inflammatory:
- systemic inflammatory disease - eg. lupus, scleroderma, sarcoid
- toxic exposures: pneumotoxic drugs, radiation, inhalational exposures
- autoimmune pulmonary alveolar proteinosis
- other: eosinophilic pneumonia, hypersensitivity pneumonitis, organizing pneumonia
55
Drugs that cause pulmonary injury? What is the timeline for lung injury?
```
Cytotoxic:
A: ATRA, ARA-C (cytosine arabinoside)
B: busulfan, bleomycin
C: cyclophosphamide, carmustine
IL-2
Methotrexate
```
Non-cytoxic:
* Antibiotics: nitrofurantoin
* IBD: mesalazine, sulfasalazine
* Seizure: keppra
* Azathioprine
*Bleomycin is the MOST important. Also remember: busulfan, cylophosphamide, carmustine
Timeline:
- typically within weeks to months of initial exposure
- with some agents such as bleomycin, carmustine (and potentially cylocphosphamide) there can be delayed toxicity:
56
Is there a pathognomonic type of lung injury with drug toxicity?
NO
- wide range of imaging findings: unilateral or bilateral reticular markings, ground glass opacities or consolidations.
- There can also be CT features of pulmonary veno-occlusive disease: enlarged central arteries, centrilobular ground glass opacities, septal thickening, and pleural effusion
- General findings on PFT: decreased DLCO is most sensitive, may see findings of restriction with low lung volumes
- wide of range of histologic findings: diffuse alveolar damage, hypersensitivity pneumonitis, eosinophilic pneumonia, alveolar hemorrhage, alveolar proteinosis
57
With alveolar proteinosis, what would you see on BAL?
- hypocellularity
- PAS positive granular material
(CHILD statement)
58
With pulmonary histiocytosis, what would you see on BAL?
- intracytoplasmic pentalaminar inclusion bodies, positive CD1a staining
59
Differential diagnosis for neutrophilia (>3%) on BAL?
- Infection --pneumonia, bronchitis, bronchiectasis
- Aspiration
- Diffuse alveolar damage/ARDS
60
Differential diagnosis for eosinophilia (>1%) on BAL?
- Drug reaction
- Parasitic infection
- Fungal infection - eg. PJP
- Asthma
- ABPA
- Churg Strauss
- Eosinophilic lung disease - eosinophilic pneumonia, usually >25%
61
Lymphocytosis (>15%) on BAL. Differential diagnosis?
- CD4+ predominant: sarcoid (often >25% lymphocytes)
- CD8+ predominant:
- Hypersensitivity pneumonitis (often >50% lymphocytes)
- Pulmonary histiocytosis
- Drug induced ILD (drug induced pneumonitis)
- Collagen vascular disease\
- Radiation induced pneumonitis
- cryptogenic organizing pneumonia
- lymphoproliferative disorder
62
BAL findings of Niemann pick disease?
- storage cells typical for niemann pick disease?
63
BAL findings for aspiration?
- Neutrophilia
| - Lipid laden macrophages
64
neonate with severe hypoxemia respiratory failure and pulmonary hypertension. Which CHILD disorders should be tested for on genetic panel?
ACD-MPV (FoxF1), Surfactant protein B, ABCA3 and maybe surfactant protein C mutation
65
Diffuse lung disease + congenital hypothroidism + neurological abnormalities. Which CHILD disorder should you test for on the genetic panel?
NKX2.1
66
Neonate with alveolar proteinosis, what disorders should you consider?
- Surfact protein B, C, ABCA3
- NKX2-1 (this is the gene that codes for TTF-1)
- methionyl transfer RNA synthetase mutation
- lysinuric protein intolerance
- CSF2RA and CSF2RB-->although these cause absence of receptor components, there is NOT neonatal onset of PAP, it's delayed by a few months
67
histological pattern of a surfactant protein disorder?
- chronic pneumonitis of infancy
- PAP
- NSIP
- DIP = diffuse interstitial pneumonitis
68
In which CHILD disorders could you see PAS positive staining?
- PAP
- PIG: widening of the interstitium with vaculoated foamy cells that contain glycogen, these cells can stain positive for PAS (I think it's the glycogen that stains positive)
69
What are the recommendations for type of lung biopsy and handling of lung biopsy in an infant with a CHILD syndrome with no diagnosis yet?
- Ideally, the biopsy should be done by VATS since can visualize more of the lung, obtain more biopsy samples,less complications
- Sample should be handled according to published protocols (would be important to speak with pathologist)
Samples to be sent for:
1. Microbiology
2. Histopathology
3. Immunohistochemistry
4. Immunofluorescence
5. Electron microscopy
Extra info:
Lung biopsy specimens should be handled as suggested by published protocols, with separate portions of the biopsy undergoing formalin fixation for histopathology and immunohistochemistry, microbiologic culture, freezing for possible immunofluorescence or other special studies, and fixation for electron microscopy.
70
What disorders can lead to a severe neonatal CHILD?
- Surfactant disorders: B, ABCA3
- TTF-1 (NKX2.1)
- ACD-MPV (Foxf1)
- acinar dysplasia
- pulmonary hypoplasia with alveolar simplification
- PIG
- pulmonary hemorrhage syndromes
- pulmonary lymphangiectasia
(surfactant protein C and ABCA3 can be rapidly or slowly progressive)
71
Which CHILD have a bad prognosis? Which have variable prognosis?
- Bad prognosis: SFTPB and ACD-MPV
- Variable: SFTPC and ABCA3
- Good prognosis: NEHI
72
Some CHILD disorders have case report evidence for hydroxychloroquine and steroid. Is this standard therapy?
- NO, this is not standard therapy, it's very low quality evidence
- make a decision on case by case basis and watch for side effects
* Need to monitor for side effects:
steroids: growth, bone mineral density, optho exam
hydroxychloroquine: CBC, optho exam
73
For the CHILD which present infancy, which are autosomal dominant?
- surfactant protein C
- NKX2.1
- especially important to have the family meet with genetic counsellor
- FOXF1 (ACD-MPV)
74
Health maintenance of an infant with CHILD?
* Respiratory support:
```
*
assess need for oxygen overnight, physical activity, during feed
*
NIV or invasive ventilation if severe respiratory impairment
*
Growth
*
Immunizations:
```
```
*
routine
*
additional pneumococcal (usually if above 2 years of age)
*
influenza
*
RSV vaccine
*
Avoid smoke exposure
*
If they are on immunosuppresion, then PJP prophylaxis
*
Genetic counselling, especially if there is a dominant mutation such as surfactant protein C or NKX2.1
```
75
What is a child syndrome ?
Child syndrome: -->this definition helps us define a phenotype for which we should the use the pediatric DLD approach
* Definition: infant <2 years with phenotype above + excluded other common causes + meeting at least 3 of 4 criteria:
```
*
respiratory symptoms: cough, rapid breathing, exercise intolerance
*
signs: resting tachypnea, adventitious sounds, retractions, clubbing, failure to thrive, respiratory failure
*
Hypoxemia
*
Diffuse abnormalities for CXR or CT
```
* Common conditions that need to be excluded:
```
*
CF
*
aspiration
*
congenital heart disease
*
immunodeficiency
*
BPD
*
pulmonary infection
*
PCD presenting with neonatal respiratory distress
```
76
Minimal obligatory investigations for CHILD syndrome?
| Describe the work up alogirthm in ATS CHILD statement
- Echo
- HRCT
- immunodeficiency work up
- Other: genetics, bronchoscopy with BAL, lung biopsy
77
Describe the classification of pediatric diffuse lung disease
- See ATS CHILD statement
78
Genetic mutation associated with ACD-MPV?
FOXF1, which is autosomal dominant
79
For lung growth abnormalities (CHILD category), what types of imaging abnormalities are seen?
- Architectrual distortion
| - cystic changes
80
Imaging findings for PIG?
- quite variable, nothing pathognomonic
- PIG is an interstitial process
- ground glass opacity
- septal thickening
- cysts
- interstitial infiltrates
81
Imaging findings for NEHI?
- ground glass opacity in RML or lingula and air trapping
82
Typical age of onset for NEHI?
- doesn't usually start at birth
| - gradual onset over the first year of life
83
Typical age of onset for PIG?
- tends to have neonatal onset, but wide range of presentation
-
* diverse
*
severe neonatal respiratory distress
*
indolent--tachypnea, hypoxia
*
may be an initial period of well being
*
starts in the first days to weeks of life
*
Can occur in term or preterm infants
(I think the significance of PIG depends if it is patchy or diffuse. PIG reflects lung immaturity, patchy PIG can be seen with many lung growth abnormalities)
84
What conditions are associated with abnormal lung growth?
Conditions limiting prenatal growth:
- oligohydramnios - PROM, bladder outlet obstruction
- Restriction of thoracic volume from space occupying lesion (eg. CDH) or thoracic deformity
- CNS, neuromuscular, other agents causing decreased fetal breathing
- BPD
- Congenital heart disease:
- disorders with poor pulmonary blood flow (eg. TOF)
- cyanotic heart disease, impairing post natal alveolarization
Genetic:
- T21: deficient post natal alveolarization
- NKX2.1
- FLNA disruption
85
Typical presentation of Churg Strauss (EGPA)?
- severe asthma
- rhinosinusitis
- eosinophilia (typically >1.5)
- migrating pulmonary infiltrates
- constitutional symptoms
86
General threshold for initiation of supplemental oxygen in a patient?
- >=5% of time <90%
*
- 3 intermittent saturations (separate values) <90%
- For BPD and pulmonary hypertension:
>=5% of time <=93% or 3 separate saturations <=93%
87
Infant with BPD on home oxygen. How much oxygen should be given during flight?
- For kids who are oxygen dependent at sea level, double the flow rate of oxygen during flight
- No need for hypoxic challenge test since you already know they need oxygen
88
Infant with BPD who is now off oxygen x 4 months. Is supplemental oxygen required during flight?
For infants and children on oxygen in the last 6 months, there should be a hypoxic challenge test completed.
(practically we may just give them oxygen)
89
Which children with CF (and other chronic lung disease) need hypoxic challenge?
FEV1<50% predicted
| If saturation <90% for CF during hypoxic challenge, then need in flight oxygen
90
Infant with chronic lung disease, not on home oxygen in the last 6 months, who is going to fly?
They do advise that these infants under 1 year of age should have hypoxic challenge test done
91
How do you interpret results of hypoxic challenge test?
<85% in infants (<1 year of age), then oxygen
<90% for older children, then oxygen
oxygen can be titrated, typical flow rate is 1-2 L
92
Recommendations for flying post pneumothorax?
- Confirmed CXR resolution + additional 7 days
- Except for traumatic pneumothorax, where 2 week delay after radiographic resolution is advised
- If there has been definitive surgical intervention via thoracotomy (I think they are referring to pleurodesis), then these patients can fly just after surgery
93
Causes of centrilobular nodules?
- things that enter lung through airway can give centrilobular nodules
- Hpersensitivity pneumonitis
- Infection - TB, mycoplasma
94
What is tree in bud appearance on CT?
- describes the CT appearance of multiple areas of centrilobular nodules with a linear branching pattern
- can be due to disease in distal airways or distal vasculature
- usually due to endobronchial spread of infection
* Invasive pulmonary aspergillosis
*
TB - endobronchial spreading
*
NTM
*
ABPA
95
What is pulmonary alveolar proteinosis? Differential diagnosis?
PAP is a syndrome that occurs in a heterogeneous group of diseases
and is defined histopathologically by the presence of a finely
granular, lipo proteinaceous material filling pulmonary alveoli and
terminal airspaces.
Congenital PAP:
◦ Onset: neonatal or early infancy
◦ Primarily interstitial changes, relatively minor alveolar proteinosis
◦ Etiologies:
◦ Lysinuric protein intolerance—this would cause a secondary PAP due to macrophage dysfunction. There are other systemic manifestations: FTT, anorexia, vomiting, diarrhea, lethargy, coma. Can present anywhere from neonatal to adulthood. There may not be any respiratory manifestations.
◦ Surfactant disorders: SFB, SFC, NKX2-1, ABCA3
◦ PAP in the context of a disorder of surfactant production: If there is abnormal surfactant, then there can be alveolar distortion and accumulation of this abnormal surfactant. But this type of PAP has a different clinical presentation than primary PAP. It doesn’t respond well to whole lung lavage.
◦ Methionyl-transfer RNA synthetase mutation
• Primary PAP: main manifestation is alveolar proteinosis. Can be hereditary or autoimmune.
◦ Hereditary: due to abnormalities in the GM-CSF receptor, specifically either CSF2RA or CSF2RB, which each code for the alpha and beta chains of the receptor
◦ Autoimmune: antibodies to GM-CSF. More common in adults, less common in children
◦ This group has similar presentation, radiology, histology (alveolar filling without marked distortion), similar response to whole lung lavage therapy
• Secondary PAP:
• Infections – eg, Nocardia asteroides, Mycobacterium tuberculosis, Mycobacterium avium-intracellulare, Pneumocystis jirovecii, HIV.
• ●Hematologic malignancies – eg, lymphoma, leukemia, and haploinsufficiency of the GATA binding protein 2 gene (GATA2), which is often associated with a myelodysplastic syndrome. Hematopoietic stem cell transplantation is considered in select cases with GATA2 mutations.
• ●Immunodeficiencies – eg, HIV infection, severe combined immunodeficiency (especially adenosine deaminase deficiency), or GATA2 mutations.
• ●Rheumatologic disorders such as systemic juvenile idiopathic arthritis
• ●Exposure to inhaled chemicals (insecticides, fumes) and minerals (silica, aluminum, and titanium)
96
CXR, BAL, CT, lung biopsy findings for PAP?
Histopath from lung biopsy: proteinaceous material and macrophages in distal airspaces , AEC 2 hyperplasia , interstitial thickening with mesenchymal cells and fibrosis depending on the underlying disease. Depending on the cause of PAP, may see other features like disorganized lamellar body or fried egg appearance of lamellar body.
○ CXR : diffuse granular alveolar and interstitial infiltrates
○ BAL:
- Visual appearance: "milky" or "wavy"
- Upon standing, it forms a sediment with "finely granular lipoproteinaceous material"
- Cell count: acellular, few cells, contains large foamy alveolar macrophages
PAS (periodic acid schiff) positive + eosin positive proteinaceous material, which fills up the distal air spaces, alveolar wall and interstitial architecture is well preserved
Electron microscopy: shows abnormalities in lamellar body
○ CT: crazy paving, ground glass inter-lobular septal thickening
97
Causes of hypersensitivity pneumonitis?
- Birds are the TOP cause due to exposure to avian proteins in excrement, feathers or bloom
- Farmer’s lung from exposure to moudly plant material (eg. Aspergillus)
- Malt worker’s lung from exposure to mouldy barley
- Hot tub lung from exposure to mycobacterium avium
- Cheese washer’s lung
- Wood insturment lung
- Important to keep house free (and instruments) free of mould b/c apart from ABPA, moulds can cause HP in an immunocompetent host
98
Pulmonary complications of IBD?
Bronchiectasis (most common), tracheal stenosis, Ileobronchial colobronchial fistula, BOOP, granulomatous and necrobiotic nodules, ILD, pulmonary vasculitis, drug induced disease(HP to sulfasalazine and mesalamine), Opportunistic infection , malignancy, pulm thromboembolism
99
Imaging findings sarcoid?
○ normal(stage 0)
○ bilateral hilar lymphadenopathy(stage 1)
○ Bilateral hilar lymphadenopathy with parenchymal infiltrates (nodules,fibrotic or alveolar) ( stage 2)
○ Parenchymal infiltrates without BHL (stage 3)
- Stage 4: pulmonary fibrosis
○ Stage 1 is the most common in children followed by stage 0 ,then stage 2 and stage 3
○ Don’t need to treat stage 0-1
100
Laboratory findings in sarcoid?
○ increased ACE (60-80% of cases)--most sensitive
○ Hypercalciuria (need to order urine calcium to creatinine ratio)
hypercalcemia
101
Pulmonary manifestations of GPA (Wegener)?
Upper airway: Sinusitis, nasal septal ulceration , subglottic stenosis, otitis media, mastoiditis
Lower airway: Diffuse alveolar hemorrhage, nodules, cavitation, which can be cavitary ,tracheobronchial stenosis, peribronchial soft tissue thickening
Nodules, Infiltrate, cavitation, mediastinal lymphadenopathy, pleural effusion, pneumo
Presentation: dyspnea, cough, hemoptysis, hoarsenss
102
Abnormalities on autopsy of T21 lungs?
- Acinar hypoplasia (decreased radial alveolar count, alveolar simplification)
- subpleural cysts
- double layered capillaries
- muscularization of arterioles
103
Treatment options for PAP?
- whole lung lavage
- anti-CD20 like rituximab
- treatment based on underlying condition like bone marrow transplant, lung transplant
104
Which surfactant disorders can anti-inflammatory agents like steroid, azithromycin, hydroxcyhloroquine be used?
- surfactant protein C
| - ABCA3
105
Pathologic finding in sarcoid? Bronchoscopy finding in sarcoid?
- Non-caseating granulomas, which are located in the perilymphatic area (peribronchovascular)
- Bronchoscopy: waxy yellow mucosal nodules (it makes sense that you would see nodules on bronchoscopy because nodules are the key interstitial finding) and nonspecific changes → erythema, edema, granularity and cobblestoning of the airway mucosa and bronchial stenosis, can also get pediatric laryngeal sarcoid
- Try and get a sample of endobronchial lesions since biopsy is required to make a diagnosis in sarcoid
- Increased CD4/CD8 ratio (3.5:1)
106
Patient with stage 1 sarcoid. Anything you need to do for them when you seem them in clinic?
- No indication for treatment from lung perspective since reasonable probability of "remission", but need to refer to opthalmology for a check up since they are at risk for uveitis and blindness
107
PFT findings in sarcoid?
- restriction + decreased DLCO
108
What is Lofgren syndrome?
subtype of sarcoid: (polyarthritis, BHL, erythema nodosum, fever)—>good prognosis, with 85% spontaneous remission in 6-12 months
109
Pathologic findings of chronic HP?
- alveolitis
- foamy alveolar macrophages
- non-caseating granulomas
- giant cells
- fibrosis
110
What are some non-infectious complications of aspergillus?
- ABPA
- asthma triggered by aspergillus
- hypersensitivity pneumonitis
111
Pulmonary manifestations and PFT findings in scleroderma?
ILD, pulmonary hypertension, aspiration (related to esophageal dysfunction) -->most important and most common
pleuritis, pleural effusions, bronchiectasis, BOOP, alveolar hemorrhage
Spontaneous pneumothorax with severe fibrosis and aspiration pneumonia associated with esophageal reflux may also be seen.
PFT: restriction (low FVC, low TLC) and low DLCO
112
14. Name three chemotherapeutics associated with lung disease.
○ What is the typical lung disease associated with each.
Most important drugs to know for lung injury:
- A: ATRA -al-trans retinoic acid
- B: Bleomycin
- Busulfan
- C: cytosine arabinoside, carmustine
- D: docetaxel
- They all cause interstitial pneumonitis and pulmonary fibrosis (this would be the same answer to go with for mechanism of injury)
- Other mechanisms of injury:
- Eosinophilic pneumonia
- Hypersensitivity pneumonitis
- COP
- ARDS, alveolar hemorrhage, diffuse alveolar damage
*This is the most up to date note for pulmonary drug toxicity
113
What is the difference between histology/histopathology and electronic microscopy?
- Histology: looking at tissues under a light microscopy
| - Electronic microscopy: is a higher level magnification, eg. if you want to see specific organelles (eg. lamellar body)
114
What is a foamy macrophage? What is a giant cell?
- Foamy macrophage --macrophage with thick cytoplasm, often due to bronchiolar obstruction. (e.g. diffuse panbronchiolitis, hypersensitivity pneumonia or cryptogenic organising pneumonia) or alternatively to exogenous lipoid pneumonia, some drug toxicity (e.g. amiodarone exposure or toxicity) and metabolic disorders (e.g. type B Niemann–Pick disease). For our conditions, it is associated with HP, PIG, PAP.
- Giant cell-- giant cell (multinucleated giant cell, multinucleate giant cell) is a mass formed by the union of several distinct cells (usually histiocytes), often forming a granuloma. Can occur with sarcoid, HP, fungal infection, TB.
115
Blood work investigations for HP?
Acute HP: leukocytosis, neutrophilia. May see high CRP, ESR.
- 50% of patients: high RF factor, high IgA/IgM/IgG
- Not much role for skin testing
- Presence of a precipitating IgG antibody
116
Treatment for HP?
Stop antigenic exposure
- Steroids such as prednisone
- Supportive such as oxygen
117
What is the difference between how surfactant protein B and C present?
- B: early onset respiratory distress in a full term neonates, respiratory distress in first few minutes to hours and progresses to respiratory failure by 3-6 months. B is bad!
- looks like RDS in a term baby, except that RDS actually gets better
- ABCA3, NKX2.1 can also present like surfactant protein B
- ABCA3 can also present like surfactant protein C
Variable age of onset, either infancy or adulthood
- Non-specific respiratory symptoms, which can be acute, subacute or chronic
- Cough, dyspnea, exercise intertolerance, tachypnea, crackles, clubbing, failure to thrive, oxygen need
- Can be triggered by viral infection
118
Lung biopsy for CHILD syndrome. What should the biopsy be sent for?
- formalin fixation for histopathology and immunohistochemistry
- microbiologic culture
- immunofluorescence
- electronic microscopy
119
Surfactant
○ 2 constituents
○ relative amount of each
- Phospholipids, 80%
- Neutral lipids, 8%
- Protein (eg. A, B, C, D), 8%
120
Pathologic findings of bronchiolitis obliterans?
- subepithelial fibrotic changes in airways
lead to concentric narrowing or complete obliteration of the airway lumen.
Less important things to remember:
The associated lesions comprise a spectrum of abnormalities that can include:
● Subtle cellular infiltrates (predominantly T lymphocytes) around the small airways
● Bronchiolar smooth muscle hypertrophy
● Bronchial filling with mucus stasis, distortion, and fibrosis
● Total obliterative bronchial scarring
121
Pathologic findings of cryptogenic organizing pneumonia?
excessive proliferation of granulation tissue, which consists of loose collagen-embedded fibroblasts and myofibroblasts, involving alveolar ducts and alveoli, with or without bronchiolar intraluminal polyps. Intraluminal plugs of granulation tissue may extend from one alveol
122
Any role for biopsy in bronchiolitis obliterans?
minimal role for biopsy since there is patchy disease, so normal biopsy would not necessarily be reassuring
123
Post HSCT patient, 100 days post transplant, with cough, wheeze, SOB. What is on your differential diagnosis?
- bronchiolitis obliterans
| - Post HSCT, PFT is done at 100 days, 1 year, 6 month intervals till 2 years
124
If you are suspecting BO, how should the CT scan be conducted?
- Inspiratory and expiratory views to look for air trapping (children less than 6 years may need sedation)
125
What are the etiologies of bronchiolitis obliterans?
- Post infectious - influenza, adeno, varicella, measles
- Post transplant
- Post HSCT (chronic GVHD)
- SJS
- Connective tissue disease - RA, Sjogren's, lupus
- Toxic gas - nitrous oxide
- Aspiration
- Drugs
- chronic hypersensitivity pneumonitis - eg. avian antigen, mould
126
BAL findings in LCH?
>5% of S100 or CD1a stain
| - absence of eosinophils
127
Findings for pulmonary hemorrhage on BAL?
Grossly blood tinged
Hemosiderin laden macrophages
Gross appearance Bloody, increasing with each sequential sample for DAH
Cytology Hemosiderin stained macrophage
128
What is the differential diagnosis of a pulmonary hemorrhage?
- Pulmonary versus non-pulmonary (GI, sinus)
- Upper airway
- Lower airway (Bronchial circulation)--infection, bronchiectasis, neoplasm, foreign, vascular to bronchial tree fistula
- Alveolar focal--eg. pneumonia
- Diffuse alveolar--immune versus non-immune
- pulmonary vascular disease-->in particular, increased pulmonary venous pressure
- bleeding disorder
- trauma
(Box 61.1 in Kendig's for more details)
129
Investigations for pulmonary hemorrhage?
* CT chest with contrast to look for PE, AVM
* Test for coagulation defects
* Bronchoscopy to look for foreign body, airway hemangioma, tumor, presence of hemosiderin laden macrophages
* Infection: blood and sputum cultures, TB testing with purified protein derivative
* If diffuse alveolar opacities—>echo to look for cardiac disease, CT with contrast, cardiac cath
* If systemic involvement (eg. renal disease, rash, joint disease)—>ANA, ANCA, CBC, ESR, CRP, urinalysis, metabolic panel, d-dimer, von willebrand factor, anti-phospholipid antibody, lupus anticoagulant
* If DAH and no cardiac, renal or systemic disease with negative antibodies (ANA, ANCA, anti-GBM)—>transthoracic biopsy either through open approach (mini-thoracotomy) or VATS
130
What is orthodeoxia and what are some causes?
- Desaturation in oxygen saturation of >2-5% when moving from supine to upright
- Causes:
- pulmonary AVM (predilection for lower lobes)
- hepatopulmonary syndrome (since there are intrapulmonary vascular dilatations which are preferentially in dependent position)
- Inter-atrial shunt like PFO
131
What is a pulmonary AVM?
Abnormal vessel, Direct connection between pulmonary artery and vein, without capillary bed in between
"aneurysmal", dilated, tortuous
Usually 1 single arterial vessel and 1 single venous vessel
132
Pathological features of desquamative interstitial pneumonitis
DIP = surfactant deficiency
DIP is a histologic pattern that has been previously utilized to describe DLD in children. In contrast with DIP in adults (which is associated with smoking), most cases of DIP in children are caused by an inborn error in surfactant metabolism, including mutations in the surfactant protein B, surfactant protein C, and ABCA3 genes. DIP is just one of many histologic expressions of these mutations
common characteristic features include interstitial widening, foamy alveolar macrophages in the airspaces, hyperplasia of the type 2 alveolar epithelial cells (AEC2), and variable amounts of granular, proteinaceous material in the distal airspaces
Lung pathology findings from patients with NKX2-1 haploinsufficiency have been infrequently reported, and included deficient alveolarization, septal fibrosis, and lung cysts, with numerous Lamellar bodies visualized by light microscopy
133
16 year old girl with arthritis and pneumonitis, diagnosed with JIA. What else should be on the differential diagnosis?
- pulmonary involvement is not very common with JIA, though pleuritis is the most commonly associated manifestation
- need to make sure she doesn't a diagnosis more commonly associated with pulmonary disease, like lupus
134
Decreased DLCO in a patient with systemic sclerosis. Etiology?
Differential:
- ILD
- pulmonary hypertension
135
Patient with rheumatologic disease and pulmonary symptoms. What is the general differential diagnosis?
- Infection (they are often on immunosuppressive medication)
- drug effect
- manifestation of underlying disease
- Cardiac
- muscle weakness
- thromboembolic
136
Pulmonary manifestations of mixed connective tissue disease?
- this is a relatively uncommon condition, but high rate of pulmonary involvement
* Typical pulmonary findings:
```
*
pulmonary fibrosis
*
pleural effusion
*
PAH - can develop rapidly
(basically a combination of Sscl and lupus)
*
Other manifestations:
```
```
*
thromboembolic disease
*
pulmonary hemorrhage
*
diaphragm dysfunction
*
aspiration
```
137
Etiologies of dyspnea in the patient with chronic liver disease?
```
Major etiologies of dyspnea in patients with chronic liver disease that should be considered during the diagnostic evaluation include hepatopulmonary syndrome, heart failure, diaphragmatic compromise from ascites (restriction), deconditioning, and other major causes of chronic dyspnea (eg, asthma, chronic obstructive pulmonary disease, interstitial lung disease), porptopulmonary hypertension
hepatic hydrothorax (passage of ascites from abdominal cavity into pleural cavity)
```
138
How do you conceptually differentiate hepatopulmonary syndrome from portopulmonary hypertension?
- both occur in patients with chronic liver disease
- HPS - intrapulmonary capillary dilatation
- hepatopulmonary syndrome--portal hypertension + PAH, with no other good reason for having PAH. This falls into group 1 PAH. In contrast to HPS, instead of vascular dilatation, there is constriction
139
Causes of pleural fluid eosinophilia?
- Infectious:
- fungal - eg. histoplasma
- parasite - eg. ascaria, strongyloides
- Inflammatory :
- acute eosinophilic pneumonia
- chronic eosinophilic pneumonia
- eosinophilic granulomatosis with polyangitis
140
Pulmonary manifestations of Hurler syndrome?
1. Obstructive sleep apnea
2. Restrictive lung disease (due to small thorax with abnormal shape)
3. Respiratory insufficiency (nocturnal hypoventilation-->daytime hypoventilation)
4. Recurrent pneumonia
141
Lung biopsy findings for surfactant protein B?
- alveolar epithealial cell hyperplasia
- mesenchymal/interstitial thickening
- finely granular lipoproteinaceous material (PAP)
- B: abnormally formed lamellar body with large whorls and vaculoar inclusions
- ABCA 3: small and dense lamellar body with eccentrically placed inclusions (fried egg appearance)
142
Timeline for presence of hemosiderin laden macrophages post alveolar hemorrhage?
• hemosider laden macrophages start to be present at day 3 post hemorrhage, peak at day 7-10 post hemorrhage and are usually dwindling by 2 months, unless ongoing bleeding
143
Diagnostic criteria for HP?
Need to have 4 major criteria:
- symptoms compatible with HP, either acute, subacute or chronic
- evidence of exposure to antigen - either IGg antibody or history of exposure
- Imaging findings consistent: reticulonodular infiltrates, ground glass, nodules, air trapping, fibrosis
- BAL: lymphocytosis, decreased CD4 to CD8 ratio
- Biopsy findings: alveolitis, noncaseating granulomas, giant cells, foamy alveolar macrophages or fibrosis
- positive natural challenge
- there needs to be 2 minor criteria:
- rales
- decreased DLCO
- hypoxemia
- recurrent episodes of symptoms
144
Treatment for bronchiolitis obliterans post infections? BO post HSCT?
- basically the same treatment for post-infectious and post HSCT BO
- anti-inflammatory: systemic corticosteroid, azithro, FAM (fluticasone, azithro, monteleukast), other: steroid sparing anti-inflammatory
- supportive care: oxygen, nutritional support, immunization, avoid cigarette smoke, airway clearance if bronchiectasis, bronchodilator if response, exercise therapy/pulmonary rehab
- FAM has been studies more for post HSCT BO, but can extrapolate to infectious
145
Causes of bronchiolitis obliterans?
- post infectious
- HSCT
- lung transplant
- SJS
- connective tissue disease
- hypersensitivity pneumonitis
146
Causes of pulmonary AVM>
- HHT
- liver cirrhosis
- post Glenn or fontan
147
findings on bubble echo for intrapulmonary versus intracardiac shunt?
bubbles from right side of heart would enter left heart within 3 – 6 cardiac cycles (< 3 cycles c/w intracardiac shunt)
148
Diagnostic criteria for hepatopulmonary syndrome?
- oxygenation defect: PaO2<80 mmHg in room air or A-a gradient >=15 mmHg on room air
- pulmonary vascular dilatation: positive finding on bubble echo
- liver disease: most commonly pulmonary hypertension, with or without cirrhosis
149
Mechanisms of hypoxemia in hepatopulmonary syndrome?
VQ mismatch
widened diffusion barrier
shunt
150
Definitive therapy for hepatopulmonary syndrome?
liver transplant
151
For extrapulmonary and extracardiac shunts (arteriovenous connections), what direction do they shunt in?
they shunt left to right (they wouldn't be a cause of hypoxemia)
152
Treatment of diffuse alveolar hemorrhage?
- treat underlying cause, if known
- pulse steroids (eg. methylpred 30 mg/kg x 3 days) then oral steroids +/- additional immunosuppresion (eg. rituximab) if the DAH is due to capillaritis (such as isolated pulmonary capillaritis or capillaritis with a systemic disorder like GPA)
- IPH is considered a bland hemorrhage so may be ok with just steroids
153
Definition of massive hemoptysis? most common cause of hemoptysis in children?
- >240 mL in 24 hours
- infectious
- otherwise healthy: TB, aspergilloma, endemic mycoses
- influenza
- staph, strep, pseudomonas
154
Hemoptysis versus hematemesis?
Hemoptysis:
- no nausea or vomiting
- lung disease
- may have asphyxia
- sputum is frothy, liquid or clotted, bright red or pink
- Labs: alkaline, mixed with macrophages and neutrophils
Hematemesis:
- presence of nausea and vomiting
- gastric or hepatic disease
- no asphyxia
- sputum: not frothy, black to brown, coffee ground
- Labs: acidic pH,mixed with food particles
155
What causes acute chest syndrome?
ACS develops when a trigger causes deoxygenation of hemoglobin S (Hgb S), leading to polymerization of Hgb S and sickling of red blood cells (RBCs) within the pulmonary vasculature, which results in vaso-occlusion, ischemia, and endothelial injury -->basically when sickling happens in the lung
Many triggers:
- top 2 causes: infection, vaso-occlusive
- commonly occurs in sickle cell patient already admitted to hospital for VOC-->acute chest due to hypoventilation from pain or from pain control medication
- sickle cell asthma-->higher risk for acute chest syndrome
156
Management for acute chest syndrome?
- Pain control to prevent hypoventilation and atelectasis, ideal to use a non-sedating agent like ketorolac. PCA prevents over sedation
- maintain saturations >=92% since hypoxemia cause sickling in the first place
- incentive spirometry
- positive pressure ventilation (CPAP or BPAP)
- bronchodilators if history of asthma
- may consider corticosteroids if asthma
- broad spectrum antibiotics since infection since infection is one of the most common -->third generation cephalosporin and macrolide
- consider transfusion simple of exchange (if the question stem says that Hb has dropped then make sure you mention this; i think it's normal for Hb drop during ACS)
- Long term, want to try and prevent ACS:
- decrease risk of infection with prophylactic antibiotic, immunization
- manage asthma
- decrease risk of sickling with use of hydroxyurea (I think patients are on hydroxyurea)
- chronic transfusion
157
Monitoring for patient with sickle cell disease?
- parents need to be educated about ACS (this is the biggest cause of death in sickle cell)
- annual spirometry (Kendig's implies this, guidelines don't stipulate this)
- ask about asthma and OSA symptoms
- guidelines doesn't stipulate routine PSG
- AHA 2015 guideline says echo at age 8 to screen for PH
- regular pulse oximetry (may be inaccurate so may need to do blood gas)
158
What is the differential diagnosis of a pulmonary hemorrhage?
- Pulmonary versus non-pulmonary (GI, sinus)
- Upper airway
- Lower airway (Bronchial circulation)--infection, bronchiectasis, neoplasm, foreign, vascular to bronchial tree fistula
- Alveolar focal--eg. pneumonia
- Diffuse alveolar--immune versus non-immune
- pulmonary vascular disease-->in particular, increased pulmonary venous pressure
- bleeding disorder
- trauma
(Box 61.1 in Kendig's for more details)
159
Investigations for pulmonary hemorrhage?
* CT chest with contrast to look for PE, AVM
* Test for coagulation defects
* Bronchoscopy to look for foreign body, airway hemangioma, tumor, presence of hemosiderin laden macrophages
* Infection: blood and sputum cultures, TB testing with purified protein derivative
* If diffuse alveolar opacities—>echo to look for cardiac disease, CT with contrast, cardiac cath
* If systemic involvement (eg. renal disease, rash, joint disease)—>ANA, ANCA, CBC, ESR, CRP, urinalysis, metabolic panel, d-dimer, von willebrand factor, anti-phospholipid antibody, lupus anticoagulant
* If DAH and no cardiac, renal or systemic disease with negative antibodies (ANA, ANCA, anti-GBM)—>transthoracic biopsy either through open approach (mini-thoracotomy) or VATS
160
What is orthodeoxia and what are some causes?
- Desaturation in oxygen saturation of >2-5% when moving from supine to upright
- Causes:
- pulmonary AVM (predilection for lower lobes)
- hepatopulmonary syndrome (since there are intrapulmonary vascular dilatations which are preferentially in dependent position)
- Inter-atrial shunt like PFO
161
What is a pulmonary AVM?
Abnormal vessel, Direct connection between pulmonary artery and vein, without capillary bed in between
"aneurysmal", dilated, tortuous
Usually 1 single arterial vessel and 1 single venous vessel
