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ILD Flashcards

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1
Q

What is Dermatomyositis associated with?

A

anti MDA-5 (associated with progressive ILD)
Mi1-2
hyperkeratosis and periungal erythema, dilated nail capillaries

Dermatomyositis is characterized by specific skin manifestations and muscle involvement.

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2
Q

Which antibodies are associated with Antisynthetase syndrome?

A

anti-jo-1, anti-PL7, anti-EJ

Antisynthetase syndrome is characterized by myositis and proximal muscle weakness.

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3
Q

What markers are used to identify Langerhans cell histiocytosis?

A

Cd1a, S-100, stellate

These markers are important in the diagnosis of Langerhans cell histiocytosis.

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4
Q

What is a key marker for LAM (Lymphangioleiomyomatosis)?

A

serum VEGF-D, mTOR mutation; HMB-45 stain positive

These findings are critical for the diagnosis of LAM.

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5
Q

What characterizes Birt-Hogg-Dube syndrome?

A

cystic; autosomal dominant, cutaneous fibrofolliculomas, renal adenocarcinoma, stain positive follicular (FLCN)

Birt-Hogg-Dube is a genetic disorder associated with skin lesions and increased cancer risk.

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6
Q

What is Pulmonary Benign Metastasizing Leiomyoma (PBML)?

A

women with history of uterine leiomyoma, spindle cell smooth muscle, can be military, multiple nodules, low or no FDG avidity

PBML is characterized by specific histological features and immunohistochemical positivity.

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7
Q

Fill in the blank: Follicular bronchiolitis is associated with _______.

A

sjogren, lymphoid hyperplasia of bronchial-associated tissue along bronchi vascular bundles

Follicular bronchiolitis can be related to autoimmune conditions.

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8
Q

What are the histological features of Nodular Lymphoid Hyperplasia?

A

interfollicular plasma cell, lymphoid, histiocytes on path, mass like, subpleural, cysts

Nodular lymphoid hyperplasia can present as a mass-like lesion in the lungs.

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9
Q

When does Bleomycin toxicity typically occur?

A

1-6 months after exposure

Risk factors include renal failure, high oxygen levels, and G-CSF administration.

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10
Q

True or False: RA associated lung involvement commonly presents with pleural effusions.

A

True

RA lung involvement can manifest in various forms including nodules and bronchiectasis.

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11
Q

What is the most common form of ILD associated with rheumatoid arthritis?

A

UIP (Usual Interstitial Pneumonia)

NSIP (Nonspecific Interstitial Pneumonia) can also be seen but is less common.

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12
Q

What is the prognosis of UIP related to autoimmune disease?

A

slower to progress and has better prognosis

Compared to other forms of UIP, autoimmune-related UIP tends to have a more favorable outcome.

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13
Q

What is the best treatment for myositis in the context of lung involvement?

A

Mycophenolate is best, but bad for RA joints

Treatment options need to be tailored based on the patient’s specific conditions.

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14
Q

What is the characteristic triad of idiopathic pulmonary hemosiderosis?

A

microcytic anemia, recurring hemoptysis, bilateral alveolar opacities without a clear cause (and bland biopsy without vasculitis)

This condition primarily affects children.

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15
Q

What histological finding is associated with Amiodarone-induced pneumonitis?

A

lipid-laden foamy macrophages in alveolar spaces

This finding is specific to the lung injury caused by Amiodarone.

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16
Q

What condition is characterized by the presence of parotid enlargement, facial nerve palsy, and anterior uveitis?

A

Heerfordt’s syndrome

Heerfordt’s syndrome is associated with sarcoidosis.

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17
Q

Fill in the blank: Lofgren’s syndrome includes _______.

A

EN, bilateral lymphadenopathy, migratory polyarthralgias

Lofgren’s syndrome is a form of acute sarcoidosis that often improves with NSAIDs.

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18
Q

What is the association of Interferon gamma receptor deficiency in young patients?

A

sarcoidosis and NTM

This deficiency can lead to increased susceptibility to certain infections and autoimmune conditions.

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19
Q

What can the end-stage HP resemble?

A

UIP

End-stage hypersensitivity pneumonitis can have similar radiological and pathological features to UIP.

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20
Q

What genetic predispositions are linked to IPF?

A

hermansky pudlak, short telomere, autoimmune, surfactant issues, dyskeratosis

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21
Q

VEXAS

A

autoinflammatory disorder in pediatrics leading to ILD later in life

Vacuoles
E1 ubiquitin activating enzyme
on the X chromosome
with Autoinflammation
Somatic (not passed down)

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22
Q

Erdheim-Chester disease

A

histiocytosis syndrome
BRAF V600E mutation
Pro-inflammatory cytokines
Sclerotic lesions of long bones
Skin findings
Retroperitonium involvement (like IgG4 disease)
PET-avid
foamy histiocytes
CD1a negative
MNG histiocytes (touton cells) - giant cells

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23
Q

Ddx of Lymphocytic BAL (>25%)

A
  1. Sarcoidosis
  2. NSIP
  3. HP
  4. Drug-induced
  5. CTD
  6. Radiation
  7. COP
  8. Lymphoproliferative disorders
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24
Q

Ddx for Eosinophilic BAL (>25%)

A
  1. Eosinophilic pneumonias
  2. Drug-induced
  3. BM transplant
  4. Asthma
  5. ABPA
  6. Infectious
  7. Hodgkin
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25
Exogenous lipoid pneumonia TBBx and BAL findings
lipid-laden macrophages
26
PAP stain
PAS stain
27
CD1a stain
PLHC (>5%)
28
Recommended number of pieces to biopsy when doing surgical lung bx in ILD
2-3 multi-lobe From areas of active disease
29
Factors increasing risk of in-hospital mortality in surgical lung bx in ILD
non-elective (16%) Male sex increased age higher comorbidity scores open vs. VATS provisional diagnosis of IPF or CTD-ILD
30
Smoking-related interstitial disease
Respiratory bronchiolitis-interstitial lung disease (RB-ILD) Desquemative Interstitial pneumonia (DIP) PLCH langerhan's AEP Acute eosinophilic pneumonia CPFE (combined pulmonary fibrosis and emphysema)
31
Idiopathic pleuroparenchymal fibroelastosis (iPPFE)
2/2 systemic disease Upper lobe predominant fibrosis Can look like UIP but upper lobe, think iPPFE or burnt out HP
32
Histology and HRCT of UIP
Path: Peripheral patchy fibrosis, FF, honeycombing, +/- inflammation CT: Subpleural and basal, reticular, traction bronchiectasis, honeycombing. If costophrenic angles are spared, it is not IPF
33
Histology and HRCT of NSIP
Path: Homogenous, chronic interstitial inflammation/fibrosis CT: homogenous, GGOs/reticular, subpleural sparing
34
Histology and HRCT of OP
PAth Patchy, polypoid granulation tissue plugs CT: patchy consolidations, GGOs, migrating
35
Histology and HRCT of RB-ILD/DIP
Path: pigmented brownish (smoker's) macrophages, bronchiolocentric DIP more busy CT: vague NODULES patchy to diffuse GGOs
36
Histology and HRCT of DAD
Hyaline membranes, granulation tissue, organizing/fibroproliferative CT: diffuse consolidation/GGOs
37
Histology and HRCT of LIP
Path: lymphoid, plasma cells (very blue) CT: patchy GGOs, nodules, cystys
38
Histology and HRCT of PPFE
Path Pleural/subpleural fibroelastosis, septal elastosis CT: UPPER/subpl fibrosis +/- PTX pneumomediastinum
39
"Typical" UIP pattern HRCT (4)
1. Honeycombing 2. Reticular + peripheral traction bronchiectasis/bronchiolectasis 3. Basal and subpleural 4. Absence of features insonsistent with UIP pattern (extensive consolidations, GGOs, mosaic attenuation, nodules, cysts)
40
"Definite" UIP histopathologic pattern
1. Dense fibrosis/architectural distortion +/- honeycombing 2. PAtchy involvement 3. Fibroblast foci 4. Subpleural +/- paraseptal predominance 5. No features suggesting alternative diagnosis (e.g. granulomas)
41
Difference between "probable" and "typical" UIP
"Probable": no honeycombing in "probable" "Intermediate": diffuse, not basilar or subpleural
42
Definitive diagnosis of ILD in undetermined type
cryobiopsy
43
"Acute exacerbation" of IPF
new widespread alveolar abnormality often precedes death antifibrotic therpy may help prevent AE in IPF, steroids not likely helpful
44
Benefit of antifibrotic therapy
Reduces rate of FVC decline (evidence based) May help with: - QOL - Reduce rate of hospitalization
45
ASCEND Study - Pirfenidone 2014 INPULSIS-1 & 2 - Nintedanib 2014 What did they find?
Decrease rate of FVC decline in both studies
46
True or false, risk of lung ca is increased in IPF
true
47
In Non-IPF ILD (Progressive fibrosing ILD and SSc-ILD), what is the role of antifibrotics
Nintedanib can decrease rate of FVC decline (INBUILD trial NEJM 2019 & SENSIS trial NEJM 2019) Pirfenidone also (maher Lancet 2019) - not as good at decreasing rate of decline of FVC
48
Classification of "progressive pulmonary fibrosis"
Non-IPF Decline in DLCO >10% Decline in FVC >5% Radiologic progression
49
Indications for Nintedanib and Pirfenidone
Nintedanib: IPF, SSc-ILD, Chronic fibrosing ILD with progressive phenotype Pirfenidone: IPF only
50
"Familial pulmonary fibrosis"
1. 2 or more relatives who share common ancestry 2. 10-20% of interstitial pneumonia/pulmonary fibrosis 3. 25% of familial cases have identifiable gene variant 4. Should do genetic testing in those with early onset (<50 yo)
51
Typical HRCT features of PLCH
upper lobe bizarre cystic changes
52
Stains typically seen with PLCH
CD1a, S100, Langerin (CD207)
53
Management of PLCH
Stop smoking BRAF inhibitors (vemurafenib) if BRAF-V600E mutation Cladribine, cytarabine,cobimetinib, MTX
54
CPFE HRCT and PFT findings
HRCT: upper lobe lung emphysema with lower lobe fibrosis PFT: Preserved spirometry and lung volumes with low DLCO High risk for PH and lung ca
55
RA associated ILD
*** UIP is most common *** then NSIP OP Can also affect airway (constrictive bronchiolitis), vascular, pleural, associated with pulmonary amyloidosis
56
Sjogren-ILD
NSIP > LIP > UIP > OP, and amyloid MALToma HRCT: cysts and nodules
57
Antisynthetase-ILD antibodies
Anti-jo 1 Aminoacyl-tRNA synthetase
58
RA-ILD is highly associated with this high risk behavior
smoking
59
Antibody associated with SSc-ILD
anti-SCl70, anti-centromere
60
Treatment for SSc-ILD
1st line MMF 2nd Tocilizumab 3rd line RTX 4th Cyclophosphamide & Nintedanib 5th HSCT
61
RA-ILD treatment
1st Steroids 2nd MMF, AZA 3rd RTX 4th Abatacept 5th Nintedanib
62
Sjogren-ILD tx
1st steroids 2nd MMF, AZA 3rd RTX
63
PM/DM-ILD tx
1st steroids 2nd MMF, AZA, MTX 3rd Tacrolimus, cyclosporine 4th RTX, IVIG 5th Cyclophosphamide
64
Tx of SLE-ILD
1st steroids 2nd AZA 3rd MMF 4th RTX 5th cyclophosphamide
65
Significance of IPAF
can't identify a CTD but they have some positive autoimmune serology 2/3 of clinical or serologic findings
66
Sarcoid tx if asymptomatic
No treatment, repeat imaging
67
3 Syndromes of definite sarcoid that do not need biopsy
1. Lofgren: E nodosum, bilateral LN, migratory polyarthralgias 2. Heerfordt's: parotid swelling, uveitis, Bell's palsy) 3. Lupus pernio
68
Diagnostic options for sarcoidosis
Biopsy of most accessible tissue lymphocytic >15% CD4/CD8 >4 (highly specific) EBUS: 80% yield TBBx 50-90% Endobronchial bx: 25% yield
69
Tx of sarcoidosis
prednisone 20-40 mg 2nd line methotrexate
70
Limit of thickness of cyst wall (to compare to cavities and emphysematous areas)
<2 mm
71
5 cystic lung diseases
LAM BHD PLCH LIP Amyloidosis
72
HMB-45 staining cells correlates with that disease
LAM
73
Threshold of serum VEGF-D to dx LAM
>800 pg/mL
74
PFT in LAM
obstructive, low DLCO
75
Diagnosis of LAM needs these
1. CT 2. LAM on biopsy 3. renal AML 4. chylothorac 5. TSC OR elevated serum VEGF >800
76
treatment of LAM
sirolimus lung transplant
77
Birt-Hogg-Dube mutation
FLCN gene
78
Distribution of cysts in BHD
lower to mid lung fields
79
If thinking LIP, what serology should you send
workup for Sjogren HIV dysproteinemia (polyclonal)
80
Cysts and nodules, think ___
LIP Amyloidosis Follicular bronchiolitis (less cysts) - favorable prognosis
81
Light Chain deposition disease vs. amyloid
Non-amyloid deposits, does not stain congo red no zonal predilection
82
Follicular bronchiolitis
Hyperplastic lymphoid follicles with reactive germinal centers along bronchovascular bundles Associated with CVID, COPA (cell trafficking issue), and sjogren's
83
Tx of light chain deposition disease
chemotherapy HSCT
84
Most frequent types of Amyloidosis
AL (primary) - light chain AA (secondary) - chronic inflammatory disorders - infrequent Infrequent with ATTR (familial)
85
Manifestations of Amyloidosis in the lungs
- Diffuse thickening tracheobronchial tree (posterior membrane NOT spared), foci of calcification - pulmonary parenchyma - nodules (amyloidomas), reticular pattern, predominantly cystic, can be calcified localized/diffuse pattern - pleural effusion - intrathoracic adenopathy - OSA from microglossia
86
Management of pulmonary amyloidosis
tracheobronchial: bronchoscopic laser and external beam therapy Nodules: observe Diffuse parenchymal: treat underlying plasma cell dyscrasia
87
IgG4 disease diagnosis
Histopathological: (2 of 3 major feature) 1. Dense lymphoplasmacytic infiltrate 2. Fibrosis, at least focally storiform (cart-wheel like) 3. Obliterative phlebitis Immunohistochemical: Increased IgG plasma cells AND IgG4 to IgG cell ratio AND Clinical context (serum IgG4 level 70-80%); response to steroids
88
Treatment of IgG4 disease
steroids 30-60 mg/d then taper Rituximab
89
IBD-related bronchiolitis tx
corticosteroids (inhaled or systemic) JAK2 inhibitor (prob not on the boards)
90
PAP etiology (primary)
autoimmune (90%) GM-CSF production disruption or receptor (anti-GSM-CSF antiboties) --> decreased surfactant
91
Causes of secondary PAP
acquired macrophage dysfunction (silicoproteinosis, fentanyl patch smoking, hematologic dyscrasias, infections)
92
Tx of PAP
whole lung lavage (benefit 15 mo) GM-CSF SQ daily, nebulized BID q other week Rituximab ?Statin PAP can recur after lung transplant
93
WHat is pulmonary alveolar microlithiasis
intraalveolar deposition of concentrically lammellated calcium phosphate spheres Dx teen-50yr CT: calcific micronodular infiltrate on imaging; "sandstorm" with black pleural line AR genetic: mutation SLC34A2 for typeIIb sodium phosphate cotransporter Tx lung transplant
94
Long bones pain, ILD, think ___
Erdheim-Chester Disease
95
Diffuse Pulmonary Lymphangiomatosis
present with chyloptysis, cough, wheezing HRCT: septal thickening that is smooth, GGOs, mediastinal infiltration Dx SLBx Management sirolimus? bevacizumab? propranolol?
96
Timing of radiation pneumonitis
4-12 weeks after radiation
97
Timing of radiation FIBROSIS
6-12 mo after irradiation (straight line effect)
98
Timing of radiation OP
1-12 mo (usually within 6 mo) OUTSIDE of the radiation field Responds well to glucocorticoids Seen in women with breast ca
99
Constrictive bronchiolitis (BOS)
allograft recipients Severe obstructive lung diasease inhalational injury - fumes, vaping DIPNECH Swyer James McLeod (idiopathic constrictive bronchiolitis after infection, UNILATERAL)
100
What is DIPNECH (what, imaging, dx, tx)
Diffuse infiltrative pulmonary neuroendocrine cell hyperplasia Associated with constrictive bronchiolitis mosaic pattern HRCT - tiny nodules Dx lung biopsy Tx: somatostatin?
101
Constrictive bronchiolitis
obstructive if very severe, insp/exp CT will not be too different due to extent of hyperinflation
102
Management of constrictive bronchiolitis
Macrolides, inhaled ICS-formoterol? Transplant
103
recurrent infection, lower lobe L>R consolidations, think ___
Bronchopulmonary sequestration
104
usually 1st year of life, recurrent pulm infections, multiple cysts, connected to tracheobronchial tree, pulmonary arterial supply. Dx?
Congenital pulmonary airway malformation (CPAM)
105
What is TGF-beta associated with
fibroblasts and IPF
106
WHat is IL-13 associated with
T-cells, IgE, asthma
107
What is IL-2 associated with
T cells, B cells, melanoma, renal cell Ca
108
Which stage of ABPA is when pt achieves remission
Stage II (sx resolution, reduced IgE) Other stages: 1 - acute (elevated IgE, asthma, eosinophilia, opacities, precipitating Ab) 3- Exacerbation (increased IgE and sx recurrence) 4- steroid dependent asthma, elevated IgE 5- fibrotic lung disease
109
Tx of ABPA
corticosteroids Itraconazole for ~16 weeks; or vori Limited evidence for omalizumab
110
Loeffler Syndrome
simple pulmonary eosinophilia from PARASITES (Ascaris, hookworms, strongyloides) Migratory opacities Pathology: eos infiltrates usually self-limited, may need corticosteroids but CAUTION if you think it's strongy
111
Timing of drug-induced AEP
febrile illness <5d BAL >25% eos or eosinophilic pneumonitis on bx
112
Idiopathic acute eosinophilic pneumonia is associated with ___
recent onset of smoking Dust Environmental stimuli WTC vaping
113
Chronic eosinophilic pneumonia is associated with ___
asthma Microabscesses and non-caseating granuloma on path
114
% of eos on BAL in CEP is usually ___
>30-40%
115
Typical age of hypereosinophilic syndrome
young middle aged males
116
Dx of hypereosinophilic sd
Unexplained eosinophilia >1500 for >6 mo or on 2 exams >1 mo apart and/or End-organ dysfunction due to eosinophils
117
Tx of HES
steroids IFN-alpha Chemotherapy Hydroxyurea Imatinib Mepolizumab
118
3 phases of EGPA
Does not always progress and not all always present: - asthmatic (8-10 years) with allergic rhinitis, atopy - eosinophilic: peripheral and tissue eosinophilia (lung, GI skin) - vasculitic - necrotizing vasculitis of small & medium vessels; extravascular granulomas- constitutional sx
119
Mononeuritis multiplex (foot drop) and asthma, think ___
EGPA
120
EGPA dx
4/6 criteria: 1. Asthma 2. Peripheral eos >10% or >1500 3. Paranasal sinus abnormalities 4. Mononeuropathy or polyneuropathy 5. Migratory pulmonary opacities (nodules, GGO, cavitation 6. Extravascular eos on biopsy
121
Serology in EGPA
pANCA, MPO 40-60% - more of the vasculitic phase if ANCA negative -- more eosinophilic phase
122
Tx EGPA
corticosteroids Ritux Azathioprine MTX Leflunomide Mepolizumab
123
What is Five Factor Score for EGPA
Age >65 Cardiac involvement Renal GI Absence of ENT involvement (0-2 with 1 point/factor) If FFS 2 or more, or organ infiltration, need stronger medications
124
Asteroid body
sarcoid
125
Treatment of refractory checkpoint inhibitor pneumonitis
Steroids --> **Infliximab** or **mycophenolate**

PULMONARY BOARDS (9 decks)

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