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Pulmonary > ILD > Flashcards

ILD Flashcards

1
Q

Path buzzword: UIP

A

Lymphoplasmacytic inflammation with fibroblastic foci and honeycomb change
–Temporal heterogeneity (juxtaposition of fibrosis and normal lung)

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2
Q

Path buzzword: LIP

A

Dense accumulation or “sheets” of lymphocytes and plasma cells within interstitium and alveolar spaces

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3
Q

Path buzzword: RBILD

A

accumulation of pigmented macrophages within respiratory bronchioles and alveolar ducts with bronchiolcentric distribution
-No significant fibrosis, interstitial inflammation, germinal centers

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4
Q

Path buzzword: DIP

A

Similar to RBILD but with alveolar spaces uniformly filled of pigmented macrophages. Homogeneous filling with preserved alveolar architecture and minimal fibrosis.

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5
Q

Features consistent with UIP

A
  1. Basal and subpleural predominant
  2. Reticular opac (with traction supportive of fibrosis)
  3. Honeycombing
  4. Absences of Inconsistent Features
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6
Q

Inconsistent with UIP

A
  1. GGO (outside areas of fibrosis)
  2. Mosaicism/air trapping
  3. Nodules- centrilobular, perilymphatic
  4. Parahilar, peribronchovascular
  5. Upper lobe distribution
  6. Peripheral fibrosis with minimal honeycombing, subpleural sparing
  7. lower lobe distribution, not subpleural
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7
Q

Mutation leading to PAM

A

SLC34A2; autosomal recessive

Type IIb sodium-phosphate cotransporter

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8
Q

PAP associated with Ab against

A

GM-CSF

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9
Q

Genetic pulmonary fibrosis:

A

hTERT and hTR are AD

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10
Q

Pathologic hallmark of NSIP

A

cellular infiltrate with temporal uniformity

BAL lymphocytosis >20%

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11
Q

RB-ILD pathology

A

Pigmented alveolar macrophages in and around respiratory bronchioles and surrounding alveoli

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12
Q

DIP pathology

A

alveolar spaces uniformly filled with pigment-laden macrophages. Homogenous with preserved alveolar architecture and minimal fibrosis or honeycombing.

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13
Q

LIP Pathology

A

interstitial and bronchovascular infiltrate of lymphocytes and plasma cells. Can have poorly formed granulomas and multinuc giant cells

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14
Q

PLCH: diagnosis

-IHC

A

BAL with >5% CD1a positive cells

-IHC with CD1a and S-100

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15
Q

PLCH: pathology

A

bronchiolocentric stellate lesions with central scar; intracellular inclusions (Birbeck granules) on EM, extensive Eos in early phase

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16
Q

LAM: pathology

A

hamartomatous proliferation of atypical smooth muscles along lymphatics in lung, thorax, abdomen/pelvis

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17
Q

LAM: Cell expression

A

HMB-45; CD-63 on
1. myofibroblast-like spindle-shaped cells 2. epithelioid-like polygonal cells

-just spindle-shaped cells: muscle alpha-actin, vimentin, desmin (on )

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18
Q

BAL Findings in IIP:

1. NSIP

A

BAL with >20% lymphs (overlap with HP and drug-induced)

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19
Q

BAL Findings in IIP:

COP

A

Charac mixed pattern: lymphocytes (20-40%), neutrophil (10%), and Eos (5%) with some plasma/mast cells
-CD4/CD8 is decreased

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20
Q

BAL Findings in IIP:

PLCH

A

> 5% CD1a-positive cells

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21
Q

BAL Findings in IIP:

AEP

A

> 25% Eos, normal periph Eos

-Tbbx with marked eosinophilic infiltration of alveoli and interstitium

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22
Q

BAL Findings in IIP:

CEP

A

> 25% Eos

-Tbbx whos eosinophilic microabscesses, low grade vasculitis, and interstitial fibrosis

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23
Q

Nintedanib

  1. Mech
  2. Side effects
A

TK receptor blockers- mediate fibrogenic growth factors (PDGF, VEGF, FGF)

Diarrhea and LFT derangements, CytP3A4

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24
Q

Pirfenidone

  1. Mech
  2. Side effects
A
  1. Antifibrotic agent, inhibits TGF-b stimulated collagen synthesis, block fibro last proliferation
  2. nausea, rash
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25
Q

Mutations leading to IPF

A

hTERT, hTR, Muc 5B

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26
Q

HRCT RB-ILD vs DIP

A
  1. RBILD: fine centrilobular nodules, bilat patch GGOs upper and lower lung zones.
  2. DIP peripheral GGO, in lower lung zones, possible cyst/emphysema, no honeycombing
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27
Q

Classic CTc findings for LIP

A
  1. Bilat GGO
  2. Centrilobular nodules
  3. Bronchovascular bundle thickening and interlobular septal thickening
  4. thin-walled cysts
28
Q

Gene assoc with LAM

A

TSC1 and TSC2

29
Q

Diagnostic test to differentiate berylliosis from sarcoid:

A

Lymph transfer test on BAL or beryllium lymphocyte proliferation test

30
Q

CD4/CD8:

A
  1. Normal= 1.5-2
  2. Sarcoidosis: >3.5 has sens of 50% and spec of 94%
  3. Organizing pneumonia: CD4/CD8 is decreased
  4. HP: decreased CD4/CD8
31
Q

Tumors associated with Birt Hogg Dube:

A

Renal cell carcinoma, oncocytic hybrid tumor, clear cell carcinoma

32
Q

Birt Hogg Dube mutation

A

AD, chr 17

BHD gene, encodes folliculin (tumor suppressor)

33
Q

Classic Histopath finding with amiodarone lung toxicity

A

Foamy macrophages with fibrosis and lamellated inclusions

34
Q

Lung toxicity with 5-ASA and sulfasalazine

A

Eosinophilia, OP, BO

35
Q

Caplan Syndrome

A

Nodular reaction in patient with RA (or will develop RA in 5-10yr) exposed to coal dust. Focal lesion with necrotic center surrounded by lymphocytes and plasma cells and coal dust

36
Q

Histology of silicotic nodule

A

Central dense hyalinzed collaged contained silica particle surrounded by concentric collagen fibers, peripheral thick capsule of dust-lade macrophages, lymphocytes, and collagen

37
Q

Talc Pathology

A

Granulom with multinucleated giant cells in interstitium and pulmonary arteries (in IVDU). WITH birefringent talc crystals

38
Q

Factors that determine degree of injury to upper and lower respiratory tract

A
  1. particle size
    >10um filtered by upper pharyngeal anatomy, <5um bypass resp tract and desposit in lower resp tract
  2. concentration
  3. water solubility
    -water soluble react with mucosal surfaces of upper airway
    -less water soluble- lower resp tract and delayed parenchymal abnml
  4. location- ventilation or not
  5. physical properties
39
Q

Water soluble inhalation injuries

A

immediate irritation and reactivity of conjunctival surfaces

  • Ammonia
  • SO2
  • chlorine
40
Q

Less water soluble agents

A

No acute, irritating symptoms, react with lower resp tract and cause delayed, severe parenchymal abnml

  • NO2 (silo fillers)
  • phosgene (welding, meth)
  • SO2
41
Q

Classic BAL Findings PAP

A

Milky effluent

  • alveolar macrophages filled with PAS- + material
  • Lamellar bodies
42
Q

Distinguish berrylium form sarcoid

A

Beryllium proliferative test (lymphocytes stimulated with soluble beryllium salts)

43
Q

Pathology: HP

A

airway-centered, variably cellular chronic interstitial pneumonia, a lymphocyte-rich chronic bronchiolitis, and poorly formed nonnecrotizing granulomas distributed mainly within peribronchiolar interstitium

44
Q

Skin manifestations of BHD

A
  1. Fibrofolliculoma
  2. trichodiscoma
  3. Skin tag
45
Q

Renal tumors seen with BHD

A
  1. Oncotic hybrid tumor
  2. RCC
  3. Clear cell carcinoma
  4. papillary renal cell carcinoma
46
Q

Most common Ab in DM/PM and ILD

A

Anti-Jo (90% with this Ab have ILD)

47
Q

Other DM/PM ILD Ab

A

SRP, Mi-2, amino-acyl transfer RNA synthetase

48
Q

Amyositis DM/PM ILD

A

anti-MDA5 (rapidly progressive)

PL-12 (anti-synthetase)

49
Q

Associated with myositis/scleroderma overlap

A

Anti-Ku

50
Q

Assoc with severe necrotizing myopathy (DM/PM)

A

anti-SRP

51
Q

CREST syndrome assoc with Ab:

A

anti-centromere

52
Q

Homogeneous/diffuse ANA assoc with

A

SLE or MCTD

53
Q

Nucleolar ANA assoc with:

A

Systemic sclerosis, PM

54
Q

Centromere ANA assoc with:

A

Systemic sclerosis

55
Q

Mixed Connective Tissue disease assoc with Ab:

A

anti-RNP, anti-U1

56
Q

Sjogren Syndrome Ab

A

SSA (Ro), SSB (La)

57
Q

Systemic scerlosis/scleroderma assoc with Ab:

A

Scl-70 (anti-DNA topoisomerase 1)

58
Q

Characteristic features of IgG4 related disease

A
  1. Dense lymphoplasmacytic infiltrate
  2. Storiform fibrosis
  3. obliterative phlebitis
  4. IHC with IgG4+ cells
59
Q

5 drugs cause drug-induced lupus

A

hydralazine, isoniazid, procainamide, quinidine, penicillamine

60
Q

Major Drugs causing drug-induced HP

A

MTX, Taxanes

Abx, TNF alpha inhibitors

61
Q

BCNU toxicity:

dose

A

timing: days to 17 years
Dose: >1500mg/m2 (25-50% will develop pulmonary toxicity)
Mech: oxidant-induced (decreased glutathione)

~90% mortality

62
Q

Bleomycin- 6wk- 6mos

-with high O2 can awaken fibrosis/inflammation (binds histones)

A

> 500 units

Increased risk with age >70, O2, radiation, abnormal renal function

63
Q

Most common type of pulmonary toxicity with methotrexate

A

HSP

64
Q

Amiodarone: dose for lung toxicity

A

Much more common at >400mg/d

-increased in >60yo

65
Q

Anti-TNF alpha inhibitors: forms of toxicity

A
  • granulomatous (sarcoid) ILD

- lymphohistiocytic reaction

66
Q

Rituximab pulmonary toxicity

A

Can cause acute/subacute cellular ILD, profound lymphocytosis on BAL
-also BOOP or pulmonary fibrosis

Pulmonary (14 decks)

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