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Pulmonology Board Review > Less Common Pulmonary Diseases > Flashcards

Less Common Pulmonary Diseases Flashcards

1
Q

Diameter of wall for classification of a cyst

A

<2 mm

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2
Q

5 major types of diffuse cystic lung disease

A

LAM, PLCH, BHD, LIP, amyloidosis

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3
Q

CT findings in LAM

A

diffuse cystic disease with normal intervening parenchyma

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4
Q

Diagnostic criteria for LAM

A

Characteristic CT findings and 1 of the following (biopsy showing LAM, renal angiomyolipomas, chylothorax, tuberous sclerosis complex, high serum VEGF-D level > 800)

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5
Q

Management of LAM

A

Sirolimus (rapamycin), supportive, lung transplant

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6
Q

Cell proliferation causing LAM

A

HMB-45+ (LAM cells)

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7
Q

Two inheritance patterns of LAM

A

Sporadic and TSC related gene mutations

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8
Q

Characteristic individual with PLCH

A

young adult smokers

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9
Q

Cells found in lesions with PLCH

A

CD1a+, langerin+ CD207

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10
Q

Mutation associated with PLCH

A

BRAF-V600E

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11
Q

2 extrapulmonary diseases associated with PLCH

A

Bone lesions and Diabetes Insipidus

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12
Q

HRCT findings in PLCH

A

Irregular cysts with basilar sparing, nodules +/- cavidation, reticular opacities

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13
Q

Diagnostic criteria for PLCH

A

Characteristic CT findings with BAL >5% CD1A cells or biopsy

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14
Q

Management of PLCH

A

Stop smoking, 2-chlorodeoxyadenosine (2-CdA, cladribine), BRAF inhibitors (vemurafenib) if BRAF mutation, Steroids, Transplant

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15
Q

Inheritance of BHD

A

autosomal dominant, phenotype variable

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16
Q

Genes and chromosome associated with BHD

A

BHD (FLCN) gene on chromosome 17p11.2, tumor-suppressor protein gene, folliculin gene

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17
Q

2 extrapulmonary findings in BHD

A

Fibrofolliculomas and renal tumors

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18
Q

Diagnostic criteria for BHD

A

Cystic disease with any of the following (family hx of BHD, characteristic skin lesions, renal tumors, or gene testing)

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19
Q

Treatment of BHD

A

Management of pneumothorax, life-long surveillance for kidney tumors, no specific therapy

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20
Q

Disease characterized by monoclonal plasma cell proliferation localized to the lung

A

Pulmonary Light Chain Deposition Disease (LCDD)

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21
Q

Substance secreted by macrophages in PLCDD that causes elastolysis and cyst formation

A

metalloproteinases

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22
Q

Diagnostic criteria for PLCDD

A

Biopsy showing non-amyloid deposits in the alveoli and small airways (no beta pleated sheets and negative congo red staining)

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23
Q

Treatment for PLCDD

A

Chemotherapy and HSCT

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24
Q

HRCT findings in PLCDD

A

cysts with nodules

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25
Q

Histopathologic findings in Follicular bronchiolitis

A

Hyperplastic lymphoid follicles with reactive germinal centers along bronchovascular bundles

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26
Q

HRCT findings in follicular bronchiolitis

A

Small nodules with patchy ground-glass opacities and occasional cysts

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27
Q

Diseases associated with follicular bronchiolitis

A

CTDs, immunodeficiency, infections, hypersensitivity reactions

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28
Q

2 rare inheritable causes of lung cysts

A

Neurofibromatosis type 1 (NF1) and Marfan syndrome

29
Q

Disease characterized by accumulation of lipoproteinaceaous material in the alveoli

A

Pulmonary alveolar proteinosis (PAP)

30
Q

Most common cause of PAP

A

autoimmune (90%) disruption of GM-CSF signaling leading to impaired surfactant metabolism by macrophages

31
Q

Labs and antibodies elevated in PAP

A

Serum LDH, surfactant A and D, KL-6, anti-GM-CSF in serum and BAL

32
Q

HRCT findings in PAP

A

GGO and/or consolidative opacities, patchy or diffuse with sharp demarcation from normal lung (Crazy Paving)

33
Q

Treatment of PAP

A

Whole lung lavage (supportive care only if mild), GM-CSF replacement, rituximab, statin

34
Q

Disease characterized by extensive intraalveolar deposition of concentrically lamellated calcium phosphate spheres

A

Pulmonary alveolar microlithiasis

35
Q

Inheritance pattern and gene associated with pulmonary alveolar microlithiasis

A

Autosomal recessive, SLC34A2 gene

36
Q

Mechanism behind pulmonary alveolar microlithiasis

A

SLC34A2 gene mutation leads to lack of type IIb sodium phosphate cotransporter in the lungs, leads to abnormal phosphorus metabolism by type II cells causing calcium phosphate microliths

37
Q

Treatment for pulmonary alveolar microlithiasis

A

none, lung transplant

38
Q

HRCT findings in pulmonary alveolar microlithiasis

A

“Sandstorm” appearence of parenchyma with black pleural line

39
Q

Rare disease characterized by foamy CD1a negative histiocytes, fibrosis, and osteosclerotic lesions of long bones

A

Erdheim-Chester Disease

40
Q

Management of Erdheim-Chester Disease

A

BRAF inhibitors (vemurafenib), interferon-alpha, chemo

41
Q

Rare disorder characterized by diffuse lymphatic proliferation in the lungs

A

Diffuse pulmonary lymphangiomatosis

42
Q

HRCT findings in diffuse pulmonary lymphangiomatosis

A

Diffuse interlobular septal thickening, patchy GGO, mediastinal infiltration, and pleural thickening/effusion

43
Q

Treatment of Diffuse pulmonary lymphangiomatosis

A

sirolimus, bevacizumab

44
Q

Most common form of amyloidosis

A

AL

45
Q

Two different patterns of parenchymal amyloidosis

A

Nodules (amyloidomas) or diffuse infiltrates with septal thickening

46
Q

Diagnosis of pulmonary amyloidosis

A

biopsy material showing apple-green birefringence with polarized light on congo red staining

47
Q

Treatment of trachobronchial amyloidosis

A

Bronchoscopic laser, external beam therapy

48
Q

Treatment of diffuse parenchymal amyloidosis

A

treat underlying plasma cell dyscrasia

49
Q

Disease characterized by lymphoplasmacytic infiltrate of IgG4 plasma cells and fibrosis

A

IgG4 related disease

50
Q

Diagnosis requirements of IgG4RD

A

2 out of 3 (dense lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis), or High IgG4 level with clinical symptoms

51
Q

Treatment of IgG4RD

A

30-60 mg/day of prednisone then taper over weeks to months, rituximab

52
Q

Timing post radiation for radiation pneumonitis

A

4-12 weeks

53
Q

Timing post radiation for radiation fibrosis

A

6-12 months

54
Q

Treatment of radiation pneumonitis

A

40-60 mg/day prednisone x 2 weeks, then taper over 4-12 weeks

55
Q

Disease characterized by congenital pulmonary manifestation with anomalous systemic arterial supply from the aorta

A

Bronchopulmonary sequestration

56
Q

Raoof article differential for true cysts that are subpleural

A

Bullae, paraseptal emphysema, honeycombing

57
Q

Raoof article differential for true cysts that are intraparenchymal and associated with GGO

A

PCP pneumonia, DIP

58
Q

Raoof article differential for true cysts that are intraparenchymal with nodules on CT

A

LIP, PLCH, Light chain deposition disease, AMyloidosis

59
Q

Raoof article differential for true cysts that are intraparenchymal, no other CT findings, and are solitary or incidental

A

Incidental cyst, pneumatocele, bronchogenic cyst

60
Q

Raoof article differential for true cysts that are intraparenchymal, no other CT findings, and diffuse/multiple found

A

BHD, LIP, Malignancy, Infection, LAM, PLCH, tracheobronchial papillomatosis

61
Q

Cystic lung disease associated with

  • Lower lobe predominant
  • Elongated
  • Subpleural and paramediastinal
  • Associated with a 24% pneumothorax risk
A

Birt Hogg Dube

62
Q
A

Fibrofolliculomas in birt hogg dube

63
Q

Cystic lung disease associated with

  • Round cysts
  • Diffuse involvement including juxtaphrenic recesses
  • Intervening normal lung
  • Chylous effusions
  • Effects women of childbearing age
  • Renal angiomyolipomas
A

LAM

64
Q

Cystic lung disease associated with

  • Cysts with nodular walls
  • Pathcy opacities and nodules
  • Areas of fibrosis
  • Multiple resections of papillomas and tracheostomies
A

Trachobronchial papillomatosis

65
Q

Cystic lung disease associated with

  • Few cysts or centrolobular nodules
  • Bilateral reticular opacities, lower lobe predominant
  • Septal thickening
  • Middle aged women
  • Hx of CVD, HIV, or CVID
A

LIP

66
Q

Cystic lung disease associated with

  • Nodules predominantly
  • Mediastinal adenopathy
  • Plasma cell dyscrasias
  • Ig deposition in kidneys, heart, and liver
A

Light chain deposition disease

67
Q

Cystic lung disease associated with

  • Nodules primarily
  • Sjogren’s syndrome
  • GGO
  • Nodules are lower lobe predominant
A

Amyloidosis

68
Q

Cystic lung disease associated with

  • Upper and middle lobe nodules
  • Variable size nodules that are bizarre shapes
  • Spares costophrenic angles
A

PLCH

Pulmonology Board Review (32 decks)

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