Rheumatologic Lung DZ Flashcards

1
Q

What are the five rheumatologic diseases that often present with pleuropulmonary symptoms

A

Systemic sclerosis, systemic lupus rheumatoid arthritis, dermatomyositis, Sjrogen Syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Which of the fiv efeatures the MOST lung dz symptoms

A

Systemic sclerosis (aka scleroderma)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Lung dz in scleroderma takes two forms, they are:

A

Interstitial and Vascular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Interstitial lung dz in scleroderma or “SCLERODERMA LUNG” most often presesnts as

A
  • non-specific interstitial pneumonia or interstitial fibrosis
  • Concentric arterial thickening
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

About 67% of systemic sclerosis pts get

A

interstitial lung dz

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

About 20% of systemic sclerosis pts suffer from

A

pulmonary hypertension

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

The pathophysiological mediators in Scleroderma Lung are:

A

IL-8, TNF,

MIF1alpha

RANTES

Endothelin 1
TGF-Beta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

IL8 does

TNF alpha does

A
IL8= neutrophil attractant and activator
TNFalpha-= neutrophil attractant and promoter of neutrophil endothelial binding
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

MIF1 alpha does

A

promoter of neutrophil chemotaxis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

RANTES

A

T cell recruiter and activator

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Endothelin 1

A

Vasoconstrictor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

TGF-beta

A

promoter of fibroplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Scleroderma lung favors which part of the lung?

A

Base, posterior, periphery.

You will first see Ground glass appearance on the CT then will become more reticular

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Early histology of scleroderma lung shows

A

Non-specific interstitial pneumonia with lymphocytes and macrophages followed by fibroblasts and collagen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Trichrome stain stains collagen what color?

A

blue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

The vascular disease component of scleroderma lung causes

A

pulmonary hypertension and cor pulmonaale

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is CREST syndrome

A

When the the vascular component of scleroderma appears without the interstitial lung disease.
It is associated with anti-centromere antibodies and limited systemic sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are the symptoms of SS lung dz

A

Dyspnea and dry cough due to increased work load of breathing due to fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Signs of SS Lung

A

Crackles due to the sudden opening of small airways abnormally closed by the pressure of interstitial inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

SS Lung dz diagnosis

A

Associtation of symptoms , signs, and radiology of interstitial lung dz with skin, esophageal, and renal manifestations of scleroderma.

  • Anti-scl70 (DNA Topoisomerase) antibodies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Treatment of systemic sclerosis

A

Cyclophosphamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Why don’t you treat SS lung dz with steroids?

A

Renal crisis has been reported

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Most common cause of death for scleroderma pts

A

Lung Disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Prognosis of scleroderma pts from initial survival?

A

12 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Pleuropulmonary disease of lupus generally has 5 manifestations. THey are:

A
  • Pleuritis
  • Acute Lupus pneumonitis
  • Nonspecific interstitial pneumonia
  • Pulmonary Vascular dz
  • Shrinking lung syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

The most common pleuropulmonary manifestation of lupus by a long shot is:

A

Pleuritis (present in 20% at onset, 50% over time, and 100% at autopsy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Lupus pleuritis is typically

A

Fibrinous

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Is fibrinous pleuritis specific for lupus>

A

Nope.

29
Q

Pleural effusions in lupus are typically

A

small and bilateral

30
Q

Acute lupus pneumonitis

A

rare, actually a form of acute lung injury, can take form of diffuse hemorrhage

31
Q

Acute lupus pneumonitis symptoms

A

dyspnea, fever, cough

32
Q

Signs of acute lupus pneumonitis

A

fever, wet pulmonary crackles

33
Q

CT:

A

Ground Glass Opacification

34
Q

How do you diagnose acute lupus pneumonitis

A

SYmptoms, signs, CT findings, context (demographics + serology)

35
Q

Treatment for acute lupus pneumonitis

A

steroids + immunosuppresion

36
Q

Prognosis=

A

50% mortality

37
Q

Nonspecific interstitial pneumonia is —–

A

RARE as shit

38
Q

Pulmonary vascular disease with lupus presents as two types, they are:

A

Concentric arterial thickening or thromboembolic disease.

39
Q

Thromboembolic disease is associated with?

A

Lupus anticoagulant or antiphospholipid antibodies

40
Q

Shrinking Lung Syndrome. How common and what causes it?

A

Rare, caused by diaphragmatic weakness

41
Q

SHrinking lung syndrome features?

A

dyspnea, small lungs on x-ray, decreased diffusing capacity, restrictive pattern abnormalities, Usually self-limited

42
Q

What percentage of RA patients have pleuritic pain?

A

20%

43
Q

Findings suggestive of RA-related disease regarding interstitial pneumonia are?

A

Abundant lymphocytes and germinal centers

44
Q

Pleuropulmonary disease in patients with RA commonly involves what>

A

The bronchioles and even more commonly involves the pleura

45
Q

Interstitial lung disease in RA patients is typically most severe where?

A

Periphery of the lung and the bases

46
Q

Mucinous microcysts (honeycomb change) and Lymphoid Follicles suggest

A

Interstitial pneumonia in RA

47
Q

Follicular bronchiolitis in RA appears as

A

Peribronchiolar or centrilobular nodules in bronchocentricdistribution

48
Q

Follicular bronchiolitis in RA responds to

A

steroids

49
Q

Obliterative bronchiolitis does not respond to steroids

A

True

50
Q

Organizing pneumonia in RA

A

responds to steroids

51
Q

What is a recognized adverse effect of methotrexate regarding the lungs?

A

Pneumonitis. It is due to cytotoxicity causing diffuse alveolar infiltrates or hypersensitivity causing chronic interstitial pneumonia.

52
Q

What is the mortality rate in methotrexate toxicity?

A

20%….surprisingly high

53
Q

Methotrexate toxicity responds to what>

A

steroid therapy

54
Q

What is the presentation of methotrexate pulmonary toxicity

A

dyspnea, cough, fever

55
Q

MEthotrexate toxicity is typically subacute in what time period

A

up to 4 mths

56
Q

RA treatment using gold can cause

A

acute lung injury or chronic interstitial pneumonitis

57
Q

RA treatment using penicillamine can cause

A

alveolar hemorrhage

58
Q

Anti-tumor necrosis factor agents such as infliximab and etanercept may cause

A

interstitial pneumonia

59
Q

NSIP pattern is similar to UIP pattern except that NSIP pattern is distinguished by temmporal homogeneity…..all the lesions are at the same phase

A

ok…this applies mostly to the NSIP pattern pneumonia seen in Scleroderma

60
Q

Mass like lesion composed of tons of blue dots (lymphocytes) think…

A

Lymphoma

61
Q

What is the most commonly complicated by lymphoma

A

Sjrogen’s syndrome….KNOW

62
Q

Loss of submucosal gland secretion causing an unrelentless dry cough is seen most commonly in ?

A

Sjrogrens syndrome

63
Q

What makes Sjrogren’s associated NSIP special

A

It is different because it has lymphoid follicles

And it is bronchiolocentric.

64
Q

Sjogren’s NSIP is characterized b y an abundance of proliferating lymphocytes. These lymphocytes will inevitable make some mistakes and become…….or……… causing ……….or……….

A

semiautonomous or autonomous causing psuedo-lymphoma or lymphoma

65
Q

Malignant neoplastic lymphomas are how much more common in Sjogren’s patients

A

44 times

66
Q

Signs that a sjogren’s patient has lymphoma?

A

Mediastinal lymphadenopathy seen on CT, pleural effusion, prominent nodularity of lung lesions, lung cysts

67
Q

Rheumatology pts receiving immunosuppresives frequently get pulmonary infections. How do you distinguish these pulmonary infections from rheumatologic lung disease?

A

Rheumatologic lung disease is never acute. It is also very rarely nodular

68
Q

Dermatomysitis/ Polymyositis is associated with ?

A

Malignancy (12% of all myositis pts)

Also, respiratory failure from muscle weakness