Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Senior - Rhuem Chart > 5 - Systemic Sclerosis > Flashcards

5 - Systemic Sclerosis Flashcards

1
Q

Who gets systemic sclerosis?

A

Choctaw Indians

Black women >50yrs

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

Systemic sclerosis is aka?

A

Scleroderma

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

Scleroderma has a STRONG association w?

A

Raynaud’s phenomenon

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

What is scleroderma?

A

Heterogeneous rheumatic diseaese characterized by thickening and fibrosis of the skin with accompanying vascular lesions especially in lungs and kidneys

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

Strongest risk factor for scleroderma?

A

Family history

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

Besides fhx what else is linked to sclerosis?

A

Autoimmunity
CMV
Environmental (silica dust)

RAYNAUDS phenomenon

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

Types of sclerosis?

A

Systemic

  • limited
  • diffuse

Localized

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

S/S of systemic sclerosis?

A 
Skin thickening (puffy)
Carcinosis (PIP, fingertips)
Skin tightening
Digital flexion contracturs
Telangectasis
Vascular 
Cardiac (arrhthmias/pericardial effusions)
Pigmentation/depigmentation (vitiligo-like)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Steroids and systemic sclerosis?

A

Dont help and

Cause renal crisis

Treat with ACEIs

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

Sclerosis Most frequent symptoms?

A
  1. Raynaud phenomenon
  2. GERD w/ w/o dysmotility
    - skin changes
    - swollen fingers
    - arthralgias
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Labs for scleroderma?

A

ANA (95% sensitive)

Anti-SCL 70
Anticentromer AB (limited scleroderma)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Imaging/tests for systemic slcerosis?

A 
CSR
High res CT (GI)
EKG
Echo  (pulm HTN)
Stress testing 
PFTs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Why order PFT (scleroderma)

A

Baseline + q 4-12 mo
Looking for:
- reduction in DLCO
- reduction OOP to decline in FVC

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

Youre working up you systemic sclerosis pt and there is a nagging thin in your mind brain, what office test should you run?

A

Nailfold capillary evaluation

Bedside exam using ophthalmoscope to visualize nailfold capillaries

Normal: thin, linear, uniform
Scleroderma: capillary dropout and dilated capillary loops

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

What meds will help cure systemic sclerosis?

A

No drug will alter the natural course of the disease

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

What is the progression for the skin manifestations?

A

Reach peak involvement over first 18-24 months then gradually improve w or w/o therapy

17
Q

Immune system targeted therapy for systemic scleroderma?

A

Cyclophosphamide - pulmonary sxs

Don’t use
Corticosteroids - RENAL CRISIS

18
Q

Symptom specific tx with scleroderma?

Lots of info, not a great flash card

A

Raynauds: CCB
HTN/Renal crisis: ACEI
Intersitital lung disease: cyclophosphamide
Pulmonary HTN: endothelin receptor antagonist, PDE5 inhibitor
GERD: PPI
Delayed gastric emptying: metoclopramide/domperidone
Mild lower GI: loperamide/fiber
Small bowel hypermotility: ocreotide
Bacterial overgrowth: tetracycline/metronidazole

19
Q

Describe limited scleroderma?

A

Symmetrical skin thickening of :

  • fingers
  • distal arms
  • legs
  • face
  • neck
20
Q

Limited scleroderma onset post raynauds?

A

Slow progression after onset of raynauds

21
Q

Limited scleroderma is a later visceral disease but:

A

Digital ischemia and pulmonary HTN are more prominent

22
Q

Labs for limited scleroderma?

A

ANA (80-95%)
SCL-70 Pos (20%)
Anticentromere pos (50%)
- (HIGHLY SPECIFIC)

23
Q

What is crest syndrome?

A

A subtype of limited scleroderma

- invovles fingers distal to MCP

24
Q

WTF does CREST mean?

A 
C: calcinosis cutis
R: raynauds 
E: esophageal dysmotility
S: sclerodactyly
T: Telangectasias
25
Q

CREST syndrome labs

- hint its the same as limited scleroderma

A

ANA (80-95%)
SCL-70 Pos (20%)
Anticentromere pos (50%)
- (HIGHLY SPECIFIC)

26
Q

What is raynauds syndrome?

A

Episodic exaggerated vasospasm of the digital arteries in association with exposure to cold temp

Can be primary (idiopathic) or secondary

27
Q

raynauds is highly associated with?

A

Scleroderma (90%)

- may precede diagnosis by years

28
Q

Pts >30 with new onset raynauds?

A

ANA

Nailfold capillary exam

29
Q

Other raynauds warning signs (for scleroderma)

A
  • Severe painful episodes of vasospasms
  • Signs of digital ischemia
  • Tissue damage
  • Systemic s/s of 2ndary dx
30
Q

Tx for raynauds?

A

Avoid cold

CCB

31
Q

Diffuse scleroderma affects?

A

Same as limited

  • fingers
  • distal arms
  • distal legs
  • face
  • neck

+

  • trunk
  • proximal aspect of limbs
32
Q

Diffuse vs limited scleroderma?

A

Diffuse is worse

It has more significant visceral disease

33
Q

Limited is more prone to pulmonary HTN but diffuse is more prone to?

A

Renal crisis

Interstitial lung disease

Pulmonary fibrosis

34
Q

Other less common problems with diffuse?

A

Cardiac disease

  • pericarditis
  • myocardial fibrosis
  • heart failure
35
Q

Labs for diffuse scleroderma?

A

ANA
SCL-70 pos
Anticentromere NEG

Senior - Rhuem Chart (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions