Brainscape's Knowledge GenomeTM

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


See full index

Rheumatology YR3 > Systemic Sclerosis > Flashcards

Systemic Sclerosis Flashcards

1
Q

What is systemic sclerosis?

A
  • Multisystem autoimmune disease
  • Previously known as scleroderma
  • Increased fibroblast activity = abnormal growth of connective tissue which leads to vascualr damage and fibrosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

2 subtypes of systemic sclerosis

A
  • Limited SSc
  • Diffuse SSc
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Limited scleroderma aka CREST syndrome signs/symptoms

A
  • Calcinosis - deposits in tips of fingers –> then cause ulcers/infection
  • Raynaud’s
  • oEsophageal dysfunction
  • Sclerodactyl - tight shiny skin
  • Telangiectasia - dilated capillaries (spider naevi)

Also cannot forget pulmonary hypertension

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

CREST syndrome presentation

A
  • Many years of Raynaud’s symptoms before any scleroderma
  • Development of pulmonary hypertension after 10 yrs of symptoms
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Diffuse scleroderma - more/less common?

A

Less common
Higher risk mortality

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

Diffuse scleroderma presentation

A

Sudden onset of skin involvement
Proximal to elbows and knees

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

Investigations for systemic sclerosis

A
  • Inflammatory markers normal
  • X-ray hands - calcinosis
  • CXR
  • HRCT
  • PFT - pulmonary disease
  • ECG and ECHO - pulmonary hypertension? heart failure, myocarditis, arrhythmias
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Antibodies in systemic sclerosis

A
  • Positive ANA 90%
  • Anti centromere antibody - limited SSc
  • Scl-70 (topisomerase) and anti RNA polymerase III antibodies associated with diffuse
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Main thing to look at in PFTs

A
  • Restrictive lung pattern - fibrosis
  • Transfer factor - DLCO decreases
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Treatment for systemic sclerosis

A
  • No cure
  • Psychological support
  • Calcium antagonist/sildenafil/iloprost infusion for Raynauds
  • Methotrexate and mycophenolate mofetil may reduce thickening of skin
  • Prednisolone for flares
  • ACEi prevent HTN crisis and reduce mortality from renal failure
  • PPI for GI symptoms
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Scleroderma M:F

A

5 times more common in women

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

What is localised scleroderma called?

A

Morphoea

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

Consequences of vascular involvement of systemic sclerosis

A
  • Raynauds
  • Ischaemic digital ulcers
  • Hypertensive crisis
  • Pulmonary arterial hypertension
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What makes systemic sclerosis diagnosis unlikely?

A

Absence of Raynauds

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

What is scleroderma renal crisis?

A
  • Accelerated HTN
  • Lead to renal failure if not treated quick
  • Seen early in diffuse scleroderma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the changes seen in Raynaud’s and why?

A
  • White - ischaemia
  • Blue - venous stasis
  • Red - reactive hyperaemia

Pain occurs when blood flow returns, may also get parasthesia

17
Q

Effects on heart of systemic sclerosis

A
  • Restrictive pericarditis - fibrous pericardium stiff
  • Heart block - from fibrotic tissue
18
Q

Effects on GI from systemic sclerosis

A
  • Malabsorption
  • Reflux
  • Oesophageal dysfunction
  • Gastroparesis
  • Pseudobstruction
19
Q

Sexual effects of SS

A
  • Erectile dysfunction
20
Q

Why do we do echo in SS?

A
  • Asses for cor pulmonale
  • Pulmonary HTN –> cor pulmonale
21
Q

Treatment for renal crisis

A

ACEi - Captopril - onset 60-90mins
Transplant

22
Q

What is renal crisis?

A

AKI due to impaired blood flow to kidney from fibrosis

23
Q

RF for renal crisis

A
  • Diffuse disease
  • Steroids
  • Anti RNA polymerase
  • Anticentromere
24
Q

Presentation of renal crisis

A
  • Malignant HTN
  • Encephalopathy
  • AKI
  • Urinalysis normal
  • Low platelets - MAHA (becomes hard to distinguish between HUS and TTP)
25
Q

Treatment for calcinosis of fingers if ulcerated/infected

A

Minocycline

26
Q

What can be used for telangiectasia?

A

Laser therapy

27
Q

Treatment for itchy skin

A
  • Emolients
  • Capsaicin
  • Menthol
28
Q

Treatment for pulmonary HTN

A
  • Bosentan
  • Tadalafil
29
Q
A

Rheumatology YR3 (18 decks)

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