Brainscape's Knowledge GenomeTM

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


See full index

RHEUM (Rheumatology) > Systemic Sclerosis (Scleroderma) > Flashcards

Systemic Sclerosis (Scleroderma) Flashcards

1
Q

What is Scleroderma?

A

Hardening of the skin - giving it a shiny tight appearance without normal skin folds (hands and face).

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

What is Systemic Sclerosis?

Epidemiology of Systemic Sclerosis.

A

Autoimmune inflammatory and fibrotic connective tissue disease, most common in women between 30-50.

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

Distribution of Systemic Sclerosis (2).

A
  1. Most notably affects the skin in all areas but also affects the internal organs.
  2. 2 Main Patterns : Limited Cutaneous and Diffuse Cutaneous.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is Limited Cutaneous Systemic Sclerosis?

A

CREST Syndrome - limited version of systemic sclerosis.

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

What are the features of systemic sclerosis? (5)

A

CREST :

  1. C - Calcinosis.
  2. R - Raynaud’s phenomenon.
  3. E - Oesophageal Dysmotility.
  4. S - Sclerodactyly.
  5. T - Telangiectasia.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the features of Diffuse Cutaneous Systemic Sclerosis? (4)

A
  1. CREST.
  2. CVS : Hypertension and Coronary Artery Disease.
  3. LUNG : Pulmonary Hypertension and Pulmonary Fibrosis.
  4. RENAL : Glomerulonephritis and Scleroderma Renal Crisis.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is Sclerodactyly?

A

Skin changes in hands - skin tightens around joints; restricted range of motion and reduced function. As skin hardens and tightens further, the fat pads are lost and the skin can break and ulcerate.

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

What is telangiectasia?

A

Dilated small blood vessels in the skin - tiny veins with a fine, thready appearance.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is Calcinosis?

A

Calcium deposits build up under the skin (commonly : fingertips).

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

What is Raynaud’s phenomenon? (2)

A
  1. Fingertips go completely white and then blue in response to even mild cold - due to vasoconstriction (can be primary (Disease) or secondary (Phenomenon)).
  2. Commoner in young women (30) with bilateral symptoms.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is Oesophageal Dysmotility caused by?

A

Connective tissue dysfunction in the oesophagus - commonly associated with dysphagia, acid reflux and oesophagitis.

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

What is systemic and pulmonary hypertension caused by in systemic sclerosis?

A

Connective tissue dysfunction in the systemic and pulmonary arterial systems - systemic is worsened by renal impairment.

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

How does pulmonary fibrosis manifest in systemic sclerosis?

A

Severe cases - gradual onset, dry cough, shortness of breath.

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

What is Scleroderma Renal Crisis?

A

An acute condition where there is a combination of severe hypertension and renal failure.

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

Differential Diagnoses of Raynaud’s phenomenon (6).

A
  1. Connective Tissue Disorders (SS, RA, SLE).
  2. Leukaemia.
  3. Type I Cryoglobulinaemia, Cold Agglutinins.
  4. Use of Vibrating Tools.
  5. Drugs : OCP, Ergot.
  6. Cervical Rib.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Pulmonary Features in Systemic Sclerosis (2).

A
  1. Limited : Pulmonary Hypertension.

2. Diffuse : Pulmonary Fibrosis.

17
Q

Diagnosis of Systemic Sclerosis.

A

Based on clinical presentation, antibodies, nail fold capillaroscopy.

18
Q

Antibodies in Systemic Sclerosis.

A
  1. ANA Positive.
  2. Anti-Centromere : Limited.
  3. Anti-SCL-70 : Diffuse (severe disease).
19
Q

What is Nailfold Capillaroscopy?

A

Magnification and examination of the area where the skin meets the fingernails at the base of the nail (nail fold) to examine peripheral capillaries.

20
Q

What signs in Nailfold Capillaroscopy indicate systemic sclerosis? (3)

A
  1. Abnormal Capillaries.
  2. Avascular Areas.
  3. Micro-Haemorrhages.
21
Q

Why is Nailfold Capillaroscopy useful to distinguish the types of Raynaud’s phenomenon?

A

Primary - Normal Nailfold Capillaries.

22
Q

Management of Systemic Sclerosis (3).

A
  1. Specialist MDT.
  2. Steroids + Immunosuppressants - Diffuse Disease and Complications.
  3. No Specific Treatment.
23
Q

Non-Medical Management of Systemic Sclerosis (3).

A
  1. Smoking Cessation.
  2. Gentle Skin Stretching + Regular Emollients + Avoiding Cold Triggers.
  3. Physiotherapy and Occupational Therapy.
24
Q

Medical Management of Systemic Sclerosis (5).

A
  1. Nifedipine - Raynaud’s.
  2. PPIs + Metoclopramide - GI Symptoms.
  3. Analgesia - Joint Pain.
  4. Antibiotics - Skin Infections.
  5. Antihypertensives - Hypertension.
25
Q

Management of Raynaud’s phenomenon (2).

A
  1. 1st Line - CCBs e.g. Nifedipine.

2. IV Prostacyclin (Epoprostenol) Infusions (lasting weeks/months).

26
Q

Prognosis of Systemic Sclerosis.

A

Diffuse - Death : Respiratory Involvement e.g. ILD or Pulmonary Arterial Hypertension (diagnosed using right heart catheterisation).

RHEUM (Rheumatology) (15 decks)

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