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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).

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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.

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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.
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4
Q

What is Limited Cutaneous Systemic Sclerosis?

A

CREST Syndrome - limited version of systemic sclerosis.

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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.
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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.
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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.

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8
Q

What is telangiectasia?

A

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

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9
Q

What is Calcinosis?

A

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

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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.
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11
Q

What is Oesophageal Dysmotility caused by?

A

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

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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.

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13
Q

How does pulmonary fibrosis manifest in systemic sclerosis?

A

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

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14
Q

What is Scleroderma Renal Crisis?

A

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

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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.
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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)

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