Scleroderma Flashcards

1
Q

Typical presentation

A

Woman, presenting with Raynaud’s syndrome

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2
Q

Pathophysiology

A

Autoimmune systemic condition
Increased fibroblast activity
Abnormal connective tissue growth
Vascular damage & fibrosis

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3
Q

Subtypes

A

Limited SSC
Diffuse SSC

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4
Q

Diffuse SSC features

A

Less common
Increased mortality
Sudden onset skin changes
Proximal joint involvement. (Knees, Elbows)

HTN -> Renal Failure

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5
Q

Limited SSC Features

A

More common

Calcinosis Cutis
Raynaud’s Syndrome
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Pulmonary HTN

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6
Q

Investigations Limited SSC

A

ANA Positive
Anti Centromere Antibody

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7
Q

Investigations diffuse SSC

A

Topoisomerase antibody
Anti RNA Polymerase III Antibody

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8
Q

General investigations

A

CXR - Pulmonary disease
Joint XR - Calcinosis Cutis
Echo/ ECG - Arrhythmia, HF, Myocarditis
CRP/ESR/PV - Normal

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9
Q

Treatment

A

NO CURE

  1. Psychosocial
  2. PDE 5 (Sildenafil)
  3. Ace inhibitors (Blood pressure, Renal protection)
  4. Methotrexate, Mycophenolate Mofetil (Skin Thickening)
  5. Prednisolone (Acute flares)
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