Systemic Sclerosis/Scleroderma

Question 1

Scleroderma What

Answer 1

Hardening of the skin

Question 2

Scleroderma Epidemiology

Answer 2

0.6-1.9/million/y
4:1 f:m
5th decade

Question 3

Scleroderma Pathology

Answer 3

Over active fibroblast -> fibrosis
Peri vascular inflammation and infiltration -> artery narrowing
Immune activation

Question 4

Clinical features skin Scleroderma

Answer 4

Inflam phase-> puffy, tight, itchy skin-> forearms p, hands and feet
Skin thickening and induration over months
Sclerodactyl-> tense, pale skin on fingers
Microstomia-> tightening around the mouth and furrowing
Loss of normal skin creases
Tethering
Flexion contracture

Question 5

Limited scleroderma

Answer 5

Calcinosis
Raynauds
Oesophageal disease
Sclerodactyl
Telangectasia 

CREST

Hands, feet, face

Question 6

Diffuse scleroderma

Answer 6

Limbs, face, trunk
Swelling
Pruritis

Question 7

Cardiovascular symptoms Scleroderma

Answer 7

Raynauds
Myocardial fibrosis
Pericarditis

-> diuretics and ACEI

Question 8

Scleroderma Pulmonary symptoms

Answer 8

25% interstitial lung disease

10-12% pulmonary hypertension

Question 9

Scleroderma Renal symptoms

Answer 9

Scleroderma renal crisis

-> real failure with accelerated hypertension

Question 10

Scleroderma GI symptoms

Answer 10

Reflux oesophagitis

Oesophageal dismotility

Question 11

Scleroderma Musculoskeletal symptoms

Answer 11

Arthralgia
Joint stiffness
Flexion contracture

Question 12

Scleroderma Neurological symptoms

Answer 12

Central and peripheral neuropathy

Question 13

Scleroderma Investigations

Answer 13

Serology
Anticentromere-> limited disease-> good prognosis
Antioplisomeroses-> diffuse disease-> bad prognosis

Question 14

Scleroderma Management

Answer 14

Screen for complications