Systemic Sclerosis

Question 1

define systemic sclerosis

Answer 1

An autoimmune connective tissue disorder involving inflammation and fibrosis of connective tissues, the skin, and organs.

Question 2

what causes sclerosis

Answer 2

Caused by excess collagen production that leads to thickening and tightening of skin

Question 3

is sclerosis more common in men or women

Answer 3

it is 4 times more commen in women

Question 4

what is scleroderma

Answer 4

relates to the hardening of the skin (it may be used interchangeably with systemic sclerosis)

Question 5

what is Limited Cutaneous Systemic Sclerosis (LCSS)

Answer 5

Also called CREST syndrome, this is a more limited version of systemic sclerosis.

Question 6

what does CREST refer to

Answer 6

Calcinosis
Raynaud’s phenomenon
oEsophageal dysmotility
Sclerodactyly
Telangiectasis

Question 7

what is Diffuse Cutaneous Systemic Sclerosis (DCSS)

Answer 7

A subtype that includes the CREST mnemonic plus cardiovascular, lung, and kidney problems.

Question 8

signs and symptoms of sclerosis

Answer 8

1. scleroderma (hardening of the skin, most notable on the hands and face)
2. Sclerodactyly (skin tightening around joints, loss of the fat pads on fingers, and ulceration/breaking of the skin surface).
3. Telangiectasia (dilated blood vessels in the skin, measuring less than 1mm in diameter)
4. Calcinosis (calcium deposits under the skin, especially on the fingertips)
5. Oesophageal dysmotility
6. Systemic and pulmonary hypertension (caused by connective tissue dysfunction)
7. Pulmonary fibrosis
8. Scleroderma renal crisis (medical emergency, with severe hypertension and renal failure)

Question 9

what is telangiectasia

Answer 9

dilated blood vessels in the skin, measuring less than 1mm in diameter)

Question 10

what is sclerodactyly

Answer 10

skin tightening around joints, loss of the fat pads on fingers, and ulceration/breaking of the skin surface).

Question 11

what is calcinosis

Answer 11

calcium deposits under the skin, especially on the fingertips)

Question 12

how to diagnose sclerosis

Answer 12

using the American College of Rheumatology/European League Against Rheumatism classification criteria.

These consider clinical features, antibodies, and nailfold capillaroscopy:

Question 13

which antibodies are present in sclerosis

Answer 13

1. ANAs present in most patients
2. Anti-centromere antibodies are most associated with LCSS
3. Anti-Scl-70 antibodies are most associated with DCSS and more severe disease.

Question 14

what is Nailfold Capillaroscopy

Answer 14

A test to examine the peripheral capillaries.

Question 15

how to treat sclerosis

Answer 15

no cure - treat the symptoms

Question 16

treatment options for sclerosis

Answer 16

DMARDs
Biological therapies
Steroids (but can cause further complications)