Systemic Sclerosis Flashcards
What is Systemic Sclerosis (SSc)?
Historically known as Scleroderma.
It is a multisystem autoimmune disease characterised by functional and structural abnormalities of small vessels, fibrosis of skin and internal organs and production of auto-antibodies.
Pathophysiology of SSc.
Increased fibroblast activity in abnormal growth of connective tissue which leads to vascular damage and fibrosis.
2 main subtypes of SSc.
Limited SSc
Diffuse SSc
Common symptoms and signs of limited SSc.
Involves mainly the face, hands and feet.
CREST
Calcinosis cutis
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia
Common symptoms and signs of diffuse SSc.
Can involve the whole body.
There is a sudden onset of skin involvement and is proximal to the elbows and knees.
Difference in presentation of limited SSc and diffuse SSc.
Limited usually starts with Raynaud’s many years in advance to any skin changes. Digital ischaemia might be seen.
In diffuse SSc there is an intial oedematous onset and the skin sclerosis will follow short afterwards. The Raynaud’s usually starts just before or concomitant with the oedema.
Multisystem involvement in SSc.
GI with heartburn, reflux or dysphagia
Renal with hypertensive renal crisis
Lung disease - fibrosis and pulmonary hypertension
Myocardial fibrosis
Vascular involvement in SSc.
Raynaud’s
Ischaemic digital ulcers
Hypertensive crisis
Pulmonary HTN
SSc with absence of Raynauds
Is very unlikely.
Reconsider diagnosis
When might SSc renal crisis be seen?
In early course of diffuse SSc.
Investigations of SSc.
FBC might show normochromic, normocytic anaemia
Serum crea and electrolytes if there is renal involvement.
Autoantibodies
Nailfold capillaroscopy
CXR
HRCT
PFT in case of pulmonary disease.
ECG, ECHO
Autoantibodies in limited SSc.
ANA positive in 90%
ANti-centromere antibody found in limited
Autoantibodies in diffuse SSc.
ANA in 90%
Scl-70 topoisomerase and anti RNA polymerase III in diffuse.
Treatment of SSc.
No cure
Psychological support.
CCB, sildenafil for Raynaud’s
MTX or Mycophenolate might reduce skin thickening
ACEi in hypertensive crisis
Prednisolone for flares
PPi for GI symptoms
Key takeaway of SSc.
CREST in limited
Diffuse has a more acute onset.
RAYNAUD’s
Explain nailfold capillaroscopy.
The area where the skin meets the fingernails at the base of the fingernail (the nailfold) is magnified and examined.
This allows us to examine the health of the peripheral capillaries.
Abnormal capillaries, avascular areas and micro-haemorrhages indicate systemic sclerosis.
It is useful to support a diagnosis of systemic sclerosis and to investigate patients with Raynaud’s phenomenon to exclude systemic sclerosis.
Patients with primary Raynaud’s without systemic sclerosis will have normal nailfold capillaries.
