Systemic Sclerosis Flashcards
Systemic sclerosis is interchangeable with which term?
Scleroderma
Scleroderma translates directly to?
Hardening of the skin
What is systemic sclerosis?
an autoimmune inflammatory and fibrotic connective tissue disease. The cause of the condition is unclear. It most notably affects the skin in all areas but it also affects the internal organs.
What are the 2 main patterns of disease in systemic sclerosis?
Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis
What are the features of Limited Cutaneous Systemic Sclerosis?
Limited cutaneous systemic sclerosis is the more limited version of systemic sclerosis. It used to be called CREST syndrome. This forms a helpful mnemonic for remembering the features of limited cutaneous systemic sclerosis:
C – Calcinosis
R – Raynaud’s phenomenon
E – oEsophageal dysmotility
S – Sclerodactyly
T – Telangiectasia
What are the features of Diffuse Cutaneous Systemic Sclerosis?
Diffuse cutaneous systemic sclerosis includes the features of CREST syndrome plus many internal organs causing:
Cardiovascular problems, particularly hypertension and coronary artery disease.
Lung problems, particularly pulmonary hypertension and pulmonary fibrosis.
Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.
What is scleroderma?
Hardening of the skin
This gives a the appearance of shiny, tight skin without the normal folds in the skin. These changes are most notable on the hands and face.
What is Sclerodactyly?
describes the skin changes in the hands
As the skin tightens around joints it restricts the range of motion in the joint and reduces the function of the joints. As the skin hardens and tightens further the fat pads on the fingers are lost. The skin can break and ulcerate.
What is Telangiectasia?
dilated small blood vessels in the skin
They are tiny veins that have dilated
They have a fine, thready appearance
What is Calcinosis?
Where calcium deposits build up under the skin
This is most commonly found on the fingertips
What is Raynaud’s phenomenon?
where the fingertips go completely white and then blue in response to even mild cold
It is caused by vasoconstriction of the vessels supplying the fingers, this commonly occurs without any associated disease, however it is a classical feature of systemic sclerosis
Oesophageal dysmotility?
caused by connective tissue dysfunction in the oesophagus
This is commonly associated with swallowing difficulties, acid reflex and oesophagitis
Systemic and pulmonary hypertension is caused by?
connective tissue dysfunction in the systemic and pulmonary arterial systems
Systemic hypertension can be worsened by renal impairment
Pulmonary fibrosis?
can occur in severe systemic sclerosis
This presents with gradual onset dry cough and shortness of breath
what is Scleroderma renal crisis ?
an acute condition where there is a combination of severe hypertension and renal failure
Autoantibodies involved with Systemic Sclerosis?
many & helpful in predicting the extent of disease and which organs will be affected
Antinuclear antibodies (ANA) are positive in most patients with systemic sclerosis. They are not specific to systemic sclerosis.
Anti-centromere antibodies are most associated with limited cutaneous systemic sclerosis.
Anti-Scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis. They are associated with more severe disease.
What is Nailfold Capillaroscopy used for?
This is a technique where 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.
Diagnosis of Systemic Sclerosis?
Diagnosis is based on classification criteria from the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) published in 2013.
This involves meeting a number of criteria for clinical features, antibodies and nailfold capillaroscopy.
Non-medical management of Systemic Sclerosis involves?
Avoid smoking
Gentle skin stretching to maintain the range of motion
Regular emollients
Avoiding cold triggers for Raynaud’s
Physiotherapy to maintain healthy joints
Occupational therapy for adaptations to daily living to cope with limitations
Medical management of Systemic Sclerosis?
Nifedipine can be used to treat symptoms of Raynaud’s phenomenon
Anti acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
Analgesia for joint pain
Antibiotics for skin infections
Antihypertensives can be used to treat
hypertension (usually ACE inhibitors)
Treatment of pulmonary artery hypertension
Supportive management of pulmonary fibrosis
