The terms systemic sclerosis and scleroderma are often used interchangeably. Most patients with scleroderma have systemic sclerosis, however there is a localised version of scleroderma that only affects the skin. Scleroderma translates directly to hardening of the skin.
Systemic sclerosis is 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.
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:
Systemic Sclerosis Presenting With Severe Digital Ischemia: A Rare Case Report Acharya S, Shukla S, Mahajan S N
Scleroderma refers to 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.
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.
Telangiectasia are dilated small blood vessels in the skin. They are tiny veins that have dilated. They have a fine, thready appearance.
Volume 98, Issue 4
Raynaud’s phenomenon is 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 systemic disease, however it is a classical feature of systemic sclerosis.
Oesophageal dysmotility is caused by connective tissue dysfunction in the oesophagus. This is commonly associated with swallowing difficulties, acid reflux 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.
Recommended New Documentary On Scleroderma
There are many autoantibodies involved in systemic sclerosis and they are helpful in predicting the extent of the disease and which organs will be affected. It is not worth memorising all of them unless you want to be a rheumatologist. The ones to remember are below.
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 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.
Sexual Dysfunction In Patients With Systemic Sclerosis
Steroids and immunosuppressants are usually started with diffuse disease and complications such as pulmonary fibrosis. There is no standardised and proven treatment for systemic sclerosis. There is ongoing research trying to find effective ways of treating the condition.
There are many autoantibodies involved in systemic sclerosis and they are helpful in predicting the extent of the disease and which organs will be affected. It is not worth memorising all of them unless you want to be a rheumatologist. The ones to remember are below.
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 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.
Sexual Dysfunction In Patients With Systemic Sclerosis
Steroids and immunosuppressants are usually started with diffuse disease and complications such as pulmonary fibrosis. There is no standardised and proven treatment for systemic sclerosis. There is ongoing research trying to find effective ways of treating the condition.
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