Autoimmune connective tissue diseases
Autoimmune connective tissue diseases include a wide range of conditions such as Systemic lupus erythematosus (SLE), Antiphospholipid syndrome (APS), Raynaud’s phenomenon, Sjorgren’s syndrome, and Systemic Sclerosis.
Lupus is an autoimmune condition, which means that the immune system, your body’s defence system, produces antibodies that attack the body’s own tissues, causing inflammation. There are two main forms of lupus:
- Discoid lupus: only affects the skin
- Systemic lupus erythematosus (SLE): affects the skin and joints and often also involves the internal organs including the heart or kidney
Antiphospholipid syndrome (APS)
Antiphospholipid syndrome (APS) can either be a primary condition or secondary to an underlying CTD such as Lupus.
APS is an autoimmune disorder where the blood tends to clot more quickly due to an excess of antiphospholipid antibodies. This condition is called ‘sticky blood’. The blood clots can affect any vessel and the most common presentation is a deep vein thrombosis (DVT) in the leg or a pulmonary embolus (PE) in the lung and can be associated with frequent miscarriage in women.
Raynaud’s phenomenon is condition resulting in colour change (white, blue, red) and pain in the hands and feet when exposed to changes in temperature or as a response to stress. This leads to episodes of decreased blood flow to the body’s extremities.
Raynaud’s commonly affects females and can affect all ages. It can be primary (no associated connective tissue disease (CTD)) or secondary to an underlying CTD such as SLE or Systemic Sclerosis.
Systemic Sclerosis (Scleroderma)
Systemic Sclerosis (Scleroderma) is a rare condition, characterized by widespread inflammation and fibrosis. The condition results in thickened skin (sclera = thick; derma =skin) and ranges from limited to diffuse and in rare cases can affect the lungs (pulmonary hypertension).
The limited form of the disease was previously known as CREST due to features including calcinosis (calcium deposits under skin), Raynaud’s phenomenon, oEsophageal involvement, Sclerodactyly (thickening of tendons in hands) and telangiectasia (small dilated blood vessels under the skin often in the face, hands and arms). In limited scleroderma, the skin involvement is confined to the hands, feet and face. In the diffuse form, the skin involvement can be over the entire body. Both conditions can be associated with internal organ involvement, particularly of the lungs, heart and gut.