Understanding vascular involvement in systemic sclerosis

Date: Thu 22nd August 2019

The extent of vascular involvement experienced by someone living with systemic sclerosis (SSc) can have a huge impact on their medical journey, often affecting their prognosis. In her talk at SRUK's regional conference in Cambridge, Dr Robyn Domsic (based at UPMC and University of Pittsburgh Scleroderma Centre) discussed a variety of topics that are crucial to understanding vascular involvement and expert recommendations on management. Below is a concise summary of the main points covered by Dr Domsic.

The word 'vascular' refers to anything regarding the blood vessels. In the context of SSc therefore, vascular manifestations can be in the form of Raynaud's phenomenon, which occurs in over 95% of people living with SSc, digital ulcers, pulmonary hypertension, and renal crisis, which is thought to affect approximately 4% of those with SSc.

As many of you will be aware, Raynaud's phenomenon attacks can be brought on by cold exposure or emotional stress. It usually affects the 'glabrous' areas (areas of the skin without hair), such as the fingers and toes, nose, earlobe and tongue, and is chiefly characterised by distinctly marked colour changes. These colour changes occur in 3 phases: pallor (white), cyanosis (blue), and hyperemia (red). Numbness, tingling, discomfort and pain, especially as the skin turns red as blood flow returns, are generally reported. Critically, it can have a profound impact on quality of life due to the anxiety associated with the condition, for example when someone is going shopping for food, they may worry about how they will cope in the cold food section. In surveys conducted in people with SSc, Raynaud's is ranked amongst the highest as having the most detrimental impact on someone's life, second only to fatigue.

Furthermore, Raynaud's is a major cause of SSc-related morbidity and is a key contributor to microvascular complications such as digital ulcers and gangrene. These in turn affect someone's ability to work and socialize due to the debilitating nature of these symptoms. The predominant concern with digital ulcers appears to be the pain when the ulcer is touched, leading to challenges in performing daily activities such as cooking and dressing. Another reason of concern is the fact that ulcers are a source of infection, as someone with SSc is particularly susceptible as they are immunosuppressed. Wound care is a crucial aspect of ulcer treatment and is part of the non-pharmacological management approach that patients can be responsible for.

Pulmonary hypertension is another fairly common vascular manifestation associated with SSc, where there is high blood pressure in the lung blood vessels. Generally, this is due to thickening of these vessels, but it can also be a complication of pulmonary fibrosis or heart involvement. This too is a major contributor of SSc-related morbidity and it can be life-threatening if untreated, but modern treatments have improved significantly. This means that long-term management of this complication is possible.

Renal crisis presents itself as elevated blood pressure, swelling of the lower extremities, fatigue and shortness of breath. It too can be fatal, as it can progress to total renal failure in the absence of dialysis. Other treatments include the use of angiotensin-converting enzyme inhibitors and measures to manage high blood pressure, often alongside hospitalization; in many cases, the condition of a person who is suffering from renal crisis will improve.

Dr Domsic also discussed the pathology behind SSc-associated vascular disease. It was explained that the skin blood vessel system is organized in different ways according to the location, and that only a small amount of blood flow is necessary to maintain skin health. Most of the blood vessel flow in the skin to help maintain core body temperature – this is the thermoregulatory process. Things that can alter this process include ambient temperature, emotional state, physical activity, trauma to the vessels and age. A vasculopathy is an abnormal condition of the blood vessel, and this can be seen when a cross-section is taken of blood vessels in scleroderma patients. There is increased thickening and perforation of the inner most layer of the arteries, meaning the blood flow is already limited, and following exposure to the cold, there is further restriction to the blood flow. This leads to a severe vasospastic response.

Consistent monitoring is necessary to ensure that the best measures are taken to control vascular involvement. The different vascular manifestations require different approaches: for Raynaud's, history taking is better than a clinical exam, whereas for digital vasculopathy, examinations are needed to looking for ulcerations; for pulmonary hypertension, echocardiograms and pulmonary function tests have been shown to improve survival; for scleroderma renal crisis, blood pressure checks and kidney monitoring tests are critical.

First-line treatments for Raynaud's are calcium channel blockers (nifedipine, amlodipine, diltiazem) and angiotensin II receptor blockers (losartan). Other treatments that may be incorporated in certain cases are selective serotonin reuptake inhibitors (fluoxetine), alpha-blockers (prazosin) and statins. Phosphodiesterase type 5 inhibitors such as sildenafil (commonly known as Viagra) are being used increasingly for SSc-related Raynaud's, and intravenous prostanoids, such as iloprost, should be considered in severe cases. Dr Domsic also made reference to the non-pharmacological management of digital ulcers through wound care. A specialist nurse may be able to provide more precise advice for you, but it is important that when the ulcers are open with lost tissue (active ulcers) they should be kept moist and covered with non-adhesive dressings and non-adhesive tape. Vitamin E oil and MediHoney provide good non-prescription wound healing environments. When the ulcers are healing, they can be left open for extended periods of time. It was specified that during all stages, control of Raynaud's and avoidance of smoking are extremely important.

It was made apparent from Dr Domsic's talk that regular check-ups are essential in order to limit the extent of which SSc can affect someone's life, due to the likelihood of developing some form of vascular involvement. To watch the full talk, please head to our Facebook page. Summaries of the other talks will be posted over the next few weeks.

If you are interested in helping SRUK to fund work like this, then please visit our donations page. We rely on the generosity of our community to continue to support groundbreaking research in both scleroderma and Raynaud's.

If you would like information on scleroderma in different parts of your body, please visit: Scleroderma and your body

If you would like to attend our regional conference in Glasgow in October, then please register here: Glasgow Conference 2019

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