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Systemic sclerosis: an unpredictable and rare disease

Systemic sclerosis: a rare disease that a lot of people probably have never heard of. It can disfigure and disable people and it is potentially fatal. Most people with systemic sclerosis experience thickening and hardening of the skin as well as pulmonary complications. So, what are the common symptoms and what does it mean to have systemic sclerosis?


Each patient’s experience of this disease is unique and symptoms can vary 

It is estimated that over two million people around the world have systemic sclerosis. It affects mostly women between twenty-five and fifty-five years of age. It is an auto-immune disease of the connective tissue, and is also referred to as scleroderma. Apart from the hardening of the skin, the term scleroderma literally means ‘hard skin’, it causes scarring of internal organs like the lungs, heart, kidneys and gut. But each patient’s experience of this disease is unique and symptoms can vary. This makes it a very difficult disease to recognize and diagnose early.

Although systemic sclerosis has its unique presentation in each patient, there are some common symptoms. Raynaud’s phenomenon for instance. This causes episodes of reduced blood flow to fingers. These episodes are often triggered by cold or emotional stress and makes the fingertips turn white and feel cold and numb, as no blood is reaching them. This can ultimately result in ulcers on the fingertips, which can be very painful and make it difficult for patients to use their hands.

Another common symptom of systemic sclerosis is interstitial lung disease (ILD). Patients with mild ILD may be asymptomatic during the early stages of the disease. But as the pulmonary fibrosis, the scarring of the lungs, gets worse they often feel more tired and experience difficulty breathing. When the doctor listens to the lungs of systemic sclerosis patients with ILD, in many cases a very specific dry breathing sound will be heard, called “velcro” crackles. A dry cough is also a common symptom in these patients. Unfortunately, ILD complications is the most common cause of death.

Systemic sclerosis is very unpredictable and the emotional and physical impact of this disease varies per patient and can even change from day to day. But if you have systemic sclerosis, there are several patient groups you can turn to. They can help you by explaining more about the disease and putting you in touch with specialists and regional centers of expertise.

Solomon JJ, Olson A L, Fischer A, et al. Scleroderma lung disease. Eur Respir Rev 2013;22:6-19.
University of Michigan Scleroderma Program. What is Scleroderma? Available at: www.med.umich.edu/scleroderma/patients/scleroderma.htm. Last accessed July 2017.
Scleroderma Foundation. What is scleroderma? Available at: www.scleroderma.org/site/PageNavigator/patients_whatis.html#.VhgSaPlViko. Last accessed July 2017.
University of Michigan Scleroderma Program. Lung Involvement. Available at: https://www.med.umich.edu/scleroderma/patients/lung.htm. Last accessed July 2017.
Vega CP, Solomon JJ, Brown KK, et al. Management of interstitial lung disease in systemic sclerosis. Int J Clin. Rheumatol 2011;6:503–15.