Systemic sclerosis

Systemic sclerosis is a rare systemic autoimmune disease which is driven by inflammation, changes of the small vessels and an increasing deposition of extracellular matrix proteins. Typically involved organs include the skin, the lungs, the heart, the gastrointestinal tract, the muscles and the joints. Systemic sclerosis shows a highly heterogeneous course of the disease ranging from mild subclinical involvement to severe life threatening organ involvement.

Symptoms

Raynaud’s phenomenon, which is a typical color change of the fingers upon cold exposure, is the most frequent presenting symptom. Raynaud’s phenomenon can occur up to many years before onset of the disease. The depositions of extracellular matrix proteins lead to fibrosis of the skin. Skin fibrosis always starts at the fingers and can involve other parts of the body during the disease course. Swelling of the fingers (puffy fingers) is another frequent early manifestation of systemic sclerosis. In addition joint and tendon inflammation frequently occurs.

Involvement of the lungs can occur in all forms of systemic sclerosis and is important for the prognosis of the disease. Lung involvement can consist of lung fibrosis and/or pulmonary hypertension. Early recognition of lung involvement is important, if possible even in subclinical stages, to initiate early specific treatments. 

Diagnostics

Due to the high heterogeneity of the diseases, the diagnostic tests have to be specifically tailored for the individual patient. An extended organ screening is recommended at a diagnosis and regularly, e.g. annually, afterwards. 

Interdisciplinary team for the sclerodermia-assessment at the clinic for rheumatology and physiotherapy/occupational therapy USZ

Inpatient Systemic Sclerosis Assessment

This is a four-day inpatient assessment program with close interaction with other medical disciplines, occupational therapists, physical therapists, as well as nurse and wound experts. This inpatient assessment has received quality awards and is most adequate for patients with a new diagnosis and with severe organ involvements. 

Outpatient Scleroderma Assessment

In this program, the annual organ screening examinations are performed within one day. The standard examinations include echocardiography, lung function tests, 6-minute-walk tests, nailfold capillaroscopy, computertomography of the lungs and lab examinations. Individual additional assessments are added. Finally, the results of the one day examinations are discussed with the specialized rheumatologist and a therapy plan is developed together with the patient. This outpatient assessment program is adequate for all patients with systemic sclerosis including early subclinical forms.

Therapy

Resent years have shown significant progress in the treatment options for patients with systemic sclerosis. While there is still no treatment available for the overall disease, specific organ manifestations have several treatment options available. These various treatment options have improved overall survival and quality of life of patients with systemic sclerosis. 

Cooperations

Because systemic sclerosis is a systemic multi-organ disease, we have close collaborations with various other departments at the university hospital, including but not limited to physiotherapy/occupational therapy, pulmonology, cardiology, radiology and clinical immunology.

The scleroderma program at the University Hospital Zurich is one of the internationally leading scleroderma research centers. It has research programs both in clinical research as well as in basic science research.