Systemic scleroderma
Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
ICD11: 4A42.Z
Last Updated: 2023-07-07
Author(s): Anzengruber F., Navarini A.
ICD11: 4A42.Z
PSS, Progressive Systemic Scleroderma, Systemic Sclerosis, Ssc, Diffuse Scleroderma.
An autoimmune disease belonging to the collagenoses, characterised by fibrosclerosis of the skin, subcutis and internal organs (vessels, lungs, intestine, skeleton, kidney, heart, liver).
Systemic manifestation
- Protection from the cold (gloves, hat, heated socks).
- Physiotherapy (no isometric exercises)/ massages/respiratory training.
- Stop smoking (vasoconstriction).
- Psychotherapy.
Topical therapy
- Re-greasing
- Light therapy
- Systemic therapy
- Combined with
- Azathioprine (Imurek®) p.o. 1x tgl.
- or with
- If therapy fails:
- Extracorporeal photopheresis
- Intravenous immunoglobulins (IVIG)
Symptomatic treatment
- Reflux oesophagitis:
- Vessels
- Lung:
Therapy of 1st choice according to Lebwohl |
Evidence level |
Nifedipine |
A |
Iloprost |
A |
ACE inhibitor |
B |
Therapy of the 2nd choice after Lebwohl |
|
Methotrexate |
A |
Cyclophosphamide |
B |
Prednisolone |
B |
Losartan |
B |
Acitretin |
C |
Colchicine (not commercially available in Switzerland) |
C |
UVA |
C |
Mycophenolate |
C |
We Value Your Privacy!
We and our partners are using technologies like cookies and process personal data like the IP-address or browser information in order to personalize the advertising that you see. This helps us to show you more relevant ads and improves your internet experience. We also use it in order to measure results or align our website content. Because we value your privacy, we are herewith asking for your permission to use these technologies. You can always change/withdraw your consent later.