Systemic scleroderma
Last Updated: 2021-11-19
ICD11: 4A42.Z
Last Updated: 2021-11-19
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 |
Share your experience and shape the future with us!
Do you have 5 minutes to take our short survey?
Together with you we would like to further develop DermaCompass.
There is the chance to win one of 3x50 EUR Amazon vouchers.
Be part of our innovation process!
Share your experience