Universität zu Köln

Jerjen, Rebekka, Nikpour, Mandana, Krieg, Thomas, Denton, Christopher P. and Saracino, Amanda M. (2022). Systemic sclerosis in adults. Part II: management and therapeutics. J. Am. Acad. Dermatol., 87 (5). S. 957 - 979. NEW YORK: MOSBY-ELSEVIER. ISSN 1097-6787

Full text not available from this repository.

Abstract

The management of systemic sclerosis (SSc) is complex, evolving, and requires a multidisciplinary approach. At diagnosis and throughout the disease course, clinical assessment and monitoring of skin involvement is vital using the modified Rodnan Skin Score, patient-reported outcomes, and new global composite scores (such as the Combined Response Index for Systemic Sclerosis, which also considers lung function). Immunomodulation is the mainstay of skin fibrosis treatment, with mycophenolate mofetil considered first line. Meanwhile vasculopathy-related manifestations (Raynaud's phenomenon, digital ulcers) and calcinosis, require general measures combined with specific pharmacologic (calcium-channel blockers, phosphodiesterase type 5 inhibitors, and prostanoids), nonpharmacologic (digital sympathectomy and botulinum toxin injections), and often multifaceted, management approaches. Patients should be screened at the time of diagnosis specifically for systemic manifestations and then regularly thereafter, with appropriate treatment. Numerous targeted therapeutic options for SSc, including skin fibrosis, are emerging and include B-cell depletion, anti-interleukin 6, Janus kinase, and transforming growth factor beta inhibition. This second article in the continuing medical education series discusses these key aspects of SSc assessment and treatment, with particular focus on skin involvement. It is vital that dermatologists play a key role in the multidisciplinary approach to SSc management.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Jerjen, Rebekka UNSPECIFIED UNSPECIFIED UNSPECIFIED Nikpour, Mandana UNSPECIFIED UNSPECIFIED UNSPECIFIED Krieg, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Denton, Christopher P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Saracino, Amanda M. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-687456
DOI:	10.1016/j.jaad.2021.10.066
Journal or Publication Title:	J. Am. Acad. Dermatol.
Volume:	87
Number:	5
Page Range:	S. 957 - 979
Date:	2022
Publisher:	MOSBY-ELSEVIER
Place of Publication:	NEW YORK
ISSN:	1097-6787
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language STEM-CELL TRANSPLANTATION; RODNAN SKIN SCORE; HEALTH-ASSESSMENT QUESTIONNAIRE; INTENSE PULSED-LIGHT; LONG-TERM TREATMENT; BOTULINUM-TOXIN-A; RAYNAUDS-PHENOMENON RESISTANT; RECEPTOR ANTAGONIST BOSENTAN; STROMAL-VASCULAR FRACTION; FINGERTIP BLOOD PERFUSION Multiple languages Dermatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/68745