E-resources
-
Hachulla, Eric; Agard, Christian; Allanore, Yannick; Avouac, Jerome; Bader-Meunier, Brigitte; Belot, Alexandre; Berezne, Alice; Bouthors, Anne-Sophie; Condette-Wojtasik, Geraldine; Constans, Joël; De Groote, Pascal; Diot, Elisabeth; Dumas, Florence; Jego, Patrick; Joly, Francisca; Launay, David; Le Guern, Veronique; Le Quintrec, Janine-Sophie; Lescaille, Geraldine; Meune, Christophe; Moulin, Bruno; Nguyen, Christelle; Omeish, Nadine; Pene, Frederic; Richard, Marie-Aleth; Rochefort, Juliette; Roren, Alexandra; Sitbon, Olivier; Sobanski, Vincent; Truchetet, Marie-Elise; Mouthon, Luc; Bayen, Marc; Bergot, Emmanuel; Berthier, Sabine; Bosco, Julia; Bouhnik, Yoram; Chaigne, Benjamin; Cottin, Vincent; Crestani, Bruno; Deligny, Christophe; Descroix, Vianney; Farge, Dominique; Godard, Dominique; Granel, Brigitte; Guilpain, Philippe; Imbert, Bernard; Le Quellec, Alain; Lega, Christophe; Lok, Catherine; Maillard, Hélène; Martin, Thierry; Pugnet, Grégory; Queyrel, Viviane; Raffray, Loïc; Rilliard, Frédéric; Romier, Mélanie; Schuller, Laurence; Servettaz, Amélie
Orphanet journal of rare diseases, 07/2021, Volume: 16, Issue: S2Journal Article
Abstract Systemic sclerosis (SSc) is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. There are two main phenotypical forms of SSc: a diffuse cutaneous form that extends towards the proximal region of the limbs and/or torso, and a limited cutaneous form where the cutaneous sclerosis only affects the extremities of the limbs (without passing beyond the elbows and knees). There also exists in less than 10% of cases forms that never involve the skin. This is called SSc sine scleroderma. The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease (which is sometimes severe), pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Another type of involvement with poor prognosis, scleroderma renal crisis, is rare (less than 5% of cases). Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis.
Author
![loading ... loading ...](themes/default/img/ajax-loading.gif)
Shelf entry
Permalink
- URL:
Impact factor
Access to the JCR database is permitted only to users from Slovenia. Your current IP address is not on the list of IP addresses with access permission, and authentication with the relevant AAI accout is required.
Year | Impact factor | Edition | Category | Classification | ||||
---|---|---|---|---|---|---|---|---|
JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
Select the library membership card:
If the library membership card is not in the list,
add a new one.
DRS, in which the journal is indexed
Database name | Field | Year |
---|
Links to authors' personal bibliographies | Links to information on researchers in the SICRIS system |
---|
Source: Personal bibliographies
and: SICRIS
The material is available in full text. If you wish to order the material anyway, click the Continue button.