RT Book, Section
A1 Moinzadeh, Pia
A1 Denton, Christopher P.
A1 Black, Carol M.
A1 Krieg, Thomas
A2 Kang, Sewon
A2 Amagai, Masayuki
A2 Bruckner, Anna L.
A2 Enk, Alexander H.
A2 Margolis, David J.
A2 McMichael, Amy J.
A2 Orringer, Jeffrey S.
SR Print(0)
ID 1162816818
T1 Systemic Sclerosis
T2 Fitzpatrick's Dermatology, 9e
YR 2019
FD 2019
PB McGraw-Hill Education
PP New York, NY
SN 9780071837798
LK dermatology.mhmedical.com/content.aspx?aid=1162816818
RD 2024/04/23
AB AT-A-GLANCEScleroderma  (systemic  sclerosis  [SSc])  is  a  multisystemic  autoimmune  disease  characterized  by  vasculopathy,  inflammation,  and  fibrosis  of  the  skin  and  many  other  organs.Differential  diagnosis  of  SSc  includes  severe  forms  of  localized  scleroderma,  as  well  as  many  other  scleroderma-like  conditions.Raynaud  phenomenon,  circulating  autoantibodies,  and  skin  sclerosis  are  almost  always  present  and  are  important  for  the  early  diagnosis.Patients  with  SSc  are  classified  into  2  major  subtypes  depending  on  the  extent  of  skin  sclerosis  (diffuse  cutaneous  systemic  sclerosis  and  limited  cutaneous  systemic  sclerosis).Patients  with  an  overlap  syndrome,  including  mixed  connective  tissue  disease,  are  characterized  by  additional  clinical  features  of  other  rheumatic  diseases.Involvement  of  internal  organs  (digestive  tract,  lung,  kidney,  and  heart)  can  lead  to  severe  dysfunction  and  determines  the  prognosis.The  heterogeneity  and  clinical  course  of  SSc  and  SSc  overlap  syndromes  require  the  urgent  need  of  interdisciplinary  collaborations  and  regular  followup  visits.Although  the  disease  is  still  not  curable,  there  have  been  substantial  advances  in  developing  new  therapeutic  approaches  and  treating  organ-based  complications  based  on  a  better  understanding  of  the  pathophysiology.