TY  - CHAP
M1  - Book, Section
TI  - Scleroderma (Systemic Sclerosis)
A1  - Mandal, Jennifer
A1  - Boin, Francesco
A2  - Stone, John H.
Y1  - 2021
N1  - 
T2  - Current Diagnosis & Treatment: Rheumatology, 4e
AB  - Scleroderma  (systemic  sclerosis)  is  a  systemic  autoimmune  disease  characterized  by  varying  degrees  of  skin  fibrosis,  vascular  damage,  and  a  wide  array  of  internal  organ  dysfunction.Most  manifestations  of  scleroderma  fall  into  two  main  categories:  fibrotic  disease  (such  as  skin  tightening  and  interstitial  lung  disease  [ILD])  and  vascular  disease  (such  as  Raynaud  phenomenon  [RP]  and  pulmonary  arterial  hypertension  [PAH]).In  limited  cutaneous  scleroderma,  skin  involvement  remains  confined  to  the  distal  extremities  (fingers,  toes)  and  face.  In  contrast,  in  the  diffuse  form  of  scleroderma,  skin  tightening  extends  proximal  to  the  elbows  and  knees  as  well  as  the  trunk.  Both  limited  and  diffuse  scleroderma  can  have  significant  internal  organ  involvement.RP  and  antinuclear  antibodies  (ANA)  positivity  are  each  present  in  more  than  95%  of  scleroderma  patients.  When  either  of  these  features  is  missing,  an  alternative  diagnosis  should  be  strongly  considered.Some  patients  with  scleroderma  develop  clinical  features  overlapping  with  other  rheumatic  disorders  such  as  rheumatoid  arthritis,  systemic  lupus  erythematosus,  inflammatory  myopathy,  and  Sjögren  syndrome.It  is  helpful  to  take  an  organized  organ-by-organ  approach  to  assess  an  individual  patient’s  specific  manifestations,  disease  activity,  extent  of  damage,  and  treatment  options.There  is  no  “universal”  pharmacologic  intervention  for  scleroderma  that  treats  the  disease  as  a  whole.  Combination  therapy  is  most  effective  to  address  the  heterogeneous  nature  of  this  disease  manifestation.  
SN  - 
PB  - McGraw Hill
CY  - New York, NY
Y2  - 2024/04/16
UR  - accessmedicine.mhmedical.com/content.aspx?aid=1180196883
ER  -