TY  - CHAP
M1  - Book, Section
TI  - Sjögren Syndrome
A1  - Tanikawa, Akiko
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.
PY  - 2019
T2  - Fitzpatrick's Dermatology, 9e
AB  - AT-A-GLANCEA  chronic,  multisystem  autoimmune  disease,  characterized  by  chronic  inflammation  involving  the  exocrine  glands.Salivary  and  lachrymal  glands  are  affected  predominantly,  leading  to  dry  mouth  and  dry  eyes.May  occur  alone  (primary  Sjögren  syndrome,  pSS),  or  may  coexist  with  other  systemic  connective  tissue  disorders  (secondary  Sjögren  syndrome).Extraglandular  manifestations  of  primary  Sjögren  syndrome  include  fatigue,  Raynaud  phenomenon,  purpura,  arthritis,  vasculitis,  interstitial  pulmonary  disease,  peripheral  or  central  neuropathy,  and  autonomic  nervous  dysfunction.Patients  with  systemic  manifestations  are  at  higher  risk  of  developing  lymphoma.Treatment  of  sicca  symptoms  is  mainly  symptomatic,  whereas  management  of  extraglandular  manifestations  is  similar  to  that  of  other  autoimmune  diseases.Both  innate  and  adaptive  immunity  contribute  to  pSS  pathogenesis  and  pSS  is  associated  with  upregulation  of  the  Type  1  interferon  signaling  pathway.  
SN  - 
PB  - McGraw-Hill Education
CY  - New York, NY
Y2  - 2024/04/19
UR  - dermatology.mhmedical.com/content.aspx?aid=1161333176
ER  -