RT Book, Section
A1 Ramos-Casals, Manuel
A1 Brito-Zerón, Pilar
A1 Sisó-Almirall, Antoni
A2 Stone, John H.
SR Print(0)
ID 1180196959
T1 Primary Sjögren Syndrome
T2 Current Diagnosis & Treatment: Rheumatology, 4e
YR 2021
FD 2021
PB McGraw Hill
PP New York, NY
SN 9781259644641
LK accessmedicine.mhmedical.com/content.aspx?aid=1180196959
RD 2024/04/19
AB Sjögren  syndrome  (SjS)  is  a  systemic  autoimmune  disease  that  presents  with  sicca  symptomatology  of  mucosal  surfaces.The  main  sicca  features  (xerophthalmia  and  xerostomia)  are  determined  by  specific  ocular  tests  (corneal  stainings  and  Schirmer  test)  and  oral  investigations  (salivary  flow  measurement  and  parotid  scintigraphy).The  histologic  hallmark  is  a  focal  lymphocytic  infiltration  of  the  exocrine  glands,  determined  by  a  biopsy  of  the  minor  labial  salivary  glands.The  spectrum  of  the  disease  includes  systemic  features  (extraglandular  manifestations)  in  some  patients,  and  is  complicated  by  the  development  of  lymphoma  in  a  small  percentage  of  patients.Patients  with  SjS  can  be  characterized  by  a  broad  spectrum  of  laboratory  features  (cytopenias,  hypergammaglobulinemia,  and  high  erythrocyte  sedimentation  rate)  and  autoantibodies,  of  which  antinuclear  antibodies  are  the  most  frequently  detected,  anti-Ro/SS-A  the  most  specific,  and  cryoglobulins  and  hypocomplementemia  the  main  prognostic  markers.