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2017 ; 114
(20
): 354-361
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The Diagnosis and Treatment of Sjögren s Syndrome
#MMPMID28610655
Stefanski AL
; Tomiak C
; Pleyer U
; Dietrich T
; Burmester GR
; Dörner T
Dtsch Arztebl Int
2017[May]; 114
(20
): 354-361
PMID28610655
show ga
BACKGROUND: Sjögren's syndrome is one of the more common inflammatory
rheumatological diseases, with a prevalence of at least 0.4% in Germany. METHODS:
This review is based on pertinent articles retrieved by a selective search in
PubMed. Special attention is drawn to updated classification criteria and current
treatment recommendations. RESULTS: Sjögren's syndrome has a wide variety of
presentations, ranging from the local involvement of exocrine glands with
keratoconjunctivitis sicca and xerostomia (the leading signs of the disease) to
the systemic, extraglandular involvement of multiple organs. Fatigue also
markedly worsens the patients' quality of life. Serologic testing reveals
antinuclear auto-antibodies (anti-Ro/ SSA and anti-La/SSB) as well as rheumatoid
factors. The histological hallmark of the disease is focal lymphocytic
infiltration in otherwise normal-appearing glandular acini. The disease also
markedly elevates the risk of non-Hodgkin lymphoma of the B-cell series, which
arises in about 5% of patients. Primary Sjögren's syndrome (pSS) differs from the
secondary form (sSS), which appears in the setting of another autoimmune disease,
particularly systemic lupus erythematosus (15-36%), rheumatoid arthritis
(20-32%), and limited or progressive systemic sclerosis (11-24%).
Disease-modifying therapy is reserved for patients with systemic involvement;
there is limited evidence for its efficacy. Because of the complexity of this
disease, some of its clinical manifestations may require interdisciplinary
treatment. CONCLUSION: The main considerations in the interdisciplinary care of
patients with Sjögren's disease are measures to improve quality of life,
pharmacological and non-pharmacological treatments to keep disease activity in
check, and management of the risk of lymphoma. Future therapeutic approaches must
take the heterogeneity of the disease into account.
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