Sjögren's syndrome (SjS) is the most common connective tissue disease with a prevalence of 1:200. Predominantly affecting women, SjS is associated with destruction of the exocrine glands, leading to xerophthalmia and xerostomia. In over 50% of patients, there are also extraglandular manifestations, leading to multiple organ manifestations including polyneuropathies and interstitial lung disease as well as symptoms such as fatigue and arthralgia. Diagnostic procedures include biomarkers, in particular anti-SS-A/Ro antibodies, histology of salivary glands, and salivary gland sonography. There are currently no licensed immunosuppressive drugs for SjS, so current treatment is often based on off-label use of drugs. The European League Against Rheumatism (EULAR) has recently published treatment recommendations based on the prevailing organ manifestations. Several promising controlled trials with novel compounds and concepts are currently in progress.
Keyphrases
- interstitial lung disease
- systemic sclerosis
- systematic review
- magnetic resonance imaging
- randomized controlled trial
- computed tomography
- end stage renal disease
- risk factors
- idiopathic pulmonary fibrosis
- adipose tissue
- metabolic syndrome
- magnetic resonance
- systemic lupus erythematosus
- skeletal muscle
- peritoneal dialysis