Sjogren syndrome

Sialographie

bei Verdacht auf Sjögren-Syndrom

Metabolic and

storage lung diseases: spectrum of imaging appearances. A man with Sjögren syndrome and amyloidosis. HRCT image shows multiple cysts (arrows) in both lower lobes, scattered nodules (arrowheads), and right lower lobe consolidation. The appearance is similar to that of lymphoid interstitial pneumonia, and the two processes may coexist in the setting of Sjögren syndrome

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Sjögren

syndrome • Sjögren syndrome - Ganzer Fall bei Radiopaedia

Schubert, R. Sjögren syndrome. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-17882 • CC by-nc-sa 3.0 (modified)

Sjögren

syndrome • Sjogren syndrome - Ganzer Fall bei Radiopaedia

Hennessy, O. Sjogren syndrome. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-33286 • CC by-nc-sa 3.0 (modified)

Sjögren

syndrome • Sjogrens disease - Ganzer Fall bei Radiopaedia

Bickle, I. Sjogrens disease. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-50847 • CC by-nc-sa 3.0 (modified)

Sjögren

syndrome • Sjögren syndrome - Ganzer Fall bei Radiopaedia

Haouimi, A. Sjögren syndrome. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-67792 • CC by-nc-sa 3.0 (modified)

Sjögren

syndrome • Sjogren's disease involvement of the parotid gland - Ganzer Fall bei Radiopaedia

spaces, H. Sjogren's disease involvement of the parotid gland. Case study, Radiopaedia.org. (accessed on 22 Oct 2022) https://doi.org/10.53347/rID-28389 • CC by-nc-sa 3.0 (modified)

Lacrimal

gland masses • Sjögren syndrome - lacrimal glands - Ganzer Fall bei Radiopaedia

Gaillard, F. Sjögren syndrome - lacrimal glands. Case study, Radiopaedia.org. (accessed on 21 Oct 2022) https://doi.org/10.53347/rID-9153 • CC by-nc-sa 3.0 (modified)

Radiopaedia • CC-by-nc-sa 3.0 • de

Sjögren syndrome, or Sjögren disease, is an autoimmune condition of the exocrine glands that produce tears and saliva.

Epidemiology

Sjögren syndrome is the second most common autoimmune disorder after rheumatoid arthritis. There is a recognized female predilection with F:M ratio of ≈9:1. Patients typically present around the 4to 5 decades.

Clinical presentation

Clinically, the cardinal features are glandular:

keratoconjunctivitis sicca (xerophthalmia)
dryness of the mucous membrane of the mouth (xerostomia)
bilateral parotid enlargement

Additionally, there may be multi-organ extra-glandular involvement, which can have a wide variety of clinical features.

Pathology

It is a chronic autoimmune disorder involving mainly the salivary and lacrimal glands and is associated with hyperactivity of the B-lymphocytes and with autoantibody and immune complex production.

Associations

Approximately 40% of cases occur in isolation. Known associations include:

thoracic changes associated with Sjögren syndrome
- non-specific interstitial pneumonitis (NSIP): considered the most common pattern
- usual interstitial pneumonitis
- lymphoid interstitial pneumonia (LIP)
- focal lymphoid hyperplasia of the lung
- small airways disease
- pulmonary amyloidosis: AL type
Mikulicz syndrome (sometimes known as Sjögren type 1 )
other connective tissue diseases
EBV infection
Hepatitis C infection
primary biliary cirrhosis (possible )
HTLV infection

Markers

anti-Ro (SSA) antibody
anti-La (SSB) antibody

Radiographic features

Ultrasound

Salivary glands

early stage: the gland can be normal or become enlarged and hyperechoic
late stages: may characteristically show a multicystic or reticular pattern within an atrophic gland

MRI

Salivary glands

Parotid gland involvement may give a salt and pepper appearance or a honeycomb appearance. A change in the size of the lacrimal glands associated with accelerated fat deposition may also be seen .

Treatment and prognosis

Complications

One of the complications of this syndrome is the development of malignant lymphoma.

History and etymology

This condition is named after Swedish ophthalmologist Henrik Sjögren (1899-1986) although it was first described by WB Hadden and JW Hutchinson in 1871 .