25th February 2025

Sjögren’s syndrome

Overview of Sjögren’s syndrome

Sjögren’s syndrome is an autoimmune disorder where the predominant pathophysiological feature is lymphocytic infiltration of exocrine glands. Clinical manifestations can vary from sicca symptoms to systemic involvement. Sjögrens can be primary, where it is an isolated phenomenon, or secondary, where it is associated with another autoimmune condition, such as rheumatoid arthritis, SLE or primary biliary cirrhosis.

It is most common in females, with onset in middle age, and has a similar prevalence to rheumatoid arthritis of 1%.

Clinical features of Sjögren’s syndrome

Mucosal dryness is the most characteristic clinical feature – involving the eyes, oropharynx, nose and vagina. These are known as sicca symptoms. There can be extraglandular manifestations, such as arthritis, cutaneous vasculitis, peripheral neuropathy and autoimmune hepatitis. Fatigue is very common and often the most troubling symptom.

There is also an increased risk of lymphoid malignancy, the strongest association being with MALT lymphoma (a non-Hodgkin’s lymphoma).

Investigations in Sjögren’s syndrome

Over 70% have a positive ANA, and anti-Ro (SS-A) and La (SS-B) antibodies are common, with anti-Ro being the most specific. Rheumatoid factor can be positive, and patients can be misdiagnosed as having rheumatoid arthritis.

Schirmer’s test can be performed in clinic: a piece of filter paper is slipped under the eyelid and left for 5 minutes; if <5 mm of the paper is wet after 5 minutes, this suggests impaired tear secretion. Other investigations include measurement of saliva production and labial gland biopsy, which can help in diagnosis of antibody-negative patients.

Management of Sjögren’s syndrome

Treatment depends on the patient’s symptoms. Artificial tears and saliva can be useful for xerophthalmia and xerostomia, as well as close contact with opticians and dentists. Advice regarding lubricants can be given for vaginal dryness. Regular pilocarpine can also help with sicca symptoms.

Hydroxychloroquine is the most common DMARD (disease-modifying antirheumatic drug) used and can be particularly helpful for joint symptoms; evidence of other benefits is limited. With more severe features such as vasculitis or ILD, systemic immunosuppression may be required, such as glucocorticoids or cyclophosphamide.

Patients who are Ro or La positive should be carefully monitored during pregnancy due to the risk of development of foetal heart block. These patients will have regular ultrasounds throughout pregnancy to detect this at an early stage, in order to prepare for potential pacemaker insertion after birth. Babies born to Ro- or La-positive mothers can also exhibit a photosensitive skin rash due to passive transfer of antibodies across the placenta, called cutaneous neonatal lupus erythematosus.

References

• Price EJ, Rauz S, Tappuni AR, Sutcliffe N, Hackett KL, Barone F, et al. The British Society for Rheumatology guideline for the management of adults with primary Sjögren’s Syndrome. Rheumatology. 2017 Jun 28;56(10)
• Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, et al. Sjögren syndrome. Nature Reviews Disease Primers [Internet]. 2016 Jul 7 [cited 2022 Jan 12];2(1):1–20. Available from: https://www.nature.com/articles/nrdp201647
• Upala S, Yong WC, Sanguankeo A. Association between primary Sjögren’s syndrome and pregnancy complications: a systematic review and meta-analysis. Clinical Rheumatology. 2016 Jun 8;35(8):1949–55
• Clunie GPR, Wilkinson N, Nikiphorou E, Deepak Jadon. Oxford handbook of rheumatology. Oxford: Oxford University Press; 2018
• Baer A. Diagnosis and classification of Sjögren’s syndrome [Internet]. Fox R, Romain P, editors. UpToDate. 2021 [cited 2022 Feb 19]. Available from: https://www.uptodate.com/contents/diagnosis-and-classification-of-sjogrens-syndrome