Clinical features of Sjögren syndrome
Sjögren syndrome is an inflammatory connective tissue disease associated with lymphoid infiltration of the exocrine glands, particularly the salivary and lacrimal glands. This leads to secretory gland dysfunction and usually severe sicca symptoms. Sicca symptoms involve dry mouth, eyes, and mucosal membranes including the upper airways and vagina.
In severe cases of Sjögren syndrome, the dryness can cause salivary gland enlargement and calculus formation. It can also affect the trachea causing dry cough or hoarse voice. Rarely, loss of gastrointestinal exocrine function can be associated with pancreatic dysfunction or pancreatitis, and atrophic gastritis.
In addition to glandular dysfunction, people with primary Sjögren syndrome often have associated:
- fatigue
- arthralgias and nonerosive arthritis
- Raynaud phenomenon
- positive antiphospholipid (aPL) antibodies or antiphospholipid syndrome.
Rarely, a wide spectrum of other extraglandular features can occur involving lymphoid infiltration of various tissues includingRamos-Casals, 2020:
- kidneys (eg glomerulonephritis, chronic kidney disease)
- lungs (eg interstitial lung disease)
- skin (eg diffuse vasculitis associated with skin ulcers)
- muscle (eg severe weakness)
- peripheral nerves (eg mono- and polyneuropathy)
- central nervous system (eg demyelination, myelitis, cerebral vasculitis)
- haematological system (eg haemolytic anaemia, thrombocytopenia)
- gastrointestinal system (eg atrophic gastritis, pancreatitis).
Severe organ-threatening and refractory disease requires urgent specialist referral (eg diffuse vasculitis, interstitial lung disease, kidney failure, severe muscle weakness, neuropathy, myelitis, haemolytic anaemia)Ramos-Casals, 2020.
Although Sjögren syndrome is usually considered a benign disorder, it can very rarely transform into a lymphoid malignancy, primarily of B-cell origin. Persistent glandular swelling or abnormal weight loss should prompt further investigation.