Sjögren Syndrome
Start 3-Month Free Access!
No institutional email?
Start your 1 week free trial, now!
Overview
- Sjögren syndrome is a disease of dryness of the mucosal surfaces and skin.
- It is ~ 9 times more common in women than in men.
Clinical Manifestations (Sicca Symptoms, Raynaud Phenomenon)
- The most common symptoms involve sicca symptoms (dryness) of the mucosal surfaces.
Sicca Symptoms:
- Dryness of the eyes with decreased tear production and resultant keratoconjunctivitis.
- Bilateral parotid gland swelling due to dysfunctional secretion production and release.
- Dryness of the mouth with decreased saliva (xerostomia). Indicate that the tongue is dry and fissured and there can be associated periodontal disease and candidiasis.
- Next, show that vaginal dryness can result in dyspareunia (pain with sexual activity).
- Show that the skin is typically dry and eczematous and indicate palpable purpura can be a sign of vasculitis from cryoglobulinemia in the setting of the Sjögren’s syndrome, a rare but important disease manifestation.
Raynaud phenomenon
- We show a hand and see that the hyperemic appearance of the hand and the chalky-white appearance of the digits that can result from vasospasm in Raynaud phenomenon.
Additional Systemic Symptoms
There are many additional potential systemic features in Sjögren’s disease, including:
- Pulmonary disease: it is more commonly bronchial than interstitial (compare this to the fibrocystic lung manifestations of sarcoidosis).
- Neurological disease: this is uncommon but can manifest with sensory ganglionopathy, small fiber neuropathy, mononeuritis multiplex, multiple cranial neuropathies (esp, CN 5 and CN 7), and myelopathies, including neuromyelitis optica.
Diagnostic Testing
Salivary gland biopsy
- Salivary gland biopsy for direct pathological visualization.
Anti-Ro/SSA & Anti-La/SSB antibodies
- Anti-Ro/SSA and anti-La/SSB antibodies. Note that these antibodies are only present in about half of cases but show high specificity for Sjögren’s disease.
Secretion Testing
- Additional helpful testing includes ocular staining, as well as testing for reduced tear and salivary production.