Sjögren Syndrome

Overview

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

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.