Sjögren Syndrome in Primary Care: A Quiz

ANSWER: Sicca symptoms, or dryness of exocrine glands, may affect up to 98% of persons with Sjögren syndrome. The most common symptoms of Sjögren syndrome are xerostomia and keratoconjunctivitis sicca. Suspect Sjögren syndrome when sicca symptoms persist for 3 months or longer. The peripheral nervous system can also be involved, but manifestations usually occur later in the disease. Sensory neuropathy is most common and may affect 10% to 25% of cases.

Source: Stefanski AL, Tomiak C, Pleyer U, et al. The diagnosis and treatment of Sjögren’s syndrome. Dtsch Arztebl Int. 2017;114:354-361. doi: 10.3238/arztebl.2017.0354.

ANSWER: Dental caries and early tooth loss are about twice as prevalent among patients with Sjögren syndrome compared with the general population. These patients are also at increased risk for recurrent oral infections with Candida albicans, with a prevalence rate up to 10 times higher than that in the general population. In addition, Sjögren syndrome carries an increased risk of non-Hodgkin lymphoma, which is important to exclude in patients with bilateral swelling of the parotid glands (up to 34% of patients with Sjögren syndrome). These patients are also at increased risk for interstitial lung disease (usually later in the course of disease). However, the most common extraglandular manifestation of Sjögren syndrome is arthralgia and non-erosive polyarthritis. Other manifestations can include dry cough (tracheobronchitis sicca), dyspareunia, vasculitis, renal involvement, sensory neuropathy, and fatigue.

Source: Stefanski AL, Tomiak C, Pleyer U, et al. The diagnosis and treatment of Sjögren’s syndrome. Dtsch Arztebl Int. 2017;114:354-361. doi: 10.3238/arztebl.2017.0354.

ANSWER: Anti-Ro/SSA antibodies and abnormal labial salivary gland biopsy have the highest diagnostic specificity for Sjögren syndrome. Both are included in the American College of Rheumatology/European League Against Rheumatism classification criteria for Sjögren syndrome. Lymphocytic infiltration of exocrine glands with intact acinar units is pathognomonic of Sjögren syndrome. While more than 80% of patients with Sjögren syndrome may have antinuclear antibodies (ANA), up to 20% of healthy persons may also have low ANA titers.

Sources
Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol. 2017;69:35-45.
Stefanski AL, Tomiak C, Pleyer U, et al. The diagnosis and treatment of Sjögren's syndrome. Dtsch Arztebl Int. 2017;114:354-361. doi: 10.3238/arztebl.2017.0354.

ANSWER: Other autoimmune diseases can initially present with sicca symptoms, termed secondary Sjögren syndrome. Secondary Sjögren syndrome is most common in rheumatoid arthritis (20% to 32%), SLE (15% to 36%), and limited and progressive systemic sclerosis (11% to 24%). Less commonly, secondary Sjögren syndrome can occur in multiple sclerosis, autoimmune hepatitis, and thyroiditis.

Source: Stefanski AL, Tomiak C, Pleyer U, et al. The diagnosis and treatment of Sjögren’s syndrome. Dtsch Arztebl Int. 2017;114:354-361. doi: 10.3238/arztebl.2017.0354.

ANSWER: Sjögren syndrome is about nine times more common in women than men, with an average diagnosis around age 56. Diagnostic criteria for Sjögren syndrome include abnormally low salivary flow rate, abnormal Schirmer’s test (which evaluates tear production in the eyes), abnormal findings on lissamine green or fluorescein staining, positive anti-Ro/SSA, and focal lymphocytic sialadenitis. The efficacy of anti-inflammatory cyclosporine A eye drops in treating dry eye in Sjögren syndrome is supported by randomized controlled trials. Other dry eye treatments include pilocarpine, cevimeline, short-term topical corticosteroids, punctal plugs, and scleral lenses. DMARDs are reserved for systemic disease, though limited evidence supports their efficacy.

Sources
Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol. 2017;69:35-45.
Stefanski AL, Tomiak C, Pleyer U, et al. The diagnosis and treatment of Sjögren's syndrome. Dtsch Arztebl Int. 2017;114:354-361. doi: 10.3238/arztebl.2017.0354.