From the Guidelines
Sjögren's syndrome is diagnosed through a combination of clinical evaluation, blood tests, and specialized procedures, with a focus on identifying autoantibodies, assessing tear production, and evaluating ocular surface damage, as recommended by the latest guidelines 1. The primary signs of Sjögren's syndrome include dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), often accompanied by fatigue, joint pain, and swollen salivary glands.
- Diagnostic testing typically begins with blood tests looking for autoantibodies, particularly anti-SSA/Ro and anti-SSB/La antibodies, which are present in many Sjögren's patients 1.
- Rheumatoid factor and antinuclear antibodies (ANA) may also be elevated, and complete blood count, erythrocyte sedimentation rate, and C-reactive protein help assess inflammation levels 1.
- Eye tests are crucial, including Schirmer's test (measuring tear production using filter paper strips) and ocular surface staining with dyes to evaluate corneal damage 1.
- Oral assessment includes salivary flow rate measurement and salivary gland biopsy, considered the gold standard diagnostic procedure, which typically shows lymphocytic infiltration 1. Additional tests may include salivary gland ultrasound, sialography (imaging of salivary ducts), and lip biopsy, and early diagnosis is important as it allows for symptom management and monitoring for potential complications like lymphoma, which occurs at higher rates in Sjögren's patients 1.
- The latest classification criteria for Sjögren's syndrome are based on the weighted sum of five items, including anti-SSA/Ro antibody positivity, focal lymphocytic sialadenitis, abnormal ocular staining score, Schirmer test result, and unstimulated salivary flow rate 1.
- A panel of experts recommends a comprehensive evaluation of patients with Sjögren-related dry eye, including symptoms, tear film stability, tear osmolarity, and ocular surface damage, to guide treatment options 1.
From the Research
Signs of Sjögren's Syndrome
- Dryness of the mouth and eyes due to involvement of the salivary and lacrimal glands 2
- Extraglandular manifestations involving skin, lung, heart, kidneys, and nervous system (peripheral and central) and haematological and lymphoproliferative disorders 2
- Symptoms of fibromyalgia (arthralgia, myalgia, fatigue) and oral and ocular dryness in the presence of circulating antinuclear antibodies 2
- Keratoconjunctivitis sicca confirmed with Schirmer's test, the ocular staining score (OSS) using lissamine green, fluorescein staining 3
Diagnostic Tests for Sjögren's Syndrome
- Biopsy sample from a minor salivary gland 2
- Autoantibody tests such as anti-SS-A and anti-SS-B 2, 4, 3
- Antinuclear antibodies (ANA) and rheumatoid factor (RF) 4
- RF-IgA, IgG, and IgM immunoglobulin classes as potential biomarkers 3
- Anti-centromere antibody (ACA) as a useful serologic marker 5
- Schirmer's test and fluorescein staining of the cornea to assess lacrimal secretion and keratoconjunctivitis sicca 5
- Salivary gland scintigraphy (SGS) and histopathologic examination of the minor salivary glands to evaluate salivary secretion and glandular damage 5
Diagnostic Criteria for Sjögren's Syndrome
- Objective signs and symptoms of dryness including a characteristic appearance of a biopsy sample from a minor salivary gland or autoantibody such as anti-SS-A 2
- Exclusions to the diagnosis include infections with HIV, human T-lymphotropic virus type I, or hepatitis C virus 2
- The 1993 European preliminary classification criteria and the 2002 revised criteria for primary Sjögren's syndrome (pSS) 4
- The 2017 ACR/Eular criteria for pSS 3