From the Guidelines
The workup for Sjögren's syndrome should include a comprehensive clinical evaluation, laboratory tests, and potentially imaging studies, as recommended by the most recent guidelines 1. Begin with a detailed history focusing on sicca symptoms (dry eyes, dry mouth), joint pain, fatigue, and systemic manifestations. Laboratory testing should include:
- Antinuclear antibodies (ANA)
- Anti-SSA/Ro and anti-SSB/La antibodies
- Rheumatoid factor
- Complete blood count
- Comprehensive metabolic panel
- Erythrocyte sedimentation rate
- C-reactive protein Specific tests for salivary gland function include:
- Unstimulated salivary flow rate measurement
- Salivary gland scintigraphy For ocular involvement, perform:
- Schirmer's test (measuring tear production)
- Ocular surface staining
- Tear break-up time A lip biopsy to evaluate minor salivary glands for lymphocytic infiltration is often considered the gold standard diagnostic procedure. Additional testing may include:
- Ultrasound of salivary glands to detect characteristic changes
- Chest imaging if pulmonary involvement is suspected
- Urinalysis to screen for renal involvement Patients should also be evaluated for associated conditions like thyroid disease, other autoimmune disorders, and lymphoma risk, as suggested by recent studies 1. This comprehensive approach helps establish the diagnosis of Sjögren's syndrome, which is important for initiating appropriate management strategies targeting both the sicca symptoms and potential systemic manifestations of this autoimmune disease. Some studies also suggest the use of new imaging devices, such as placido-ring corneal topographer and color camera, to evaluate the health of the ocular surface 1. It is also important to note that patients with Sjögren's syndrome may develop other ocular manifestations of immune dysfunction, including scleritis, sterile keratitis, and uveitis, and are at increased risk for potentially life-threatening vasculitic or lymphoproliferative disorders 1. Therefore, a multidisciplinary approach, including rheumatologists, ophthalmologists, and other specialists, is essential for the management of patients with Sjögren's syndrome.
From the FDA Drug Label
Sjogren's syndrome Patients Two separate studies were conducted in patients with primary or secondary Sjogren’s Syndrome. In both studies, the majority of patients best fit the European criteria for having primary Sjogren’s Syndrome [“Criteria for the Classification of Sjogren’s Syndrome” (Vitali C, Bombardieri S, Moutsopoulos HM, et al: Preliminary criteria for the classification of Sjogren’s Syndrome. Arthritis Rheum 1993; 36:340-347.)] A 12-week, randomized, double-blind, parallel-group, placebo-controlled study was conducted in 256 patients (14 men, 242 women) whose mean age was 57 years with a range of 24 to 85 years. Another 12 week randomized, double-blind, parallel-group, placebo-controlled study was conducted in 373 patients (16 men, 357 women) whose mean age was 55 years with a range of 21 to 84.
The workup for Sjögren's syndrome is not explicitly stated in the provided drug labels. However, the labels mention that the majority of patients in the studies best fit the European criteria for having primary Sjogren’s Syndrome 2. The criteria are referenced from the article "Preliminary criteria for the classification of Sjogren’s Syndrome" by Vitali C, Bombardieri S, Moutsopoulos HM, et al, published in Arthritis Rheum 1993; 36:340-347.
- The European criteria may be used for the classification of Sjogren’s Syndrome.
- The workup may involve assessing dry mouth symptoms such as severity of dry mouth, mouth discomfort, ability to speak without water, ability to sleep without drinking water, ability to swallow food without drinking, and a decreased use of saliva substitutes.
- The global improvement of dry mouth can be evaluated using a 100 mm visual analogue scale.
Note that the provided drug labels do not provide a comprehensive workup for Sjögren's syndrome, and the information provided is limited to the context of the studies conducted for the drug pilocarpine.
From the Research
Diagnostic Criteria for Sjögren's Syndrome
The diagnostic criteria for Sjögren's syndrome involve a combination of:
- Histology
- Immunology
- Ophthalmology
- Salivary flow 3 The American College of Rheumatology and European League Against Rheumatism (EULAR) classification criteria are used for diagnosis 4
Clinical Features and Laboratory Investigations
The cardinal features of Sjögren's syndrome are summarized through clinical features, laboratory investigations, lower lip biopsy, and imaging findings 5 Some patients present with systemic manifestations, including dry mouth and dry eyes symptoms 4
Imaging Characteristics
Modern imaging techniques provide complementary and additional information to diagnosis, staging, and surveillance 3 Imaging findings of the central nervous system disorders associated with Sjögren's syndrome, including the neuromyelitis optica spectrum disorder, can be identified 3
Treatment and Management
There is no curative treatment for Sjögren's syndrome, but symptom relief can be achieved through local treatments, such as artificial tears or oral topical sprays, and systemic treatment 6 Oral pilocarpine significantly improves sicca symptoms in the eyes, mouth, and other sites 6 Recent clinical trials showed a significant decrease of systemic activity of SS in patients treated with iscalimab (anti-CD40) and ianalumab (anti-BAFF-R) 4 Targeted drugs, low-side-effect drugs, and combination therapies should be the focus of future research 7