Scientific case study
Sjögren’s Disease – What Are You Missing?
Sponsored by Novartis Pharmaceuticals Corporation
- 10-minute read
Shereen N. Mahmood, MD, RhMSUS
Medical Director, Medical Affairs at Novartis
See clinical perspectives from Dr Mahmood throughout.
Sjögren’s disease is a systemic, heterogeneous autoimmune disease1,2
In a survey of 3622 people with Sjögren’s disease,
up to 48 symptoms were reported, and presentation varied widely between individuals3
In a survey of 3622 people with Sjögren’s disease, up to 48 symptoms were reported, and presentation varied widely between individuals3
30% to 40% of patients with Sjögren’s disease have systemic disease4,*
Patients with Sjögren’s disease are 10x to 44x more likely to develop lymphoma compared with healthy individuals5
This underscores the importance of patient global measures, as feeling better looks different for each person.
What is the
ESSDAI?
What is the
ESSDAI?
It is a clinical tool that assesses 12 organ domains to determine disease activity in Sjögren’s disease6
*Prevalence can be variable and range depending on a multitude of factors (ie, definition of the manifestation, study inclusion, specificity of symptom).
Test your knowledge
Illustrative case study
What are you missing?
- Sex: Female
- Age: 45 years
- Sex: Female
- Age: 45 years
ESSDAI Score13
9
Moderate
disease activity
Domain
Glandular:
Articular:
Constitutional:
Score
2 (low)
4 (moderate)
3 (low)
Clinical history:
- Patient had multiple visits to her PCP for tired vision, fatigue, brain fog, metallic taste in her mouth, and joint aches
- PCP attributed her concerns to pickleball and perimenopause
- Referred to rheumatology after worsening joint pain and intermittent fevers
- Diagnosed with Sjögren’s disease by her rheumatologist
Visual summary:
- Joint count: Synovitis in 5 joints*
- Clinical exam: Lacrimal swelling (0.7 cm)
- Laboratory results and testing: Anti-SSA positive, abnormal salivary flow (<1.5 mL saliva over 15 minutes), positive lip biopsy
*Synovitis joint count is based on the DAS28 evaluation.13
What are you missing?
How could you assess this patient?
QoL impact of moderate disease activity
What are you missing?
“Nonspecific symptoms such as fatigue and joint pain are what make a diagnosis of Sjögren’s disease so elusive, reminding us that this disease can hide in plain sight until a fuller picture emerges.”
Shereen N. Mahmood, MD, RhMSUS
How could you assess this patient?
Potential assessments
Questions to consider asking
- Have you had pain in your hands/wrists/ankles and/or feet in the past 4 weeks?13
- Are you stiff in the morning for at least 30 minutes?13
- Do you have difficulty swallowing dry foods without water?15
- Do you require frequent visits to the dentist and/or dental work?3
QoL impact of moderate disease activity
Patients with Sjögren’s disease with moderate disease activity had significantly worse scores in their physical and mental SF-36 summary scores, compared to those with low disease activity16
aSF-36 is a 36-item questionnaire used to measure health-related QoL. Each item is scored 0 to 100, with 100 representing the best possible health.16
Illustrative case study
What are you missing?
- Sex: Female
- Age: 52 years
- Sex: Female
- Age: 52 years
ESSDAI Score13
10
Moderate
disease activity
Domain
Glandular:
Lymphadenopathy:
Biological:
Score
4 (moderate)
4 (low)
2 (moderate)
Clinical history:
- Chronic parotid gland swelling with jaw pain, especially when chewing
- Glandular symptoms have fluctuated over time but recently worsened
- After receiving her Sjögren’s disease diagnosis, she learned about the associated lymphoma risk online and feels concerned
Visit summary:
- Clinical exam: Enlarged parotid gland (3.5 cm), pre-auricular lymphadenopathy on the right side (1.7 cm)
- Laboratory results and testing: Anti-SSA positive, IgG 22 g/L, historically positive Schirmer’s test, high ESR and CRP, cryoglobulinemia
What are you missing?
How could you assess this patient?
Lymphoma monitoring
What are you missing?
“Imaging, such as ultrasound, can help identify suspicious lymph nodes masked by swollen glands. The risk of lymphoma in patients with Sjögren’s disease can be up to 44x higher than the general population,5 with persistent salivary gland swelling and high focus scores on labial biopsies being important risk factors.17,18 Clinicians should maintain a low threshold for initiating lymphoma screening when suggestive signs and symptoms are
present.”
Shereen N. Mahmood, MD, RhMSUS
How could you assess this patient?
Potential assessments
Questions to consider asking
- Have you experienced unexplained fever, unintentional weight loss, and night sweats?19
- Have you noticed swelling in your neck, armpit, or groin?13
Lymphoma monitoring
Independent risk factors for lymphoma in patients with Sjögren’s disease18
- Salivary gland enlargement
- Lymphadenopathy
- Anti-SSA and/or anti-SSB positivity
- Rheumatoid factor positivity
- Monoclonal gammopathy
- C4 hypocomplementemia
- Raynaud’s phenomenon
Ultrasound imaging of the neck and major salivary glands has diagnostic potential in patients displaying signs of lymphoma or presenting with suspicious new lesions20,21
Illustrative case study
What are you missing?
- Sex: Male
- Age: 67 years
- Sex: Male
- Age: 67 years
ESSDAI Score13
16
High
disease activity
Domain
Biological:
Hematological:
Pulmonary:
Score
2 (moderate)
4 (moderate)
10 (moderate)
Clinical history:
- Persistent cough for 2 months, attributed to alternative diagnoses such as pneumonia and bronchitis
- Treated with antibiotics and later a course of steroids, with minimal improvements
- Pulmonologist diagnosed ILD and referred the patient to rheumatology; Sjögren’s disease confirmed
- Denied sicca symptoms
Visit summary:
- Clinical exam: Crackles at bases bilaterally, shortness of breath on 6-minute walk
- Laboratory results and testing: Anti-SSA positive, IgG 21 g/L, mild anemia (hemoglobin 9 g/dL); ILD shown by high-resolution CT; pulmonary function test with FVC of 70%
What are you missing?
How could you assess this patient?
ILD in Sjögren’s disease
What are you missing?
“Up to 20% of patients with Sjögren’s disease can have ILD.22 Of these, over 50% present without any sicca symptoms.23 In this type of patient, a positive anti-SSA raised the suspicion of Sjӧgren’s related ILD.”
Shereen N. Mahmood, MD, RhMSUS
How could you assess this patient?
Potential assessments
Questions to consider asking
- Have you been experiencing a persistent, dry cough?13
- Are you short of breath while at rest or with mild exertion?13,25
ILD in Sjögren’s disease
The initial symptoms of ILD in patients with Sjögren’s disease are nonspecific (cough, sputum production, dyspnea)26
Symptoms such as dry cough may precede Sjögren’s disease diagnosis by several years24
Patients with Sjögren’s disease and advanced age, dry cough, or dyspnea should be systematically evaluated for ILD involvement26
Raynaud’s phenomenon and lymphopenia may predict the onset of ILD26
Illustrative case study
What are you missing?
- Sex: Female
- Age: 43 years
- Sex: Female
- Age: 43 years
ESSDAI Score13
12
Moderate
disease activity
Domain
PNS:
Biological:
Score
10 (moderate)
2 (moderate)
Clinical history:
- Presented to neurology with lower limb pain that started in the feet and progressed up the right calf; reported that her lower limbs felt “on fire”
- Neurology confirmed peripheral neuropathy on EMG without apparent etiology; referred to rheumatology
- Rheumatologist noted mild to moderate xeropthalmia and xerostomia requiring her to always carry artificial tears and bottled water; diagnosed with Sjögren’s disease
Visit summary:
- Clinical exam: No glandular swelling; functional impairment secondary to discomfort
- Laboratory results and testing: Anti-SSA positive, IgG 22 g/L, NCS findings consistent with axonal sensorimotor polyneuropathy, small fiber neuropathy confirmed by punch biopsy
What are you missing?
How could you assess this patient?
Multidisciplinary management
What are you missing?
“In some patients, neuropathic manifestations can be so prominent and disruptive to daily life that the hallmark symptom of dryness is overlooked. This reminds us that Sjögren’s disease can present in diverse ways, and a broad lens is needed when evaluating patients.”
Shereen N. Mahmood, MD, RhMSUS
How could you assess this patient?
Potential assessments
Questions to consider asking
- Have you experienced burning pain, tingling, or numbness anywhere on your body?29
- Have you experienced any loss of balance or coordination?13
- Do these symptoms disturb your sleep or daily activities?30
Multidisciplinary management
A collaborative model, involving community HCPs, rheumatologists, and specialists, can help optimize patient outcomes31,32
Rheumatologists often coordinate Sjögren’s disease care and are well positioned to make timely diagnoses; cross-specialty collaboration may further support both diagnosis and management31,33
Summary
Measuring disease activity beyond sicca matters
Sjögren’s disease is a systemic disease with manifestations extending far beyond sicca that can cause a significant burden and can potentially lead to irreversible consequences9,34
Follow-up monitoring for patients with systemic disease should be frequent and guided by the individual’s disease activity state31
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Abbreviations
C, complement
CRP, C-reactive protein
CT, computed tomography
DAS28, disease activity score-28
DLCO, diffusing capacity of the lungs for carbon monoxide
EMG, electromyography
ENT, ear, nose, and throat
ESSDAI, European Alliance of Associations for Rheumatology Sjögren’s syndrome disease activity index
ESR, erythrocyte sedimentation rate
FVC, forced vital capacity
HCP, health care professional
IgG, immunoglobulin G
ILD, interstitial lung disease
MRI, magnetic resonance imaging
NCS, nerve conduction study
PCP, primary care physician
PNS, peripheral nervous system
QoL, quality of life
SF-36, Short-Form 36
SSA, Sjögren’s syndrome type A
SSB, Sjögren’s syndrome type B
References
- MCoy SS et al. Arthritis Rheumatol. 2022;74(9):1569-1579.
- Maleki-Fischbach M et al. Arthritis Res Ther. 2024;26(1):43.
- Sjögren’s Foundation. Living with Sjögren’s patient survey. Accessed September 8, 2025. https://sjogrens.org/living-with-sjogrens/patient-survey-results
- Mariette X, Criswell LA. N Engl J Med. 2018;378(10):931-939.
- Huo R et al. Open Med (Wars). 2023;18(1):20230829.
- Cooper C et al. Rheumatol Ther. 2022;9(6):1499-1515.
- McCoy SS et al. Clin Rheumatol. 2022;41(7):2071-2078.
- Negrini S et al. Clin Exp Med. 2022;22(1):9-25.
- Neumann M et al. Cureus. 2021;13(1):e12996.
- Perella C et al. Rheumatol Ther. 2023;10(3):601-614.
- Lackner A et al. Arthritis Res Ther. 2024;261(1):169.
- Carsons SE, Blum MA. Sjogren Syndrome. In: StatPearls. StatPearls Publishing; 2023. Accessed September 2, 2025.
https://www.ncbi.nlm.nih.gov/books/NBK431049/
- Seror R et al. RMD Open. 2015;1(1):e000022.
- Carubbi F et al. Clin Exp Rheumatol. 2020;38(4)(suppl 126):166-173.
- Sjögren’s Foundation. Diagnosis. Accessed May 2. 2025.
https://sjogrens.org/understanding-sjogrens/diagnosis - Omma A et al. Arch Med Sci. 2018;14(4):830-837.
- Risselada AP, et al. Ann Rheum Dis. 2014;73(8):1537-1540.
- Fragkioudaki S et al. Medicine (Baltimore). 2016;95(25):e3766.
- Ansell SM. Mayo Clin Proc. 2015;90(11):1574-1583.
- Hüper D et al. Scand J Rheumatol. 2025;54(1):49-57.
- Price E et al. Rheumatology (Oxford). 2017;56(10):e24-e48.
- Sambataro G et al. Autoimmun Rev. 2020;19(2):102447.
- Gao H et al. Int J Rheum Dis. 2018;21(7):1423-1429.
- Flament T et al. Eur Respir Rev. 2016;25(140):110-123.
- Stojan G et al. Curr Allergy Asthma Rep. 2013;13(4):354-360.
- Lin W et al. BMC Pulm Med. 2022;22(1):73.
- McCoy SS, Baer AN. Curr Treatm Opt Rheumatol. 2017;3(4):275-288.
- Liampas A et al. Eur J Neurol. 2023;30(1):255-265.
- Johns Hopkins Sjögren’s Center. Accessed July 30, 2025. https://www.hopkinssjogrens.org/disease-information/sjogrens-disease/neurologic-complications/
- Hackett KL et al. Br J Occup Ther. 2018;81(4):218-226.
- Ramos-Casals M et al. Ann Rheum Dis. 2020;79(1):3-18.
- Jadeed M, Nuhaily S. Consultant. 2018;58(9):229-241.
- McCoy SS et al. Abstract presented at: EULAR Annual European Congress of Rheumatology 2025; June 11-14, 2025; Barcelona, Spain. Poster POS0848.
- Brito-Zeron P et al. eClinicalMedicine. 2023;61:102062.
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