Highlighting Men with Sjögren’s Disease: A Rheumatologist's Perspective

Highlighting Men with Sjögren’s Disease  

In honor of Men’s Health Awareness month, the Sjögren’s Foundation is highlighting our male patient experience living with Sjögren’s disease. We wanted to highlight men with Sjögren’s, who do not get enough attention in a historically promoted “women’s disease”. Current data suggests that the ratio of men to women with Sjögren’s is 1 in 10, and because of this, men with Sjögren’s have not received the attention and research needed to understand their experience and how it may differ from female patients. Because we estimate that 50% of Sjögren’s patients are undiagnosed, there is a possibility that the ratio of male to female Sjögren’s patients is higher. 

For this blog series, we will first highlight the perspective of Nancy Carteron, MD— a rheumatologist and Sjögren’s expert— about her experience treating male patients. After her viewpoint, we will share current research on male Sjögren’s patients.  

Throughout Men’s Health Awareness Month, we will highlight three men with Sjögren’s that have different backgrounds and, as with all Sjögren’s patients, have heterogenous symptoms and paths in their journey to diagnosis and treatment. 

Experience with Sjögren’s in Men from a Rheumatologist’s Perspective 

By Nancy Carteron, MD, FACR
University of California Berkeley & San Francisco, Rheumatology 

I reviewed many of the male cases I have seen over the years and they really have been a full spectrum, and I have not seen a difference between males and females in how Sjögren’s can present. If a male presents with complaints of dry eye or dental damage or neuropathy or undiagnosed multi-system illness, Sjögren’s Disease (SjD) is in my differential for the diagnostic hypothesis. I would recommend obtaining a blood panel for SSA/SSB (Sjögren’s antibodies), antinuclear antibody (ANA) by indirect immunofluorescence assay (IFA), and rheumatoid factor (RF). If the blood tests are not helpful, I would discuss obtaining a minor salivary gland biopsy (lower lip) to assess for focal lymphocytic sialadenitis. Sometimes the salivary gland ultrasound may be enough for a clinical diagnosis of Sjögren’s, if it shows the classic “cystic” changes (OMERACT Grade 2-3). However, the diagnostic work-up varies based on many factors including patient preferences, insurance coverage, and how the findings will affect treatment options or risk stratification and prognosis in the individual case. 

In my experience, the main issue is Sjögren’s is not considered as a possibility in men, and thus not fully investigated, leading to a delay in diagnosis. This is compounded if SSA/B blood tests were done and are negative, and the investigation has stopped there without ocular and oral assessments. 

I have seen the same multi-system involvement in males and females, including autonomic dysfunction, peripheral neuropathies, T and B-cell lymphomas, cytopenias, lichen planus, sclerosis and Raynaud’s, as well as dryness, musculoskeletal pain, and severe fatigue. 

Urological involvement in men with Sjögren’s can manifest as chronic urethritis, prostatitis, balanitis, bladder dysfunction, and pelvic pain. 

I have seen a number of Sjögren’s males with SSB+, but they are SSA and ANA negative, who had focus scores over 1.0. I do not know if this has any clinical significance or not. My personal numbers are not large enough to have a feel if some of what has been reported in the literature holds up in the population I've seen, like lower rates of SSA/B/ANA, increased pulmonary/interstitial disease, increased lymphoma, or an increase in cardiovascular diseases in men. 

The Foundation truly appreciates Dr. Carteron’s for sharing her perspective and advocating for men with Sjögren’s as well as her continued work with the Sjögren’s Foundation. 

Highlights from Recent Studies on Men with Sjögren’s Compared to Women with Sjögren’s 

Unfortunately, research on differences in men with Sjögren’s and women with Sjögren’s is limited. However, the few studies that have been performed do show differences in risk factors of the disease as well as comorbidities.  

A recent study in Seminars in Arthritis and Rheumatism showed that males were more susceptible to comorbid conditions like hyperlipidemia, hypertension, and cancer compared to females.¹ Males also had higher disease activity based on ESSDAI (European Alliance of Associations for Rheumatology Sjögren’s Syndrome Disease Activity Index) scores and had higher constitutional, lymphadenopathy, pulmonary, and liver involvement. Laboratory analyses showed that males had a lower incidence of high-titer ANA, IgM hypergammaglobulinemia, neutropenia, and anemia. 

Another study in Frontiers in Medicine, found that men with Sjögren's had a higher risk of developing cardiovascular diseases than women with Sjögren’s.² While a couple of studies have  shown that men with Sjögren’s had less dryness and serologic responses^3,4 but had a higher prevalence of parotid enlargement and interstitial lung disease compared to women with Sjögren’s.³ 

While research studies have shown subtle differences between comorbidities and symptoms of men and women with Sjögren’s, more research studies are needed to further explain not only sex differences but the overall diversity in clinical manifestations experienced by all patients with Sjögren’s.  

References:  

1. Fang J, Wang J, Luo J, et al. Clinical stratification of 1318 primary Sjögren’s syndrome patients. Semin Arthritis Rheum. 2024;68:152537. doi:10.1016/j. semarthrit.2024.152537 
2. Bruno KA, Morales-Lara AC, Bittencourt EB, et al. Sex differences in comorbidities associated with Sjögren's disease. Front Med (Lausanne). 2022;9:958670. Published 2022 Aug 4. doi:10.3389/fmed.2022.958670 
3. Zhang Y, Chen JQ, Yang JY, Liao JH, Wu TH, Yu XB, Huang ZW, He Q, Wang Q, Song WJ, Luo J, Tao QW. Sex Difference in Primary Sjögren Syndrome: A Medical Records Review Study. J Clin Rheumatol. 2023 Aug 1;29(5):e78-e85. doi: 10.1097/RHU.0000000000001962. Epub 2023 Apr 17. PMID: 37068269; PMCID: PMC10368225. 
4. Park Y, Lee J, Park SH, Kwok SK. Male patients with primary Sjögren's syndrome: A distinct clinical subgroup?. Int J Rheum Dis. 2020;23(10):1388-1395. doi:10.1111/1756-185X.13940