Sjögren Disease Extends Beyond Dry Eyes, Dry Mouth: Steven Carsons, MD

Sjögren disease reaches well beyond dry eyes and dry mouth, and its variable presentation and the need for several specialists to confirm it contribute to delayed diagnosis, said Steven Carsons, MD, chief of the Division of Rheumatology, Allergy, and Immunology at NYU Langone Hospital–Long Island and vice dean and professor of medicine at NYU Grossman Long Island School of Medicine.

In part 1 of an interview with The American Journal of Managed Care^® (AJMC^®), Carsons discusses the range of symptoms patients experience, how the risk of B-cell lymphoma shapes monitoring, how fatigue and pain are tracked in practice, and how care coordinated across specialties supports patients after diagnosis.

This interview has been edited for clarity.

AJMC: Sjögren disease is very commonly described by its dryness symptoms: the dry eyes and dry mouth. What does the fuller clinical picture look like in the patients you see?

Carsons: More than 90% of patients do at some point have sicca, or dryness, symptoms, mainly involving the eyes and mouth,¹ and I think that’s how Sjögren disease is generally known by the medical profession and the lay public. However, since Sjögren disease can involve exocrine glands more broadly, patients also very frequently experience other dryness symptoms, which sometimes we refer to as other xeroses. These can include nasal dryness, throat dryness, and bronchial dryness, which can lead to a dry cough. Dry skin can be particularly problematic in low-humidity climes and in the wintertime and can lead to severe itching and excoriation. Genital dryness, particularly vaginal dryness in women, can lead to discomfort and dyspareunia.

Having said that, regarding exocrine gland function and sicca symptoms, up to 50% of patients with Sjögren can experience extraglandular manifestations.² This includes visceral involvement and can include organ systems such as the lungs, kidneys, and the peripheral and central nervous system, rarely the gastrointestinal tract—including pancreatic insufficiency and gastroesophageal reflux. Vascular system involvement can be manifested by Raynaud’s phenomenon with tricolor change and pain in the digits, as well as vasculitis, mainly of the skin, but sometimes involving the nerves and other organs as well.

The lymphoid system can be involved with inflammation resulting in lymphadenopathy, and 5% to 10% of patients in published cohorts can develop a B-cell non-Hodgkin lymphoma,³ which, of course, is a very great concern to the physicians caring for them, as well as the patients themselves.

AJMC: How does the potential risk for developing B-cell lymphoma shape the way you monitor patients over time? What warning signs do you want everybody to look for when treating the patient?

Carsons: I just returned from the International Symposium on Sjögren’s Disease in Paris, where there was a presentation from a group that’s attempting to develop a risk score for patients with Sjögren disease to assess risk for lymphoma.⁴ The elements of that risk score comprise a lot of the things that we do in practice when we follow patients with Sjögren disease. There is a periodic, thorough physical exam of the glandular and lymph node areas and blood tests that we use to monitor for abnormal proteins such as monoclonal proteins, low complement, or the presence of rheumatoid factor. All of those have been established in observational studies as having increased odds ratios for the development of lymphoma.⁵

Imaging may be useful if, for instance, a parotid gland is felt to be enlarged or continually enlarging or has a suspicious area on examination. Ultrasound, CT scan, or MRI can be useful, followed by a biopsy if indicated.

AJMC: Fatigue and pain are disabling aspects of the disease, but they’re hard to quantify. How do you assess and track them in practice, and how well do these measures really capture the actual patient experience?

Carsons: In clinical practice, there’s no standardized methodology for capturing these more subjective manifestations, such as fatigue and pain. In clinical trials, we use varying scales, such as ESSPRI, the European Sjögren Syndrome Patient Reported Index, as well as other standardized scales for fatigue, such as the SF-36 [36-Item Short Form Health Survey], fatigue-specific scales, PROMIS [Patient-Reported Outcomes Measurement Information System], etc. In clinical practice, it’s really the physician’s assessment, visit to visit, based on the patient’s report of symptoms.

AJMC: There is a diagnostic delay in Sjögren disease. What typically causes a patient to go years before getting the correct diagnosis?

Carsons: I believe that a large part of the delay is that the disease is so heterogeneous, meaning that it can present in different ways in different individuals. Some have predominantly dry eyes. Some have predominantly dry mouth. Some can present with dental problems. Some can present with glandular swelling, and some can present with extraglandular manifestations, such as joint pain, rash, or neuropathy.

The other facet that aligns with diagnostic delay is that you often need the cooperation of several specialists to make a diagnosis, and that’s far more difficult than, for instance, a patient with rheumatoid arthritis or psoriatic arthritis going to a rheumatologist, where the patient can have a physical exam, imaging studies ordered, and lab studies to make a diagnosis. For Sjögren disease we need the cooperation of an ophthalmologist for a good, quantitative ophthalmology exam that captures things like ocular staining and the Schirmer test, tear break-up time, etc. We also need cooperation of oral medicine, ENT [ear, nose, and throat], or a dental specialist to perform a minor salivary gland biopsy, as well as the rheumatologist. Having to engage different specialists to look at different aspects of the disease can result in a delay in diagnosis.

Additionally, some of these symptoms can, in the beginning, be very nonspecific. Eye dryness is not generally perceived by patients as actual dryness; it’s perceived as a foreign body sensation, like a grain of sand in the eye, accompanied sometimes by itching and ocular fatigue. This is often misinterpreted as an allergy or simply a vision problem. Similarly, dry mouth may be interpreted as a nutritional deficiency or anxiety, because people’s mouths often get dry when they’re anxious. All of these issues can lead to this delay in diagnosis.

AJMC: Coordination among specialists is part of the reason for the delay in diagnosis, but that coordination needs to continue once a patient is diagnosed. How do all these different specialties play a role in managing the full burden of the disease?

Carsons: As a rheumatologist, when managing a patient with documented Sjögren disease, I recommend at least a biannual complete ophthalmologic examination and at least 3 times a year or quarterly dental examination and treatment because, honestly, these are the exocrine target organs of the disease. If these recommendations aren’t fastidiously followed, patients can develop corneal abrasions and eye infections, leading to serious visual consequences. Similarly, dental loss is unfortunately very common in patients with Sjögren disease. A dry mouth loses the bactericidal properties of saliva, so the microbiome and the bacterial flora can be altered and can predispose to dental decay. The ophthalmologic and dental referrals are routine. Then, depending on which extraglandular manifestations are present, additional consultations with other specialists, such as the pulmonologist or the neurologist, may be necessary.

References

1. Thomas D. Sjögren's disease signs and symptoms. Sjögren's Foundation. Accessed October 9, 2026. https://sjogrens.org/understanding-sjogrens/sjogrens-disease-signs-and-symptoms
2. Gairy K, Knight C, Anthony P, Hoskin B. Burden of illness among subgroups of patients with primary Sjögren's syndrome and systemic involvement. Rheumatology (Oxford). 2021;60(4):1871-1881. doi:10.1093/rheumatology/keaa508
3. Nocturne G, Pontarini E, Bonbardieri M, Mariette X. Lymphomas complicating primary Sjögren’s syndrome: from autoimmunity to lymphoma. Rheumatology (Oxford). 2019;60(8):3513-3521. doi:10.1093/rheumatology/kez052
4. Mourot A. SCOLYSS: score of lymphoma risk in Sjögren’s syndrome. Presented at: International Symposium on Sjögren’s Disease 2026; September 29 to October 2, 2026; Paris, France.
5. Fragkioudaki S, Mavragani CP, Moutsopoulos HM. Predicting the risk for lymphoma development in Sjogren syndrome. Medicine (Baltimore). 2016;95(25):e3766. doi:10.1097/MD.000000000000376