Sjogren’s syndrome / disease

{| Central Nervous System Sjogren’s | Primary Sjogren’s Syndrome | Secondary Sjogren’s Syndrome}

Summary

Sjögren’s Disease is a disease that causes dryness in your mouth and eyes. It can also lead to dryness in other places that need moisture, such as your nose, throat and skin. Most people who get Sjogren’s are older than 40, nine of 10 are women. Sjogrens is a highly prevalent autoimmune disease, with an estimated 4 million cases in the U.S., making it the second most common autoimmune rheumatic disease in the country. Sjogren’s is sometimes linked to rheumatic problems such as rheumatoid arthritis. In Sjogren’s, your immune system attacks the glands that make tears and saliva. It may also affect your joints, lungs, kidneys, blood vessels, digestive organs, and nerves. The main symptoms are dry eyes, and dry mouth. Treatment focuses on relieving symptoms.

(2022, Autoimmune Association)

Central Nervous System Sjogren’s

CNS or Central Nervous System Complications of Sjögren’s Syndrome

Myelitis

Patients with Sjögren’s syndrome may have “myelitis,” which is inflammation of the spinal cord. Myelitis can cause weakness, numbness, and difficulty with urination and/or defecation. Myelitis can present quickly (i.e. within hours). However, symptoms due to inflammation of the spinal cord may evolve more slowly, progressing over weeks or months. This slower progression may be difficult to distinguish from Multiple Sclerosis (MS). Unfortunately, patients with myelitis and Sjögren’s syndrome can be misdiagnosed with MS. Appropriate tests, which may include spinal tap and MRI of the brain/spinal cord, can lead to diagnostic clarity. The importance of accurately distinguishing between Multiple Sclerosis and Sjögren’s syndrome is of paramount importance. MS and Sjögren’s have completely different treatments. The approved treatments for MS are ineffective for Sjögren’s syndrome, and may actually lead to “flares” or worsening of Sjögren’s syndrome.

Patients with Sjögren’s syndrome may have another autoimmune disorder called “Devic’s syndrome,” or “Neuromyelitis Optica.” Neuromyelitis Optica (NMO) causes inflammation of the nerves connecting the eye to the brain, or “optic neuritis,” as well as myelitis. The pattern of myelitis which can occur in Neuromyelitis Optica is much different than Multiple Sclerosis. Typically, the myelitis in Neuromyelitis Optica is more severe, causing severe weakness, and may cause future relapses.

Again, the treatment for Neuromyelitis Optica is much different than MS. Patients with Sjögren’s syndrome, who are diagnosed with MS, need to be skeptical about receiving these dual diagnoses. Below, I offer some helpful hints, which may especially cast doubt on the diagnosis of MS.

(2022, Birnbaum)

Symptoms

The two main symptoms of Sjogren's syndrome are:

• Dry eyes. Your eyes might burn, itch or feel gritty — as if there's sand in them.

• Dry mouth. Your mouth might feel like it's full of cotton, making it difficult to swallow or speak.

Some people with Sjogren's syndrome also have one or more of the following:

• Joint pain, swelling and stiffness

• Swollen salivary glands — particularly the set located behind your jaw and in front of your ears

• Skin rashes or dry skin

• Vaginal dryness

• Persistent dry cough

• Prolonged fatigue

(2022, Autoimmune Association)

Diagnostic Criteria

None. (Aggarwal et al, 2015)

Study Classification Criteria

(Shiboski et. al, 2017)

ACR = American College of Rheumatology. EULAR = European League Against Rheumatism.

Diagnostic Tests

Under Investigation

Organized Autoimmunity

(Alternative Autoimmune Disease Classification: FIEM, MIEM or BIEM, or FEM, MEM or BEM)

1. sex predominance (is an autoimmune disease primarily found in genetic Females, Males, or equally in Both?)

   1. Under Investigation

2. Inherited and acquired gene variations that cause increased susceptibility

   1. Human Leukocyte Antigen (HLA) Associations

      1. Under Investigation

   2. Other Gene Variations (mutations)

      1. Variations (mutations) in islet cell autoantigen 1 (ICA1) (Johar et. al, 2015 and National Institutes of Health, 2022)

3. Gene Triggering Environmental Exposures

   1. Infections

      1. COVID-19 (Chang et. al, 2023, found a significantly higher risk of Sjogren’s syndrome following COVID-19 infection in a study of 3,814,479 participants)

   2. Toxins

      1. Under Investigation

   3. Stress

      1. Needs to be assessed for each patient

4. Multiple interactive and destructive immune system pathologies

   1. Under Investigation

Tissue-Type or Cell-Type Attacked

(2021, Rojas et. al)

PPV: positive predictive value (the probability that a patient with a positive (abnormal) test result actually has the disease). NPV: negative predictive value (the probability that a person with a negative (normal) test result is truly free of disease). (1999, NY State Department of Health). AUC: area under the curve.

Treatment(s)

Under Investigation

Managing Specialist(s)

• Rheumatologist

Research Authors

Under Investigation

Research Institutions

Under Investigation

Average Time from Symptom Onset to Diagnosis

Under Investigation

Last Updated

September 05, 2023

References

Aggarwal, R., Ringold, S., Khanna, D., Neogi, T., Johnson, S.R., Miller, A., Brunner, H.I., Ogawa, R., Felson, D., Ogdie, A., Aletaha, D. and Feldman, B.M. (2015), Distinctions Between Diagnostic and Classification Criteria?. Arthritis Care & Research, 67: 891-897. https://doi.org/10.1002/acr.22583

Birnbaum, Julius M.D. Neurologic Complications: A PRIMER ON THE NEUROLOGICAL COMPLICATIONS OF SJÖGREN’S SYNDROME. Johns Hopkins Sjogren’s Center. (2022). Retrieved November 16, 2022, from https://www.hopkinssjogrens.org/disease-information/sjogrens-syndrome/neurologic-complications/

Chang R, Yen-Ting Chen T, Wang SI, Hung YM, Chen HY, Wei CJ. Risk of autoimmune diseases in patients with COVID-19: A retrospective cohort study. EClinicalMedicine. 2023 Feb;56:101783. doi: 10.1016/j.eclinm.2022.101783. Epub 2023 Jan 10. PMID: 36643619; PMCID: PMC9830133.

Disease Screening-Statistics Teaching Tools. New York State Department of Health. (1999, April). Retrieved November 15, 2022, from https://www.health.ny.gov/diseases/chronic/discreen.htm

ICA1. National Institutes of Health, National Library of Medicine, National Center for Biotechnology Information. (2022, September 22). Retrieved from https://www.ncbi.nlm.nih.gov/gene/3382

Johar AS, Mastronardi C, Rojas-Villarraga A, Patel HR, Chuah A, Peng K, Higgins A, Milburn P, Palmer S, Silva-Lara MF, Velez JI, Andrews D, Field M, Huttley G, Goodnow C, Anaya JM, Arcos-Burgos M. Novel and rare functional genomic variants in multiple autoimmune syndrome and Sjögren's syndrome. J Transl Med. 2015 Jun 2;13:173. doi: 10.1186/s12967-015-0525-x. PMID: 26031516; PMCID: PMC4450850.

Rojas M, Ramírez-Santana C, Acosta-Ampudia Y, Monsalve DM, Rodriguez-Jimenez M, Zapata E, Naranjo-Pulido A, Suárez-Avellaneda A, Ríos-Serna LJ, Prieto C, Zambrano-Romero W, Valero MA, Rodríguez Y, Mantilla RD, Zhu C, Li QZ, Toro-Gutiérrez CE, Tobón GJ, Anaya JM. New insights into the taxonomy of autoimmune diseases based on polyautoimmunity. J Autoimmun. 2022 Jan;126:102780. doi: 10.1016/j.jaut.2021.102780. Epub 2021 Dec 16. PMID: 34923432.

Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X; International Sjögren's Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjögren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2017 Jan;69(1):35-45. doi: 10.1002/art.39859. Epub 2016 Oct 26. PMID: 27785888; PMCID: PMC5650478. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5650478/pdf/nihms813745.pdf

Sjogren’s Disease. Autoimmune Association. 2022, August 02). Retrieved November 15, 2022, from https://autoimmune.org/disease-information/sjogrens/