Why We All Need To Support Research On Sjogren’s & How To Do So
Investing in quality basic and clinical research is important for defining how a disease is initiated and progresses. Without this research, effective therapies for managing and treating a disease cannot be developed. Moreover, basic and clinical research informs medical education. What doctors and health insurance companies are taught about a disease impacts the availability and quality of care; something almost every person with Sjogren’s (SHOW-grins), including me, has learned the hard way. (I’ll be walking for Sjogren’s at the West Coast Walk for Sjogren’s, hosted by the Sjogren’s Foundation on October 19.)
Sjogren’s is the second most common autoimmune rheumatic disorder after rheumatoid arthritis (RA). It’s three times more common than lupus. Sjogren’s affects roughly 4 million people in the US, 9 out of 10 of whom are women, and numbers are growing because COVID-19 increases your chances of developing autoimmune diseases, including Sjogren’s. Yet research on Sjogren’s lags decades behind research on RA and lupus. Unlike RA and lupus, there are only a few landscape studies of Sjogren’s and none of them are comprehensive. We have an incomplete picture of all the ways Sjogren’s can manifest in people, which makes diagnosis, management, and treatment extremely difficult. People with Sjogren’s are forced to rely on other patients and a variety of resources to manage their disease.
Sjogren’s is a serious, systemic disease that is more than “just dryness”. Sjogren’s is the second leading cause of dysautonomia (dysfunction of the autonomic nervous system). Peripheral nervous system manifestations often precede dryness symptoms and can occur in up to 60% of people with Sjogren’s. Sjogren’s can impact every organ and system; some manifestations of Sjogren’s are life-threatening.
Sjogren’s is a hidden disability. A hidden disability is a physical, mental, or neurological condition that is not visible from the outside. Most people with Sjogren’s look well and may have normal labs even when they are very sick. The 2021 Living With Sjogren’s patient survey conducted by the Sjogren’s Foundation shows that 80% of people with Sjogren’s experience cognitive impairment (brain fog).
Sjogren’s is expensive and has a high burden of disease. Sjogren’s patients consistently score lower on health-related quality of life measures than people with closely related diseases e.g., RA and lupus. Multiple studies show that 30-40% of working age Sjogren’s patients leave their jobs because of disabling symptoms. Medical insurance does not cover a lot of the products and services Sjogren’s patients need. Dental work is medically necessary for Sjogren’s care, but is not covered by health insurance, something for which the Sjogren’s Foundation is advocating.
Despite all the evidence to the contrary, misconceptions about Sjogren’s are widespread among healthcare professionals. Often, it’s the patients’ words and hard-earned knowledge against the “experts’” misconceived notions of what Sjogren’s is. These misconceptions are perpetuated in the medical literature, medical education, and publicly available medical platforms, e.g., Mayo Clinic and Cleveland Clinic websites. Sjogren’s Advocate (www.sjogrensadvocate.com) provides valuable evidence-based tools, resources, and strategies for countering misconceptions about Sjogren’s and advocating for diagnosis and care. (Disclaimer: I work with Sjogren’s Advocate and edit the website.)
Sjogren’s is mischaracterized as a mild, dryness syndrome because of how it’s studied. The original understanding of Sjogren’s as a benign syndrome limited to dry eyes and dry mouth remains deeply entrenched in the medical community. When conducting clinical research, standardized definitions called classification criteria are used to ensure that only people who definitely have the condition being studied are included. This is problematic for an invisible illness like Sjogren’s, where many of the manifestations of the disease are not easily observable or measurable. As a consequence, the classification criteria for Sjogren’s are heavily weighted towards dryness, the most visible and easily quantifiable aspects of the disease, which reinforces the misconception that Sjogren’s is mostly dryness.
What started as data has become dogma. The 2016 classification criteria exclude a significant subset, likely 20-40%, of people with Sjogren’s. So, studies based on these criteria only reflect a subset of the population and a portion of the disease. This is problematic because clinical research informs medical education, which, in turn, affects the availability and quality of medical care you receive from your doctor and whether your health insurance will cover it.
Fortunately, the Sjogren’s Foundation offers credible, up-to-date information and develops clinical practice guidelines that inform healthcare professionals on the management of Sjogren’s. Earlier this year the Sjogren’s Foundation created the soon-to-be published clinical practice guidelines for one of the most overlooked targets of Sjogren’s, the peripheral nervous system. Doing so has made this hidden, systemic manifestation more visible. According to Dr. George Sarka, MD, DrPH, MPH, member of the Sjogren's Foundation Peripheral Nervous System Guidelines Topic Review Group, "Traditionally, Sjögren's was looked at as a dry eye and dry mouth disease, but we realize now that it's so much broader than that…It’s a severe, systemic, and complex illness that can affect any body organ or system, and the nervous system is frequently affected.”
We need studies of large populations of people with Sjogren’s that accurately document the true landscape of the disease, including the hidden and hard-to-measure manifestations. We need to understand the basic etiology of Sjogren’s, so that therapies that are effective for more than a subset of people with Sjogren’s are developed. We also need studies designed to understand the complex and systemic manifestations of Sjogren’s, such as fatigue, widespread pain, dysfunction of the nervous system (dysautonomia, neuropathy), and arthritis.
The Sjogren’s Foundation offers 4 types of grants to advance the diagnosis and care of Sjogren’s. These grants are available to both junior and senior investigators; basic or clinical scientists with advanced degrees (MD, PhD, DDS, DMD, OD and similar). If you’re looking for funding, please consider applying. If you know of someone in need of a project and/or funding, please share this article with them.
The Pilot Research Grant and High Impact Research Grant application periods begin on November 1st and end on February 1st. The Pilot Research Grant is designed to, “assist investigators in conducting feasibility studies, collecting preliminary data or other research assistance necessary to advance the project.” The High Impact Research Grant is designed to, “support more fully developed research proposals, which should already have some of the necessary preliminary data and methodology in place to move forward into the next phase of the project”. Recipients are typically notified in May.
The Dynamic Research Grant and Partner Research Grant applications are accepted and reviewed on a rolling basis. These grants are designed to, “support time-sensitive and critical work that falls outside of the Foundation’s normal grant cycle and funding opportunities”. The difference between the two is that the Dynamic Research Grant's administration and deliverables are fully controlled and managed by the Foundation, whereas the Partner Research Grant’s are not.
Supporting Sjogren’s research may help you. If you happen to develop Sjogren’s after a COVID-19 infection and don’t fit the classification criteria, you may find yourself in desperate need of care and unable to get any.
Research on Sjogren’s is essential for ensuring that people with Sjogren’s get the care they need. The Sjogren’s Foundation raises funds for research and advocacy through their annual Walks for Sjogren’s. The West Coast Walk for Sjogren’s is happening on October 19, and I’ll be walking for Sjogren’s. To support research on Sjogren’s, I invite you to join me in walking for Sjogren’s, making a donation on my Walk for Sjogren's page, or sponsoring the Walk with your business. I can be reached at bexiphd.com.
A version of this blog post is published in my local newspaper, The Davis Enterprise.
