The Link between Sjögren’s and Dysautonomia

Sjogren’s Syndrome, POTS, and Other Dysautonomias

Sjogren’s syndrome and dysautonomia are interconnected conditions that significantly impact the autonomic nervous system (ANS), leading to a range of debilitating symptoms. Dysautonomia occurs in nearly 50% of individuals with Sjogren’s, making it the second most common cause of autonomic neuropathy after diabetes. It's common enough that well known celebrities have these overlapping conditions. Both Halsey and Solange Knowles famously have Sjogren’s syndrome and POTS.

As we explore the unique challenges faced by people with both conditions, this article sheds light on the symptoms, diagnosis, and treatments available to improve their quality of life.

What is Sjogren’s Syndrome?

Sjögren’s syndrome, often pronounced as “SHOW-grins” and sometimes spelled without the apostrophe, is a systemic autoimmune condition that impacts multiple organ systems, often with severe effects. This disorder primarily targets moisture-producing glands, resulting in hallmark symptoms of dry eyes and dry mouth. However, it also extends far beyond dryness, causing fatigue, joint and muscle pain, chronic respiratory infections, gastrointestinal issues, and, in severe cases, significant organ involvement.

Sjogren’s syndrome affects approximately four million Americans, primarily women, with an average age of diagnosis in the late 40s. It can, however, affect people across all ages, including children. The severity and range of symptoms vary significantly, with some experiencing mild discomfort while others face debilitating complications that impact quality of life. Beyond the dryness of eyes and mouth, common symptoms include swallowing difficulties, sinus issues, swollen glands, and autonomic nervous system dysfunctions like tachycardia and blood pressure fluctuations.

What is Dysautonomia?

Dysautonomia, also referred to as autonomic dysfunction, involves disruptions in the autonomic nervous system, which regulates vital involuntary body functions, including heart rate, blood pressure, digestion, and glandular secretions. The ANS comprises both the sympathetic nervous system, which triggers the “fight or flight” response, and the parasympathetic system, responsible for the “rest and digest” functions. Dysautonomia can result from physical damage to autonomic nerves or neurotransmitter imbalances, leading to impaired communication between the brain and organs like the heart, gastrointestinal system, and lungs.

What is POTS?

Postural Orthostatic Tachycardia Syndrome (POTS) is one of the most common forms of dysautonomia. Characterized by a rapid increase in heart rate upon standing, POTS results from abnormal blood flow regulation. When a person with POTS stands up, their heart rate may increase by 30 beats per minute (bpm) or more within the first 10 minutes, without significant drops in blood pressure. This rapid heart rate is an attempt to compensate for blood that pools in the lower extremities due to inadequate constriction of blood vessels.

The symptoms of POTS vary widely and can include:

• Dizziness and Lightheadedness: Often triggered by standing or prolonged sitting.
• Heart Palpitations: Patients may feel their heart racing or experience a pounding sensation.
• Exercise Intolerance: Physical activities, especially those that involve standing, can lead to fatigue and exacerbation of symptoms.
• Fatigue and Brain Fog: Mental clarity and energy are commonly affected, complicating daily tasks.
• Temperature Dysregulation and Sweating Issues: POTS patients may experience abnormal sweating or difficulty tolerating heat.

POTS symptoms can overlap significantly with those of Sjogren’s-related dysautonomia, which often complicates diagnosis and treatment. A tilt table test is typically used to confirm a POTS diagnosis, measuring how heart rate and blood pressure respond to changes in posture.

Diagnosing Sjogren’s Syndrome

The journey to a Sjogren’s diagnosis is often challenging due to overlapping symptoms with other conditions, along with a lack of specific tests. Many patients undergo years of misdiagnoses before finally being diagnosed. On average, it can take about three years to achieve an accurate diagnosis. Key diagnostic tools include:

• Blood Tests: To identify specific antibodies associated with Sjogren’s.
• Tear Production and Salivary Gland Tests: These tests, such as the Schirmer tear test or salivary gland biopsy, assess the dryness levels and immune response.
• Comprehensive Symptom Assessment: Reviewing medical history and examining the array of symptoms to differentiate Sjogren’s from other autoimmune conditions.

Dysautonomia in Sjogren’s Syndrome

Sjogren’s syndrome is an autoimmune disorder primarily known for causing dry eyes and mouth, but it can also lead to severe systemic effects. In Sjogren’s patients, dysautonomia often presents as one of the earliest symptoms and tends to be more common than in other autoimmune conditions. Common manifestations include:

• Heart Rate and Blood Pressure Dysregulation: Symptoms range from lightheadedness, heart palpitations, and chest pain to presyncope (feeling faint), syncope (fainting), and fluctuating blood pressure.
• Gastrointestinal Dysmotility: Nerve impairment affects the muscles in the esophagus, stomach, and intestines, leading to swallowing difficulties, nausea, vomiting, constipation, diarrhea, and delayed stomach emptying (gastroparesis).
• Bladder Dysfunction: Increased urinary frequency and urgency are common in Sjogren’s-related dysautonomia.
• Secretory Gland Dysfunction: Decreased production of saliva, tears, mucus, and sweat can cause dry eyes, dry mouth, and difficulty with temperature regulation.

Unraveling the Connection Between Sjogren’s and Dysautonomia

Sjogren’s syndrome can provoke inflammation and damage within the nervous system, leading to neuropathy and dysautonomia, which includes conditions like POTS and Orthostatic Intolerance (OI). It is one of the leading causes of autonomic neuropathy, second only to diabetes. Research shows that a notable portion of idiopathic dysautonomia patients who report dryness symptoms also exhibit early-stage Sjogren’s antibodies, even without a confirmed Sjogren’s diagnosis.

Managing Sjogren’s Syndrome

Managing Sjogren’s involves a personalized treatment plan that addresses each patient’s specific symptoms. Although there is no cure, effective management of symptoms can enhance quality of life. Key strategies include:

• Lifestyle Adjustments: Keeping hydrated, using humidifiers, and applying artificial tears or saliva substitutes to manage dryness.
• Medications: Depending on the severity, doctors may prescribe anti-inflammatory drugs, immunosuppressants like hydroxychloroquine, or biologics such as rituximab.
• Specialized Care: Treatment is often multidisciplinary, involving rheumatologists, eye specialists, oral health experts, and occasionally neurologists for nervous system involvement.

Diagnosing Dysautonomia in Sjogren’s Patients

Diagnosis of dysautonomia in Sjogren’s often requires specialized tests since the symptoms overlap with other conditions and can vary widely in severity. Basic evaluation may involve:

• Postural Changes: Vital signs are taken after postural shifts (lying to standing) to assess heart rate and blood pressure responses.
• Tilt Table Testing: Used for a formal diagnosis, this test evaluates changes in blood pressure and heart rate with posture adjustments.
• Nerve Testing: Neurologists may perform tests like small fiber neuropathy evaluations to determine nerve health and function in autonomic neuropathy.

In primary care, measuring heart rate and blood pressure changes in response to position shifts can provide initial insight, but autonomic testing with specialists offers more detailed assessments.

Managing Dysautonomia in Sjogren’s Syndrome

Treatment for dysautonomia in Sjogren’s focuses on symptom management, lifestyle modifications, and medications to stabilize the autonomic nervous system. Key approaches include:

• Lifestyle Adjustments: Compression stockings, abdominal binders, and increased salt and fluid intake help improve blood pressure regulation and reduce orthostatic symptoms. Regular exercises, like recumbent biking and swimming, support cardiovascular health without overstressing the ANS.
• Medications: Beta blockers, vasoconstrictors, and antihistamines can aid in controlling heart rate and blood pressure. In severe cases, immunomodulatory drugs, such as hydroxychloroquine, low-dose naltrexone, rituximab, and intravenous immunoglobulins (IVIG), may provide additional support by modulating immune responses and reducing nerve damage.
• Gastrointestinal Management: Prokinetic agents, antacids, and dietary changes help manage digestive symptoms. Eating small, frequent meals and avoiding trigger foods can alleviate discomfort from gastroparesis and reflux.

The Role of Research in Advancing Treatment

Dysautonomia Intrational and The Sjogren’s Foundation have recently funded research to better understand dysautonomia in Sjogren’s, including research exploring how infections, like Mycoplasma pneumoniae, may trigger neuropathic POTS and other autonomic dysfunctions. These studies may lead to targeted immune therapies to manage autonomic neuropathies more effectively in the future. With ongoing research, clinicians and scientists aim to expand options for treating and supporting individuals with Sjogren’s and dysautonomia.

Celebrities Raising Awareness for Sjogren’s and Dysautonomia

Public figures with POTS, such as singer Solange Knowles and musician Halsey have spoken openly about their struggles with Sjogren’s, dysautonomia, and related conditions, helping to reduce the stigma and increase awareness. Their experiences with these complex conditions highlight the reality for many patients, inspiring broader support and understanding within the chronic illness community.

Conclusion

Navigating life with Sjogren’s syndrome and dysautonomia is challenging, with symptoms affecting nearly every aspect of daily functioning. The journey toward understanding and treating these conditions requires a combination of lifestyle adaptations, medical interventions, and continued research into immune and autonomic nervous system interactions. Through advocacy, public awareness, and ongoing research, there is hope for improved quality of life and future treatments for those facing the complexities of Sjogren’s and dysautonomia.

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