As a condition that involves sympathetic vasomotor dysfunction, common symptoms of POTS include fatigue, brain fog, headache, orthostatic intolerance, muscle pain and difficulty exercising.1 Its diverse potential etiologies and mechanisms are similar to immune-mediated processes including COVID-19, Lyme disease and Epstein-Barr virus, as well as several autoimmune conditions, such as Sjogren’s disease, systemic lupus erythematosus and celiac disease. Given the potential connections to autoimmune disorders, rheumatologists need to become familiar with the symptoms associated with POTS to help differentiate it from a potential autoimmune condition.
It’s POTS … Or Is It?
Dr. Adler
Dr. Adler discussed how to differentiate POTS from other syndromes with similar clinical features that overlap with other dysautonomia syndromes. She highlighted cases of POTS and dysautonomia that rheumatologists might encounter in their everyday practice and discussed how physicians can distinguish key symptoms of POTS from such overlapping conditions as myalgic encephalomyelitis/chronic fatigue syndrome, mast cell activation and other autonomic disorders.
She said that POTS and other forms of dysautonomia are already in rheumatology practices but that they are not yet recognized by rheumatologists. “Rheumatologists should include dysautonomia in the differential diagnosis when evaluating patients with systemic symptoms and develop a working knowledge of these conditions,” she said, adding that recognizing POTS can lead to better management of a subset of patients whose symptoms don’t fit neatly into a traditional rheumatic diagnosis.
Dr. Adler cautioned, however, that diagnosing patients with POTS is complicated and tricky, and the syndrome can be diagnosed only by clinical presentation because of the lack of a lab biomarker. She used several case studies to illustrate what rheumatologists should look for when making the differential diagnosis of POTS, and ended her talk by highlighting the red flags that rheumatologists should be looking for. Among the most prominent symptoms of POTS, she said, is nonspecific fatigue and brain fog. Other symptoms include orthostatic intolerance, gastrointestinal symptoms, recurrent interstitial cystitis, hypermobility/Ehlers-Danlos syndrome, concurrent pain syndrome or small-fiber neuropathy, color changes in dependent extremities and flushing and itching hives.
Picking up on the symptom of small-fiber neuropathy, one audience member asked about the use of intravenous immunoglobulin in patients with POTS. Dr. Adler said that the question reflected a broader curiosity among rheumatologists about the role of immune dysregulation in POTS and postinfectious chronic illness and what that may mean for treatment. “The possibility that a subset of patients may have an autoimmune etiology raises important questions about when immunotherapy might be appropriate,” she said.
Dr. Adler urged rheumatologists to consider dysautonomia when making a differential diagnosis of a patient, saying that not doing so risks misinterpreting symptoms and managing patients in ways that don’t address their underlying physiology. “By recognizing POTS, we can provide more accurate diagnoses, guide appropriate referrals and improve quality of life for patients who feel misunderstood,” she said.
Management of POTS
Mr. Albert Mears
As a physical therapist, Mr. Mears has been treating POTS for about a decade, and he reinforced the need for an interdisciplinary approach to treatment that includes physical therapy, occupational therapy and speech language pathology therapy. All of these treatment approaches, he said, overlap but each provides specific rehabilitation needs that drives the main goal of management: improving functional capacity.
As a physical therapist, Mr. Mear’s first aim is to narrow the scope of the myriad symptoms with which patients with POTS present, such as whether the patient has heart palpitations, dizziness, fatigue, brain fog or unrestful sleep. Any or all of these symptoms can decrease functional capacity due to poor activity tolerance, emphasizing that poor activity tolerance is the real issue underlying patients with POTS.
He said that therapy starts with understanding the limits of what a patient can do: how much stress can they tolerate while still allowing a timely recovery. To assess this, he uses both subjective and objective measures.
Subjective measures include assessing what a patient’s daily demands are and how well they are tolerated, such as sleep hygiene and how long it takes for them to get up and get going. Mr. Mears uses two subjective scales, the Brief Questionnaire of Exertional Malaise and the Malmo POTS score. He said he gets a pretty good idea of how a patient’s daily living functioning is once he gets the scores from these tests.
He then makes an objective assessment that includes a Static Standing Tolerance Test at every visit. In the test, patients stand for 10 minutes and are asked how they feel while their vital signs are also measured. Other measures include gait speed, a six-minute walk test and an exercise tolerance test. For higher functioning patients, he uses the Functional Index Tool, which is good for measuring endurance.
Critical to successful management of POTS is to educate patients on staying at a consistent exercise level because often patients will overdo it on a good day and do nothing on a bad day. Mr. Mears said that although many exercise protocols are good, they tend not to focus on what he thinks is a key component of POTS management: the ability to recover well from activity. He said a patient can’t move beyond an activity until they reach a good recovery afterward.
Mr. Mears reiterated that the big focus of managing patients with POTS is on recovery and everything else builds from there.
Mary Beth Nierengarten is a freelance medical journalist based in Minneapolis.
Reference
- Liebowitz J. Under pressure: Understanding dysautonomia & POTS. The Rheumatologist. January 1, 2025. https://www.the-rheumatologist.org/article/under-pressure-understanding-dysautonomia-pots/?singlepage=1.
