<p>As presented at the recent <strong>ACR Convergence 2025</strong> meeting, the clinical manifestations of autonomic neuropathy in patients with Sjögren’s disease (SjD) are diverse and can involve the cardiovascular, gastrointestinal, and genitourinary systems. A referral to a neurologist, cardiologist, gastroenterologist, and/or urologist may be warranted for diagnosis and management in such cases.</p> <p><br></p> <p><em>Following this session, featured expert R. Hal Scofield, MD, was interviewed by</em> Conference Reporter <em>Medical Director Lauren Weinand, MD. Clinical perspectives from Dr Scofield on these issues are presented here.</em></p>

The 3 main neurologic manifestations of SjD are mononeuropathy, polyneuropathy, and autonomic neuropathy. Mononeuropathy really often starts with either facial or trigeminal neuropathy, and the history and physical examination can help differentiate whether it is a fifth or seventh cranial nerve dysfunction. Another mononeuropathy to consider and evaluate is one that causes hand pain, numbness, and weakness. The most common polyneuropathy in SjD is a distal peripheral sensory neuropathy.

<br>

In general, a neurologist is able to evaluate many of the peripheral nervous system manifestations of SjD. However, patients with suspected autonomic neuropathy may need a referral to a specialist because of the kinds of tests that are required. Autonomic neuropathy can cause all types of symptoms and dysfunction, including postural hypotension or a postural orthostatic tachycardia syndrome–like syndrome, tachycardia, gastric motility problems, and sweating difficulties. In our clinic, we administer a very nice questionnaire addressing this called COMPASS-31 that is designed to screen people for these types of clinical manifestations. Based on the initial evaluation, if the patient has evidence of autonomic neuropathy, then the new clinical practice guidelines on peripheral nervous system manifestations in SjD can help you decide who you may need to refer them to.

<br>

If the patient has both autonomic failure and sensory neuropathy or ataxia, which can cause difficulty walking, an urgent referral to a neurologist may be required. For this type of patient, the guidelines recommend that you should consider intravenous immunoglobulin (IVIG). If the patient has orthostatic intolerance or tachycardia, then, again, they need to be referred to a center that has tilt table testing available; this might necessitate an evaluation by a peripheral neuropathy neurologist or cardiologist. If the patient has genitourinary or bladder dysfunction, they may need to be referred to a urologist. For gastrointestinal dysmotility problems, they may need to see a neurologist or gastroenterologist. As far as therapy recommendations for this type of immune-mediated severe autonomic dysfunction, we would consider IVIG; if IVIG fails, then rituximab can be considered.

<br>

Much of the treatment of autonomic neuropathy that causes patients to have symptoms of tachycardia and orthostatic hypotension includes patient education and nonpharmacologic tools, together with multidisciplinary management, which may be a combination of rheumatology, neurology, and cardiology. There are, however, some pharmacologic treatments for these patients. If they have low blood pressure or severe tachycardia, for example, there are agents that may be used to slow down their heart rate. However, again, this may require a referral to a neurologist, a cardiologist, or another specialist in that area.

<br>

During our presentation at ACR Convergence 2025, we discussed many of the signs of this type of autonomic neuropathy, including postural hypotension in which the heart rate does not go up or tachycardia associated with postural orthostatic tachycardia syndrome, nonreactive pupils, bladder trouble, gastroparesis, diarrhea, and/or constipation. One of the main tests to help identify autonomic neuropathy, which, with a little bit of expertise, can be performed by a rheumatologist, is heart rate variability. And, as mentioned by Arun Varadhachary, MD, PhD, the neurologist on the presentation panel, you can do so just by measuring the R-R interval on an electrocardiogram and seeing if the R-R interval varies with inspiration. However, if the R-R interval is fixed with inspiration, then you may have an autonomic neuropathy.

<br>

We also presented the case of a 31-year-old woman who had positional dizziness, chronic constipation, aching between her shoulder blades, and a lot of what seemed to be nonspecific complaints. On examination, she had sluggish pupils; dry, cracked skin; and a loss of pinprick sensation. The patient’s motor examination was normal, but she had difficulty walking and was mostly in a manual wheelchair. We then discussed what you might want to look for in this scenario. Again, the R-R interval is an important test, and we talked about other neurologic tests that you might want to perform in this type of patient. Also, should you empirically offer IVIG to this person, who, in a subacute way, has had difficulty walking during the past few weeks or months, potentially due to her peripheral and autonomic neuropathies?