Dr. Scofield said that nerve conduction study would be warranted with this patient. If the results come back normal, a skin biopsy would be a reasonable next step.
Autonomic Nervous System Neuropathies
Autonomic nervous system neuropathies are common in people with Sjögren’s disease, Dr. Varadhachary said.
“Because autonomic nerves are widely distributed cutaneously and viscerally, manifestations can be all over the place,” Dr. Varadhachary said. “People with lots of complaints may have autonomic nervous system neuropathy.”
Some of the symptoms include orthostatic intolerance, vasomotor dysfunction, gastrointestinal (GI) disturbances and bladder dysfunction.
Use of the Composite Autonomic Symptom Score-31 (COMPASS-31) and measurement of orthostatic blood pressure and pulse are useful when autonomic neuropathy is suspected. If findings are abnormal, refer to a specialist or to an autonomic testing center for further evaluation.
For autonomic failure, sensory neuropathy and sensory ataxia, make an urgent neurologist referral. For symptoms of orthostatic intolerance, refer to an autonomic testing center. For other symptoms, such as genitourinary or GI dysfunction, refer to the appropriate specialist.
Although testing is critical, keep in mind that tests are sometimes overly sensitive. “It’s easy to overinterpret a test where someone is still taking a beta blocker and then goes to an autonomics lab and the data is uninterpretable, Dr. Varadhachary said.
Therapy will depend on the exact symptoms. For example, immune-mediated autonomic dysfunction that is severe and progressive may require IVIG and rituximab. Orthostatic tachycardia that causes low blood pressure may require vasopressors or fluid expansion.
“A lot of autonomic neuropathy is patient education and having a multidisciplinary team to educate the patient about lifestyle,” Dr. Scofield said.
The presenters shared the case example of a 31-year-old female with both Sjögren’s and diabetes who came to clinic with positional dizziness, chronic constipation and pain behind her shoulder blades. Her vital signs showed a resting tachycardia and orthostatic hypotension as well as sluggish pupils, stocking-glove dry skin and non-length dependent loss of pin-prick sensation. Her gait was mildly antalgic, and she preferred to travel in a manual wheelchair.
Dr. Scofield said the determination of what tests to do next may depend on how fast the symptoms started.
“Trying to figure out if this is due to diabetes or Sjögren’s is a very difficult task, but clarifying the timeline can inform what you can offer the patient,” Dr. Varadhachary said.
