Dry Mouth
Managing xerostomia remains a challenge, particularly when patients don’t respond to over-the-counter agents or cholinergic agonists like pilocarpine or cevimeline. However, Dr. Johr notes the maximum dose of pilocarpine (30 mg daily) is often underused.
He also encourages patients to think beyond simply sipping water. “If your skin were dry, you wouldn’t just repeatedly wet it with water—you’d use an emollient. It’s the same for the mouth. Patients can try artificial saliva sprays or oil pulling. Green tea may help. It’s also important to avoid medications that worsen dryness.”
Interstitial Lung Disease
For patients with Sjögren’s-related interstitial lung disease (SjD-ILD), the 2023 ACR guidelines (see p. 18) serve as a management framework. Drs. Johr and DiRenzo both follow these. They stress the importance of collaboration with pulmonologists, especially as anti-fibrotic therapies become more common.
“Recent pulmonary literature demonstrates the ILD patients at especially high risk of developing respiratory failure are those with progressive pulmonary fibrosis,” says Dr. Johr. “I keep an eye out for such patients and consider adding an antifibrotic agent in those who don’t respond to initial therapy as recommended in the Consensus Guidelines for the Evaluation and Management of Pulmonary Disease in SjD published in Chest in 2021.”7
Neuropathy
Sensory neuropathy is underappreciated and highly variable feature of SjD. “When it comes to sensory neuropathy, a lot depends on the timing,” says Dr. DiRenzo. “What’s the tempo of it? If it’s not rapidly progressive, treat the symptoms with neuropathic pain medications. If quickly progressing, think about a skin biopsy to look for evidence of vasculitis as a driver. If vasculitis is present, consider immunomodulation.”
“I offer alpha-lipoic acid for milder cases,” says Dr. Johr.
Autonomic neuropathy presents its own challenges. “LDN may help. But in cases of significant dysfunction,” says Dr. Johr, “I consider intravenous immunoglobulin (IVIG). I tell the patient that the evidence behind it isn’t strong, counsel them on the potential risks and warn them that insurance often won’t cover the costs. This is where shared decision making comes in. I find that most patients who are significantly debilitated from their neuropathy are open to it.”
Of note, the Sjögren’s Foundation is slated to release guidelines for neurologic manifestations of SjD later this year.
Cutaneous Manifestations
For rashes in SjD, Dr. DiRenzo uses hydroxychloroquine, and escalates to mycophenolate mofetil if needed. Dr. Johr notes that methotrexate and quinacrine are also options.
