November 10-15

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ACR Convergence 2023

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Sjögren’s syndrome challenges start with underdiagnosis


4 minutes

Often underdiagnosed, there are many facets to the diagnosis and management of Sjögren’s syndrome. A symposium at 4:30 pm Tuesday in Ballroom A, Clinical Conundrums in Sjögren’s Syndrome, will address three common and perplexing clinical challenges managing Sjögren’s syndrome patients.

Alan N. Baer, MD

Alan N. Baer, MD, Associate Professor of Medicine and Director of the Jerome Greene Sjögren’s Syndrome Clinic at Johns Hopkins University School of Medicine in Baltimore, will outline the differential diagnosis and an evaluation strategy for patients with salivary and lacrimal gland enlargement. Potential etiologies include Sjögren’s syndrome, IgG4-related disease, sarcoidosis, and lymphoproliferative disorders.

“Salivary or lacrimal gland lymphoma is a well-recognized complication of Sjögren’s syndrome and can present as either an isolated mass or diffuse glandular enlargement,” Dr. Baer said. “Accurate diagnosis is critical to effective treatment of affected patients.”

Dr. Baer will provide an algorithm for diagnostic evaluation, including the use of imaging and tissue biopsy. He noted that ultrasound-guided core needle biopsy with fine needle aspiration of the major salivary glands has emerged as an effective tool for diagnostic evaluation of salivary gland enlargement.

“Collection of saliva and expressed mucus plugs for cytologic analysis and culture can provide useful diagnostic information,” he said. “There is a range of diseases that can cause salivary and/or lacrimal gland enlargement and accurate diagnosis is imperative to define the best treatment. Much greater sophistication now exists in differentiating among non-malignant causes of this clinical presentation.”

Julius Birnbaum, MD, MHS, Associate Director of the Johns Hopkins Jerome L. Greene Sjögren’s Syndrome Center in Baltimore, will discuss the peripheral nervous system manifestations of Sjögren’s syndrome and how patient descriptors of their symptoms can provide clues to underlying mechanisms.

“For neuropathic pain, the descriptors come in a lot of different flavors,” Dr. Birnbaum said. “Patients can describe tingling, numbness, paroxysmal shock-like pain, burning, sensitivity to touch, and sensitivity to deep pressure. What is underappreciated, and what is emerging in the research, is that symptoms of neuropathic pain, in many cases, actually reflect mechanisms.”

Dr. Birnbaum said that when a patient presents with a spontaneous burning pain or shock-like sensation, that likely reflects ongoing injury to nociceptive neurons, whereas numbness reflects a region of denervation, usually due to a loss of larger fiber nerves.

“Large fiber nerves generally mediate some uncomfortable, but not necessarily noxious, stimuli. Damage to these areas can easily be detected by nerve conduction studies,” he said. “The burning or shock-like sensations are usually mediated by smaller caliber nerves, which can defy diagnosis in traditional nerve conduction studies. In recent years, it’s been shown that a technique called punch skin biopsy, which is a very quick and minimally invasive test, can be used as a biomarker of a small fiber neuropathy.”

When it comes to treatment, Dr. Birnbaum said patients who describe a spontaneous burning sensation are often responsive to anti-epileptic medication for neuropathic pain, while more deep-seated pain or mechanical sensitivity might respond to some fibromyalgia therapeutic agents.

William St.Clair, MD

The clinical utility and efficacy of biologic therapy in Sjögren’s syndrome will be the subject of a presentation by ACR Immediate Past President William St.Clair, MD, W. Lester Brooks, Jr. Professor of Medicine and Chief of the Division of Rheumatology and Immunology at Duke University School of Medicine in Durham, NC.

“The treatment of the glandular manifestations of primary Sjögren’s syndrome consists mainly of symptomatic therapy, such as tear supplements and pharmacologic secretagogues,” Dr. St. Clair said. “Unfortunately, no disease-modifying therapy has been approved for the treatment of primary Sjögren’s syndrome. Investigators have now turned their attention to the biologic therapies, which target molecular components and pathways believed to be important in the immunologic mechanisms of this disease.”

To date, he said, most clinical studies have focused on the efficacy of rituximab, noting that trials are also underway testing abatacept and a combined belimumab-rituximab regimen for the treatment of primary Sjögren’s syndrome.

“Rituximab therapy may be considered in select cases of severe extraglandular involvement, such as interstitial lung disease but, thus far, the use of rituximab or other biologic therapy for improving the glandular disease in primary Sjögren’s syndrome is not supported by evidence from clinical studies,” Dr. St.Clair said. “However, the pipeline for new agents is robust, with numerous other drugs slated for investigation in this disease.”

Clinical Conundrums in Sjögren’s Syndrome
4:30 – 6:00 pm Tuesday • Ballroom A