Uncovering gastrointestinal involvement in systemic sclerosis requires 'asking the right questions'
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Although rheumatologists are more attuned to the skin symptoms associated with systemic sclerosis, there should be a “heightened awareness” of gastrointestinal involvement, which is highly prevalent among this patient population and can precede skin manifestations, according to data presented at the virtual ACR State-of-the-Art Clinical Symposium.
“In systemic sclerosis, the gastrointestinal tract is incredibly important to understand,” Tracy M. Frech, MD, MS, director of the Systemic Sclerosis Clinic and director of clinical trials for the division of rheumatology at the University of Utah Hospital, said in her presentation. “It is the most common involved internal organ in systemic sclerosis, and can be the presenting feature in 10% of our patients. During the course of an illness, 95% of systemic sclerosis patients will complain of gastrointestinal symptoms. Additionally, 6% to 12% of our patients will die from gastrointestinal tract involvement.”
“However, it’s difficult for the rheumatologist to understand treatment and management of systemic sclerosis gastrointestinal illness, because our patients can have very heterogeneous clinical presentations as well as disease courses,” she added.
Moreover, Frech noted that gastrointestinal tract involvement is not adequately differentiated among the subgroups of patients with systemic sclerosis. She explained that gastrointestinal symptoms can precede any laboratory or anatomical abnormalities found through imaging. Additionally, an absence of symptoms does not exclude dysfunction, and rheumatologists need to be watchful for surprising gastrointestinal symptom manifestations.
“Importantly, cough can be a manifestation of esophageal symptoms,” Frech said. “We commonly associate cough with interstitial lung disease or lung involvement in systemic sclerosis so we have to have a heightened awareness on how we can approach GI tract symptoms in our patients.”
Frech remarked that rheumatologists are particularly adept at physical examination of the hands for systemic sclerosis, with concerns focused on the telltale signs of vasculopathy, immune dysfunction and fibrosis of the skin and visceral organs.
“For gastroenterologists, however, they may see that patient as a snapshot in time, meaning they may see small vessel vasculopathy in the form of gastric antral vascular ectasia (or GAVE),” she said. “They may have [a biopsy report] consistent with systemic sclerosis or a manometry report that shows classic scleroderma with dysmotility of the lower esophageal sphincter. When the gastroenterologist refers to rheumatology to ask us whether their patient has systemic sclerosis, we need to do a good job of looking at the hands for puffy fingers, skin thickening, telangiectasis, digital tip lesions and capillaroscopy to make that diagnosis.”
Frech noted that many rheumatologists are hesitant to order gastrointestinal tract procedures, but the use of patient-reported questionnaires — like the UCLA SCTC GIT 2.0 or PROMIS — to assess gastrointestinal symptoms and their severity can help alleviate some of their concerns.
“We are trying to understand how we can ask the right questions and order the right procedures,” Frech said. “A rheumatologist may be concerned that if they directly order the GI tract procedure, they will get a challenging result. For instance, if the procedure is particularly invasive, the rheumatologist may [wonder] ‘did I order the right procedure, or should I have deferred that to the gastroenterologist?’”
Additionally, there are concerns regarding the toll diagnostic testing will take on the patient, both their time and the costs of the procedures. Frech noted that more extensive use of questionnaires by rheumatologists can help minimize the burden on a patient’s time and reduce the costs incurred by unnecessary or redundant gastrointestinal tract procedures.
“We want to make sure we order the right procedure to get the right answer to move the patient’s management plan forward,” Frech said. “There is nothing more frustrating than when we order a procedure and receive results that say, ‘findings are consistent with systemic sclerosis’. When we order a procedure, we want to know it will provide us with some kind of guidance. As rheumatologists, we need to be very comfortable in how we are going to ask our questions properly so we can identify [GI tract] problems, as well as order testing to make a diagnosis and move that patient’s care forward.” – by Robert Stott
Reference:
Frech T. Management of GI Manifestations in Scleroderma. Presented at: American College of Rheumatology State-of-the-Art Clinical Symposium. May 16-17, 2020 (virtual meeting).
Disclosures: Frech reports no relevant financial disclosures.
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Frech T. Management of GI Manifestations in Scleroderma. Presented at: American College of Rheumatology State-of-the-Art Clinical Symposium. May 16-17, 2020 (virtual meeting).
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