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Early diagnosis in systemic sclerosis key to limiting comorbidities
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SAN DIEGO — Early screening and early diagnosis are critical to managing systemic sclerosis, according to a presentation at the 2019 Congress of Clinical Rheumatology West.
Dinesh Khanna, MBBS, MSc, professor of rheumatology and internal medicine at the University of Michigan, framed the discussion in terms of take-home messages. “The first take home message is that if someone has sclerodactyly, or a thickening of the fingers, make the diagnosis of systemic sclerosis in the clinic,” he said.
Khanna reported that 95% of patients with systemic sclerosis have sclerodactyly and 90% have Raynaud’s phenomenon.
Moreover, 95% of systemic sclerosis patients have antinuclear antibodies (ANA) as assessed by immunofluorescence, according to Khanna. “Autoantibodies can predict what kind of phenotype the patient has, and what kind of outcomes they will have over time,” he said.
The next take-home message from Khanna was that, after diagnosis, early intervention is key. “Everything that goes wrong in a patient with systemic sclerosis goes wrong in the first 4 to 5 years,” he said. This includes comorbidities in the heart, lung, kidney, and gut. If possible, clinicians should diagnose these comorbidities at first onset.
Understanding predictors of rapidly progressing systemic sclerosis can also help physicians act fast. Some of these possible factors include diffuse systemic sclerosis, renal crisis, interstitial lung disease, pulmonary hypertension, myocardial involvement and/or digital ulcers. “Getting that panel up front, early, can help you prognosticate and educate the patient,” Khanna said.
While early intervention is critical in the lungs and heart, Khanna encouraged clinicians not to forget about the hands. “We have an aggressive hand therapy program at Michigan,” he said. “Whenever you see scleroderma disorder, think about aggressive hand therapy.”
Screening is another critical component of early intervention, noted Khanna. For pulmonary hypertension, he suggested that echocardiogram is functional, but not the gold standard. “You have to look beyond echocardiogram when you are screening this population,” he said, and recommended right heart catheterization as an important screening method. “Once you have diagnosed a patient with pulmonary hypertension in systemic sclerosis, they can go off the cliff quickly.”
Regarding renal involvement, Khanna stressed that patients with rising blood pressure and creatinine are likely in renal crisis. “These patients are resistant to treatment and should be managed in the hospital setting,” he said.
That said, screening for renal involvement can be tricky. Targeted screening, rather than screening all patients with early signs of systemic sclerosis, is a preferable and more cost-effective approach.
There are fewer questions surrounding treatment of renal disease, however. “How do you treat systemic sclerosis with renal involvement?” Khanna said. “ACE inhibitors.”
Shifting back to Raynaud’s phenomenon, Khanna urged education as a cornerstone of clinical intervention. “Avoid skin dryness,” he said. “Pharmacologic management involves local therapy with a wound care specialist. Also, pain management is important in patients with an acute digital ulcer.”
In closing, Khanna reiterated the message of early intervention. “As soon as you diagnose a patient with systemic sclerosis, do the internal organ screenings as necessary,” he said. “Don’t wait for abnormal lung function. Gastrointestinal disease can become very hard to treat if the symptoms go for too long. Use Prozac [fluoxetine, Eli Lilly] in patients who have anxiety.” — by Rob Volansky
Reference:
Khanna D. 2019 Congress of Clinical Rheumatology West. Update on the Management of Scleroderma, Including Raynaud's & Digital Ulcers. September 26-29, 2019; San Diego.
Disclosure: Khanna reports associations with a number of device and pharmaceutical companies.