GCA, Takayasu’s and polyarteritis nodosa: Implementing ACR guidelines
ATLANTA — A panel at ACR/ARP 2019 offered an overview of the new guidelines for giant cell arteritis, Takayasu’s syndrome, and polyarteritis nodosa.
Sharon Chung, MD, associate professor of rheumatology at the University of California, San Francisco School of Medicine, presented the methods of the guideline development. “The first step was to develop relevant clinical questions regarding diagnostic testing, treatment, and management,” she said.
There were 27 questions for GCA, 27 for Takayasu’s syndrome (TAK) and 21 questions for polyarteritis nodosa (PAN). Once the questions were developed, the group performed a literature review that ultimately included 399 articles for GCA, 347 for TAK, and 127 articles for PAN. “After the literature review, two panels convened,” Chung said.
Mehrdad Maz, MD, division director of rheumatology at the University of Kansas Medical Center, reviewed the highlights of the GCA guidelines, but offered two important caveats. “We only have one FDA-approved drug for GCA,” he said.
The other caveat, which applies to all three forms of vasculitis presented in the session, is that due to lack of evidence, most of the recommendations are conditional. For example, temporal artery biopsy is conditionally recommended over temporal artery ultrasound for diagnosis of GCA. “This may change as expertise among rheumatologists and radiologists changes in the U.S.,” Maz said.
Other conditional recommendations for diagnosis of GCA include unilateral over bilateral temporal biopsy; a temporal biopsy longer than 1 cm compared with a segment less than 1 cm; and a biopsy within 2 weeks of initiating oral glucocorticoids rather than waiting for a longer period.
The key treatment recommendation calls for high-dose over low-dose oral glucocorticoids. Daily glucocorticoids are also recommended over alternate-day therapy. However, Maz offered another important warning for clinicians treating GCA: “The optimal duration of therapy is still unknown, as you know,” he said.
Beyond treatment, Maz also noted that the guidelines address patients who are in remission. “For those in apparent remission, we strongly recommend long-term clinical monitoring over no monitoring,” he said.
Guidelines for TAK
Anisha B . Dua, MD, MPH, associate professor of rheumatology at the Northwestern University Feinberg School of Medicine, then presented clinical applications of the TAK guidelines. “Patients often present with a nonspecific constitution of symptoms,” she said. “It can be a very difficult one to figure out.”
Looking at the take-home messages from the guideline document, Dua said that in patients with severe active TAK, high-dose oral steroids should be initiated with a nonglucocorticoid immunosuppressive agent such as methotrexate or azathioprine. TNF inhibitors should be considered before tocilizumab (Actemra, Genentech), abatacept (Orencia, Bristol-Myers Squibb), rituximab (Rituxan, Genentech), or ustekinumab (Stelara, Janssen) in patients with refractory disease.
Dua also discussed patients who are in clinical remission. “Our goal for steroids for these patients is zero,” she said.
Other recommendations for patients in remission call for monitoring without use of immunosuppressive therapy in patients who demonstrate increases in ESR and CRP; monitoring of progression in patients with a previously defined radiographic lesion; and escalation of immunosuppression in patients who develop new vascular lesions or rapid progression.
Guidelines for PAN
Jason M. Springer, MD, MS, assistant professor of allergy, clinical immunology and rheumatology at the University of Kansas Medical Center, reviewed guidelines that dealt largely with primary idiopathic PAN, as opposed to hepatitis B–associated PAN. “I will also talk about adenosine deaminase 2 deficiency, or DADA2,” he said.
Regarding diagnosis, deep skin biopsy is conditionally recommended over superficial punch biopsy. Nerve and muscle biopsy are also conditionally recommended, as is visceral vascular imaging in suspected PAN.
For newly diagnosed disease, pulse IV glucocorticoids are conditionally recommended over high-dose glucocorticoids. Other conditional treatment recommendations call for use of cyclophosphamide and glucocorticoids over high-dose glucocorticoids along, along with cyclophosphamide over rituximab.
Springer added a warning about glucocorticoids for younger patients. “We know that in pediatric patients, glucocorticoids can be detrimental to childhood growth,” he said.
Like with GCA, there is little evidence on the optimal duration of therapy for PAN. “The primary goal should be disease control over steroid side effects,” Springer said.
Regarding DADA2, TNF-alpha inhibitors are recommended over glucocorticoids alone, according to Springer. – by Rob Volansky
Session 4M025. GCA, TAK & PAN: How to apply the new ACR guidelines. Presented at: American College of Rheumatology/Association of Rheumatology Professionals Annual Meeting; Nov. 9-13, 2019; Atlanta.
Disclosure: Chung, Dua and Maz report no relevant financial disclosures. Springer reports associations with InflaRx.