Real-World Experience With Avacopan in Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis.

Introduction: Postmarketing data on outcomes of avacopan use in antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) are lacking. Methods: We performed a multicenter retrospective analysis of 92 patients with newly diagnosed or relapsing AAV who received therapy with avacopan. The coprimary outcome measures were clinical remission at 26 and 52 weeks. We use descriptive statistics and univariate logistic regression to assess outcomes and predictors of remission, respectively. Results: Of the 92 patients, 23% (n = 21) had a baseline estimated glomerular filtration rate (eGFR) < 15 ml/min per 1.73 m2 and 10% on kidney replacement therapy at baseline. Among those with kidney involvement, mean (SD) enrollment eGFR was 33 (27) ml/min per 1.73 m2 with a mean (SD) change of +12 (25) and +20 (23) ml/min per 1.73 m2 at weeks 26 and 52, respectively. In addition to avacopan, 47% of patients received combination therapy of rituximab and low-dose cyclophosphamide, and 14% of patients received plasma exchange (PLEX). After induction, the median (interquartile range [IQR]) time to start avacopan was 3.6 (2.1-7.7) weeks, and the median time to discontinue prednisone after starting avacopan was 5.6 (3.3-9.5) weeks. Clinical remission was achieved in 90% of patients at week 26 and 84% of patients at week 52. Of the patients, 20% stopped avacopan due to adverse events, with the most common being elevated serum aminotransferases (4.3%). Conclusion: A high rate of remission and an acceptable safety profile were observed with the use of avacopan in the treatment of AAV in this postmarketing analysis, including the populations excluded from the ADVOCATE trial.

Targets and Combs: A Case of Lupus Enteritis.

Lupus is a common autoimmune disorder with the potential to affect all organ systems. Lupus enteritis is a rare complication that is seen in a subset of patients that present with gastrointestinal symptoms. Its diagnosis commonly involves imaging, showing bowel wall edema as the target sign and vascular engorgement of bowel vessels as the comb sign on CT scans. These findings can help guide the diagnosis, but they are nonspecific and are also found in other conditions that cause bowel wall ischemia. These symptoms are reversible if treated with immunosuppressants. Unfortunately, recurrence is common in lupus enteritis and perforation needs to be ruled out on presentation. In this report, we present the case of a patient with known lupus who was diagnosed with lupus enteritis on imaging and was treated with immunosuppressants. The patient's symptoms resolved subsequently. Our case highlights the fact that the appropriate diagnosis and management of this condition require physical exams, labs, and imaging.