Disease Severity and Response to Induction Therapy in Hispanic Patients With Antineutrophilic Cytoplasmic Autoantibody-Associated Vasculitis-Related Diffuse Alveolar Hemorrhage

Abstract

Objectives We examined the response to induction therapy of Hispanic patients with antibody-associated vasculitis (AAV)-related diffuse alveolar hemorrhage (DAH). This study aimed to determine the severity of disease at presentation and the response to induction therapy in our patient population. Methods We retrospectively reviewed the clinical data of Hispanic patients hospitalized with antineutrophil cytoplasmic antibody (ANCA) vasculitis between October 1, 2010, and December 31, 2021. We identified 98 Hispanic patients hospitalized with AAV and 19 admitted with AAV-related DAH. The Birmingham Vasculitis Activity Score (BVAS) was obtained from all patients on presentation. Results Based on the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides, 12 patients met the diagnostic criteria for microscopic polyangiitis (MPA) and seven met the criteria for diagnosing granulomatosis with polyangiitis (GPA). All patients received methylprednisolone therapy. Induction therapy consisted of cyclophosphamide pulse therapy (n=3), cyclophosphamide plus plasmapheresis (PLEX) (n=1), rituximab induction therapy (n=8), and rituximab induction plus plasmapheresis (n=6), and one patient received one dose of cyclophosphamide followed by rituximab plus plasmapheresis. The average BVAS was 25.53 at presentation. Survival at six months included 67% (n=2) treated with cyclophosphamide alone, 75% (n=6) treated with rituximab alone, and 50% (n=3) treated with rituximab plus PLEX. The patient who received an initial loading dose of cyclophosphamide followed by rituximab plus PLEX did survive for six months; however, the patient treated with cyclophosphamide plus PLEX did not have early survival. Conclusions Hispanic patients with ANCA-associated vasculitis present with a more severe disease burden at presentation based on BVAS. Approximately 37% of our patient population had early death (death at <6 months) despite adhering to the standard of care for induction therapy. Due to the more significant disease burden at presentation, it is vital to include ethnic minorities in large clinical trials to help improve outcomes in these patient populations.

The Efficacy and Safety of Rituximab in ANCA-Associated Vasculitis: A Systematic Review

“…Adults comprise a higher proportion of AAV patients than pediatrics [ 81 ]. Thietart et al [ 82 ] evaluated RTX for induction and maintenance therapy in AAV patients older than 75 years. It was shown that RTX induction therapy resulted in remission in 86.4% of patients and the relapse rate was lower when RTX was used as a maintenance regimen compared to induction therapy.…”

“…Another study on 251 AAV patients with severe renal disease showed that although RTX and CYC both induce remission in AAV patients, CR was better induced by RTX than CYC [ 98 ]. A study on Hispanic patients showed that RTX and CYC have a similar effect on the induction of remission; however, RTX proved more effective in the prevention of relapse [ 82 ]. Although RTX was found to be as effective as CYC, it was found that RTX is more efficient than Infliximab, an anti-TNF-α agent.…”

“…A pulmonary nodule is a common presentation of GPA; however, Henderson et al [ 143 ] demonstrated that in GPA patients with pulmonary nodules who did not respond to conventional immunosuppression, RTX can remove the nodules and miniaturize the large nodules. A study on 19 Hispanic AAV patients with diffuse alveolar hemorrhage showed that a regimen of CYC as initial therapy followed by RTX and plasmapheresis is superior to CYC plus plasmapheresis without RTX and provides six months of survival [ 82 ].…”

Granulomatosis With Polyangiitis Presenting With Diffuse Alveolar Hemorrhage: A Systematic Review

“…In the Loftis et al. study, 19 patients with a diagnosis of DAH associated with ANCA vasculitis were analyzed [ 5 ]. Interesting, GPA was less frequent in this cohort, with only seven patients (37%).…”

“…DAH is a feared form of presentation of GPA. DAH is clinically marked by hemoptysis, anemia, diffuse alveolar infiltrates on imaging, and hypoxemic respiratory failure [ 5 ]. The presence of hemosiderin-laden macrophage on bronchoalveolar lavage (BAL) is considered diagnostic [ 6 ].…”