Presentation and progression of MPO-ANCA interstitial lung disease

Autor: Lorenzo Salvati, Boaz Palterer, Elena Lazzeri, Emanuele Vivarelli, Marina Amendola, Marco Allinovi, Leonardo Caroti, Alessio Mazzoni, Laura Lasagni, Giacomo Emmi, Edoardo Cavigli, Marco Del Carria, Linda Di Pietro, Mariangela Scavone, Daniele Cammelli, Federico Lavorini, Sara Tomassetti, Elisabetta Rosi, Paola Parronchi
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: Journal of Translational Autoimmunity, Vol 8, Iss , Pp 100235- (2024)
Druh dokumentu: article
ISSN: 2589-9090
DOI: 10.1016/j.jtauto.2024.100235
Popis: The association between MPO-ANCA-associated vasculitis (AAV) and interstitial lung disease (ILD) has been well established. Pulmonary fibrosis may coexist with, follow, or even precede the diagnosis of AAV, and its presence adversely affects the prognosis. The optimal approach to investigating ANCA in patients with ILD remains a subject of ongoing debate. Here we aim to describe presentation and progression of MPO-ANCA ILD. We conducted a retrospective evaluation of a cohort of individuals diagnosed with MPO-ANCA ILD, with or without accompanying renal impairment, at the Immunology and Cell Therapy Unit, Careggi University Hospital, Florence, Italy, between June 2016 and June 2022. Clinical records, imaging studies, pathologic examinations, and laboratory test results were collected. Among the 14 patients identified with MPO-ANCA ILD, we observed a significant association between MPO-ANCA titers assessed at the time of ILD diagnosis and renal involvement. Renal impairment in these cases often manifested as subclinical or slowly progressive kidney damage. Interestingly, complement C3 deposits were consistently found in all renal biopsy specimens, thereby suggesting the potential for novel therapeutic targets in managing renal complications associated with MPO-ANCA ILD. The presentation of MPO-ANCA vasculitis as ILD can be the first and only clinical manifestation. MPO-ANCA levels at ILD diagnosis could warn on the progression to renal involvement in patients with MPO-ANCA ILD, hence caution is needed because renal disease can be subclinical or smoldering.
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