Interstitial Lung Disease and Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

FOR RELATED ARTICLE, SEE PAGE 2334Ethnically diverse case series and cohort studies have reported the association of interstitial lung disease (ILD) microscopic polyangiitis (MPA), an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis.1Tzelepis G.E. Kokosi M. Tzioufas A. et al.Prevalence outcome pulmonary fibrosis in polyangiitis.Eur Respir J. 2010; 36: 116-121Crossref PubMed Scopus (78) Google Scholar, 2Comarmond C. Crestani B. Tazi al.Pulmonary antibodies vasculitis: a 49 patients review literature.Medicine (Baltimore). 2014; 93: 340-349Crossref 3Schirmer J.H. Wright M.N. Vonthein R. al.Clinical presentation long-term 144 with monocentric German cohort.Rheumatology (Oxford). 2016; 55: 71-79Crossref (29) Scholar Patients afflicted both conditions represent significant treatment challenges poor prognosis.1Tzelepis 2334 Up to 10% ILD or experience development ANCAs over time, most which target myeloperoxidase (MPO), rather than proteinase 3 (PR3).4Kagiyama N. Takayanagi Kanauchi T. Ishiguro Yanagisawa Sugita Y. Antineutrophil antibody-positive conversion idiopathic fibrosis.BMJ Open Res. 2015; 2e000058Crossref (30) 5Hozumi H. Oyama Yasui significance myeloperoxidase-anti-neutrophil pneumonias.PLoS One. 2018; 13e0199659Crossref (12) 6Liu G.Y. Ventura I.B. Achtar-Zadeh clinical North American fibrosis.Chest. 2019; 156: 715-723Abstract Full Text PDF MPO-ANCAs, but not PR3-ANCAs, are at risk for subsequent MPA.4Kagiyama Thus, precedes MPA cases, because has either been identified long before diagnosis was detected incidentally same time.1Tzelepis Scholar,2Comarmond Scholar,4Kagiyama can be grouped as usual pneumonia (UIP) non-UIP, latter comprising nonspecific (NSIP), organizing pneumonia, bronchiolitis, other unclassifiable fibroinflammatory findings.1Tzelepis Scholar,7Baqir Yi E.E. Colby T.V. Cox C.W. Ryu Specks U. Radiologic pathologic characteristics myeloperoxidase-antineutrophil antibody-associated disease: retrospective analysis.Sarcoidosis Vasc Diffuse Lung Dis. 195-201PubMed Scholar,8Maillet Goletto Beltramo G. al.Usual ANCA-associated prognostic factor.J Autoimmun. 2020; 106: 102338Crossref (3) The report by Hozumi al9Hozumi Kono Hasegawa its acute exacerbation polyangiitis.Chest. 2021; 159: 2334-2345Abstract (1) this issue CHEST stands out thorough analysis characteristics, outcomes, factors MPA. authors compare (MPA-ILD) without (MPA-non-ILD). As first, they also MPA-ILD control group (ILD-alone) matched age, sex, chest high-resolution CT pattern. key finding is that mortality rate higher alone. This study reports frequency observed elsewhere,3Schirmer Scholar,9Hozumi may linked men smokers compared MPA-non-ILD, smoking factor ILD. Sex history were different between ILD-alone groups. Alternatively, ethnic differences cohorts need consideration.3Schirmer Moreover, overall cohorts.3Schirmer could received glucocorticoids immunosuppressants reduce rates considered part standard treatment.10Yates Watts R.A. Bajema I.M. al.EULAR/ERA-EDTA recommendations management vasculitis.Ann Rheum 75: 1583-1594Crossref Regardless these differences, provides solid novel information about complex relationship confirm portends prognosis MPA.1Tzelepis Scholar,3Schirmer common causes death ILD-related.9Hozumi Multivariate revealed reduction FVC associated independently all-cause mortality, reflected elevated serum KL-6 levels biomarker severity.9Hozumi 1-year cumulative incidence exacerbations 7.2% ILD-alone.9Hozumi within range frequencies UIP NSIP, context connective tissue disorders.11Leuschner Behr Acute disease.Front Med (Lausanne). 2017; 4: 176Crossref (33) Within group, numerically among pattern non-UIP pattern; contrast another report, significant.8Maillet Despite ILD-alone, significantly MPA-ILD.9Hozumi attributable vasculitis- treatment-related complications; specifically, exposure immunosuppression infections high bleeding complications included diffuse alveolar hemorrhage (DAH) group.9Hozumi Relapses presenting DAH more frequent begging question whether predisposes MPA, mild becomes clinically relevant early impaired function from In presence ILD, severity type (UIP vs non-UIP) determine patient outcomes response immunosuppressive therapy, theme resembles disorders-associated ILD.12Fischer du Bois Interstitial disorders.Lancet. 2012; 380: 689-698Abstract (202) Conversely, who develop manifestations aggravate above expectation What clinicians do? posed best addressed coordinated fashion team experts vasculitis Because usually autoantibodies include precede detectable autoimmune disease, newly diagnosed should undergo ANCA-testing. recommendation goes beyond recent ATS/ERS/JRS/ALAT guideline IPF, only consider ANCA-testing “if suspected.” For empiric management, MPO-ANCAs overt categorized based on findings: those definite probable patterns (UIP-ILD) all others (non-UIP). evidence active vasculitis, treated like IPF future vasculitis. glomerulonephritis insidiously, urine such monitored least quarterly microhematuria. All cause symptoms prompt diagnostic evaluation. If ground-glass opacities prominent feature needs excluded BAL manifestation many do hemoptysis. Once developed, require therapy according guidelines Extrapulmonary will respond immunosuppression. Non-UIP improve under targets By contrast, course UIP-ILD benefit immunosuppression; fact, PANTHER trial present indicate increased death. remission induced, minimized offered antifibrotic therapy. remains unclear date there causal promotes isolated some two completely unrelated comorbidities patients. Answering questions possible pathogenic relationships requires research provide new insights into chronic fibrosing processes MPO-ANCA-associated ranges bland recurrent subglottic stenosis sclerosing glomerulonephritis. Clinical Significance Disease Its Exacerbation Microscopic PolyangiitisCHESTVol. 159Issue 6PreviewMPA-ILD represented distinct phenotype prognosis. Lower %FVC independent factor. lower had developing AE, strong determinant. specific AE established. Full-Text