TABLE 1

Published studies of anti-neutrophil cytoplasmic antibody associated interstitial lung disease

Reference, year, countryStudy descriptionNumber of patientsMean age (years)Male (%)ILD (%)Systemic vasculitis (%)PF preceding AAV (%)Median follow-up period (months)ANCA specificity (%)HRCT pattern
Nada et al. [2], 1990, USAPF in patients with pulmonary-renal vasculitis37333100100 MPA33 preceding, 67 concurrent12067 p-ANCAND
Arimura et al. [3], 1995, JapanPulmonary involvement in patients with MPO-ANCA4661.1ND4328 MPANDND100 MPOND
Hiromura et al. [4], 2000, JapanMPO+rapidly progressive GN during course of IPF46750100100 MPA100ND100 MPOND
Eschun et al. [5], 2003, CanadaPF presenting manifestation of MPA669.8 (63–78)50100100 MPA10036100 p-ANCA3/6 UIP-like,
2/6 fibrotic NSIP,
1 ND
Homma et al. [6], 2004, JapanPF in MPO-ANCA31695510025 MPA100120100 MPO84% UIP
Foulon et al. [7], 2008, FranceANCA-associated PF17667110041 MPA86 preceding, 14 concurrent57±416/17 MPO,
1/17 PR3
100% UIP
Nozu et al. [8], 2009, JapanComparison of ANCA-positive and -negative patients with PF19/53 with ANCA+ PF, 34/53 ANCA− PF69 (52–80)5810021 MPA100 preceding1–9017/19 MPO,
2/19 PR3
73.3% UIP
Hervier et al. [9], 2009, FrancePF associated with AAV1270.77510083 MPA,
17 GPA
66 concurrent, 25 preceding, 8 following AAV49.2 (7–116)100 MPO 1006/12 UIP,
1/12 NSIP,
5/12 ND
Tzelepis et al. [10], 2010, GreecePrevalence and outcome of PF in MPA36576939100 MPA92 concurrent, 8 following AAV38±3085 p-ANCA,
8 p-ANCA+c-ANCA
54% UIP,
31% NSIP
Arulkumaran et al. [11], 2011, UKILD and AAV1467.371100100 MPA14 preceding, 64 concurrent, 21 post90100 MPOND
Tanaka et al. [12], 2012, JapanIP associated with MPO- ANCA962.166100NoneND39.1100 MPO66% UIP,
11% NSIP,
11% OP,
11% DAD
Ahn et al. [13], 2012, KoreaClinical features and outcomes of MPA55 patients with MPA, 13 with ILD (23.6%),59.29±13.60 MPA, ND in ILD patients60% MPA, ND in ILD patients24100 MPAND46.07 ±39.98100 MPO84.6% UIP,
1 patient OP,
2 patients NSIP
Ando et al. [14], 2013, JapanIncidence of MPO-ANCA and MPA in course of IPF61 patients with initial dx IPF, 9/61 ANCA+69 (57–75)751522 (2/9) MPA100 preceding40 (1–121)100 MPO78% UIP,
22% ND
Comarmond et al. [15], 2014, FrancePF in AAV4966 (57–72)6110082 MPA,
18 GPA
45 preceding, 43 concurrent, 12 post48 (14–88)88 MPO, 4 PR343% “typical” UIP,
14% “atypical” UIP,
7% fibrotic NSIP,
9.5% NSIP
Huang et al. [16], 2014, ChinaMPA+PF1963.642100100 MPA68 preceding,
32 concurrent
29.9 (8–93)100 MPO100% UIP
Yu et al. [17], 2014, ChinaCT image analysis before and after treatment of ANCA-ILD872.688100NDNDND75 MPO,
12.5
PR3+MPO, 12.5 PR3
ND
Fernandez Casares et al. [18], 2015, ArgentinaMPA associated with PF9 patients with PF out of 28 MPA patients60±14563210056 preceding, 44 concurrent76±60100 MPO89% UIP
Flores-Suárez et al. [19], 2015, MexicoSurvival in MPA patients with PF40 patients, 17 (42.5%) with PF54.2 (total cohort including non-PF)5342.510082 preceding43 (11–213)90 MPO-ANCA,
5 PR3,
2.5 MPO+PR3
88% UIP
Ono et al. [20], 2015, JapanCharacteristics of MPO-positive GPA patients14 patients with ILD out of 41 MPA patients72.3 (total cohort)4434100ND38.6100 MPA (patients positive for MPO-ANCA)100% UIP
Kagiyama et al. [21], 2015, JapanANCA positive conversion and MPA development in IPF patients504 PF, 36 ANCA+73611009/36 ANCA positive with MPA100 preceding2955 MPO,
45 PR3
ND
Hosoda et al. [22], 2016, JapanClinical features of UIP with ANCA compared to IPF12 ANCA/UIP patients65.2 (48–74)6710025 MPA100 preceding72 (14–195)100 MPO100% UIP
Hozumi et al. [23], 2016, JapanClinical implication of PR3 in patients with IIPs16 PR3+ of 360 IIP7275100NoneND22100 PR3 (MPO patients excluded)37.6% UIP/possible UIP, 31.3%
NSIP, 31.3% “unclassifiable” CT pattern
Tashiro et al. [24], 2017, JapanCharacteristics and prognosis of MPA with bronchiectasis23 patients with ILD out of 45 patients with MPA72±9.210051100 MPAND52.9 (1–125)100 MPOND
Hozumi et al. [25], 2018, JapanClinical significance of MPO in patients with IIPs26 MPO+ of 305 patients707710024.3 MPA100 preceding69100 MPO100% UIP/possible UIP
Juman et al. [26], 2019, UKILD associated with ANCA-positivity69 patients with ILD and 18 ANCA+675110025 coexisting AAV59 precedingND55 p-ANCA,
45 c-ANCA,
23 MPO,
12 PR3
ND
Baqir et al. [27], 2019, USARadiologic and pathologic characteristics of MPO-ILD58 (43–75)5610061 MPA61 (11/18) of patients with existing MPA, 3/18 developed MPA on follow up52100 MPO22% UIP,
29% NSIP,
7% OP
Liu et al. [28], 2019, USAPrevalence and significance of ANCA in IPF patients745 patients, 34 with ANCA+67.9±8.9531005/34 ANCA+ patients with MPA100 preceding18.353 MPO,
26 PR3,
18 ND
75% UIP/possible UIP
Watanbe et al. [29], 2019, JapanPrognosis of MPO-UIP in patients with AAV nephritis3174 (58–88)5210097 MPA,
3 GPA
All cases had pre-existing ANCA nephritisND100 MPO100% UIP
Maillet et al. [30], 2020, FranceUIP in AAV62 AAV-ILD665510085 MPA,
15 GPA
52 preceding, 39 concurrent, 10 after40.5 (21–68)89 MPO,
5 PR3
63% UIP
39% NSIP
Kwon et al. [31], 2020, USAILD in AAV patients24 (14 with MPA, 8 with GPA, 2 EGPA)73 (19–94)45.810058 MPA,
33 GPA,
8 EGPA
20.8 preceding, 45.8 concurrent, 33.3 after4266.7 MPO,
33.3 PR3
50% UIP/probable UIP, 41.7% alternative diagnosis (25% fibrotic HP pattern, 12.5% NSIP, 4.2% OP), 8.3% indeterminate
Sun et al. [32], 2021, ChinaClinical features and long-term outcomes of ILD with ANCA antibody80604538.7 MPA-ILD
61.25 isolated ANCA-IIP
38.75
MPA
ND4056.25 MPO,
86 p-ANCA,
2.5 PR3,
13.75 c-ANCA
8.75% UIP,
63.75% NSIP,
27.5% “unclassifiable”

AAV: ANCA-associated vasculitis; ANCA: anti-neutrophil cytoplasmic antibody; c-ANCA: cytoplasmic ANCA; AAV: ANCA-associated vasculitis; CT: computed tomography; DAD: diffuse alveolar damage; dx: diagnosis; EGPA: eosinophilic granulomatosis with polyangiitis; GN: glomerulonephritis; GPA: granulomatosis with polyangiitis; HP: hypersensitivity pneumonitis: HRCT: high-resolution computed tomography; IIP: idiopathic interstitial pneumonia; ILD: interstitial lung disease; IPF: idiopathic pulmonary fibrosis; MPA: microscopic polyangiitis; MPO: myeloperoxidase; ND: not described; NSIP: non-specific interstitial pneumonia, OP: organising pneumonia; p-ANCA: perinuclear ANCA; PF: pulmonary fibrosis; PR3: proteinase 3; UIP: usual interstitial pneumonia (the most common pattern of ANCA-ILD).