Published studies of anti-neutrophil cytoplasmic antibody associated interstitial lung disease
| Reference, year, country | Study description | Number of patients | Mean age (years) | Male (%) | ILD (%) | Systemic vasculitis (%) | PF preceding AAV (%) | Median follow-up period (months) | ANCA specificity (%) | HRCT pattern |
| Nada et al. [2], 1990, USA | PF in patients with pulmonary-renal vasculitis | 3 | 73 | 33 | 100 | 100 MPA | 33 preceding, 67 concurrent | 120 | 67 p-ANCA | ND |
| Arimura et al. [3], 1995, Japan | Pulmonary involvement in patients with MPO-ANCA | 46 | 61.1 | ND | 43 | 28 MPA | ND | ND | 100 MPO | ND |
| Hiromura et al. [4], 2000, Japan | MPO+rapidly progressive GN during course of IPF | 4 | 67 | 50 | 100 | 100 MPA | 100 | ND | 100 MPO | ND |
| Eschun et al. [5], 2003, Canada | PF presenting manifestation of MPA | 6 | 69.8 (63–78) | 50 | 100 | 100 MPA | 100 | 36 | 100 p-ANCA | 3/6 UIP-like, 2/6 fibrotic NSIP, 1 ND |
| Homma et al. [6], 2004, Japan | PF in MPO-ANCA | 31 | 69 | 55 | 100 | 25 MPA | 100 | 120 | 100 MPO | 84% UIP |
| Foulon et al. [7], 2008, France | ANCA-associated PF | 17 | 66 | 71 | 100 | 41 MPA | 86 preceding, 14 concurrent | 57±41 | 6/17 MPO, 1/17 PR3 | 100% UIP |
| Nozu et al. [8], 2009, Japan | Comparison of ANCA-positive and -negative patients with PF | 19/53 with ANCA+ PF, 34/53 ANCA− PF | 69 (52–80) | 58 | 100 | 21 MPA | 100 preceding | 1–90 | 17/19 MPO, 2/19 PR3 | 73.3% UIP |
| Hervier et al. [9], 2009, France | PF associated with AAV | 12 | 70.7 | 75 | 100 | 83 MPA, 17 GPA | 66 concurrent, 25 preceding, 8 following AAV | 49.2 (7–116) | 100 MPO 100 | 6/12 UIP, 1/12 NSIP, 5/12 ND |
| Tzelepis et al. [10], 2010, Greece | Prevalence and outcome of PF in MPA | 36 | 57 | 69 | 39 | 100 MPA | 92 concurrent, 8 following AAV | 38±30 | 85 p-ANCA, 8 p-ANCA+c-ANCA | 54% UIP, 31% NSIP |
| Arulkumaran et al. [11], 2011, UK | ILD and AAV | 14 | 67.3 | 71 | 100 | 100 MPA | 14 preceding, 64 concurrent, 21 post | 90 | 100 MPO | ND |
| Tanaka et al. [12], 2012, Japan | IP associated with MPO- ANCA | 9 | 62.1 | 66 | 100 | None | ND | 39.1 | 100 MPO | 66% UIP, 11% NSIP, 11% OP, 11% DAD |
| Ahn et al. [13], 2012, Korea | Clinical features and outcomes of MPA | 55 patients with MPA, 13 with ILD (23.6%), | 59.29±13.60 MPA, ND in ILD patients | 60% MPA, ND in ILD patients | 24 | 100 MPA | ND | 46.07 ±39.98 | 100 MPO | 84.6% UIP, 1 patient OP, 2 patients NSIP |
| Ando et al. [14], 2013, Japan | Incidence of MPO-ANCA and MPA in course of IPF | 61 patients with initial dx IPF, 9/61 ANCA+ | 69 (57–75) | 75 | 15 | 22 (2/9) MPA | 100 preceding | 40 (1–121) | 100 MPO | 78% UIP, 22% ND |
| Comarmond et al. [15], 2014, France | PF in AAV | 49 | 66 (57–72) | 61 | 100 | 82 MPA, 18 GPA | 45 preceding, 43 concurrent, 12 post | 48 (14–88) | 88 MPO, 4 PR3 | 43% “typical” UIP, 14% “atypical” UIP, 7% fibrotic NSIP, 9.5% NSIP |
| Huang et al. [16], 2014, China | MPA+PF | 19 | 63.6 | 42 | 100 | 100 MPA | 68 preceding, 32 concurrent | 29.9 (8–93) | 100 MPO | 100% UIP |
| Yu et al. [17], 2014, China | CT image analysis before and after treatment of ANCA-ILD | 8 | 72.6 | 88 | 100 | ND | ND | ND | 75 MPO, 12.5 PR3+MPO, 12.5 PR3 | ND |
| Fernandez Casares et al. [18], 2015, Argentina | MPA associated with PF | 9 patients with PF out of 28 MPA patients | 60±14 | 56 | 32 | 100 | 56 preceding, 44 concurrent | 76±60 | 100 MPO | 89% UIP |
| Flores-Suárez et al. [19], 2015, Mexico | Survival in MPA patients with PF | 40 patients, 17 (42.5%) with PF | 54.2 (total cohort including non-PF) | 53 | 42.5 | 100 | 82 preceding | 43 (11–213) | 90 MPO-ANCA, 5 PR3, 2.5 MPO+PR3 | 88% UIP |
| Ono et al. [20], 2015, Japan | Characteristics of MPO-positive GPA patients | 14 patients with ILD out of 41 MPA patients | 72.3 (total cohort) | 44 | 34 | 100 | ND | 38.6 | 100 MPA (patients positive for MPO-ANCA) | 100% UIP |
| Kagiyama et al. [21], 2015, Japan | ANCA positive conversion and MPA development in IPF patients | 504 PF, 36 ANCA+ | 73 | 61 | 100 | 9/36 ANCA positive with MPA | 100 preceding | 29 | 55 MPO, 45 PR3 | ND |
| Hosoda et al. [22], 2016, Japan | Clinical features of UIP with ANCA compared to IPF | 12 ANCA/UIP patients | 65.2 (48–74) | 67 | 100 | 25 MPA | 100 preceding | 72 (14–195) | 100 MPO | 100% UIP |
| Hozumi et al. [23], 2016, Japan | Clinical implication of PR3 in patients with IIPs | 16 PR3+ of 360 IIP | 72 | 75 | 100 | None | ND | 22 | 100 PR3 (MPO patients excluded) | 37.6% UIP/possible UIP, 31.3% NSIP, 31.3% “unclassifiable” CT pattern |
| Tashiro et al. [24], 2017, Japan | Characteristics and prognosis of MPA with bronchiectasis | 23 patients with ILD out of 45 patients with MPA | 72±9.2 | 100 | 51 | 100 MPA | ND | 52.9 (1–125) | 100 MPO | ND |
| Hozumi et al. [25], 2018, Japan | Clinical significance of MPO in patients with IIPs | 26 MPO+ of 305 patients | 70 | 77 | 100 | 24.3 MPA | 100 preceding | 69 | 100 MPO | 100% UIP/possible UIP |
| Juman et al. [26], 2019, UK | ILD associated with ANCA-positivity | 69 patients with ILD and 18 ANCA+ | 67 | 51 | 100 | 25 coexisting AAV | 59 preceding | ND | 55 p-ANCA, 45 c-ANCA, 23 MPO, 12 PR3 | ND |
| Baqir et al. [27], 2019, USA | Radiologic and pathologic characteristics of MPO-ILD | 58 (43–75) | 56 | 100 | 61 MPA | 61 (11/18) of patients with existing MPA, 3/18 developed MPA on follow up | 52 | 100 MPO | 22% UIP, 29% NSIP, 7% OP | |
| Liu et al. [28], 2019, USA | Prevalence and significance of ANCA in IPF patients | 745 patients, 34 with ANCA+ | 67.9±8.9 | 53 | 100 | 5/34 ANCA+ patients with MPA | 100 preceding | 18.3 | 53 MPO, 26 PR3, 18 ND | 75% UIP/possible UIP |
| Watanbe et al. [29], 2019, Japan | Prognosis of MPO-UIP in patients with AAV nephritis | 31 | 74 (58–88) | 52 | 100 | 97 MPA, 3 GPA | All cases had pre-existing ANCA nephritis | ND | 100 MPO | 100% UIP |
| Maillet et al. [30], 2020, France | UIP in AAV | 62 AAV-ILD | 66 | 55 | 100 | 85 MPA, 15 GPA | 52 preceding, 39 concurrent, 10 after | 40.5 (21–68) | 89 MPO, 5 PR3 | 63% UIP 39% NSIP |
| Kwon et al. [31], 2020, USA | ILD in AAV patients | 24 (14 with MPA, 8 with GPA, 2 EGPA) | 73 (19–94) | 45.8 | 100 | 58 MPA, 33 GPA, 8 EGPA | 20.8 preceding, 45.8 concurrent, 33.3 after | 42 | 66.7 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, China | Clinical features and long-term outcomes of ILD with ANCA antibody | 80 | 60 | 45 | 38.7 MPA-ILD 61.25 isolated ANCA-IIP | 38.75 MPA | ND | 40 | 56.25 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).