Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Mar;19(3):e70061.
doi: 10.1111/crj.70061.

Differences in Radiological and Pathological Findings by ANCA-Subtype in ANCA-Positive Idiopathic Interstitial Pneumonias

Tetsuro Sawata  1   2 , Susumu Sakamoto  2 , Yusuke Usui  2 , Aika Suzuki  2 , Hideya Kitamura  3 , Tae Iwasawa  4 , Shoichiro Matsushita  5 , Yasuhiro Terasaki  6   7 , Shinobu Kunugi  6 , Kazuma Kishi  2 , Tomoyuki Fujisawa  8 , Takafumi Suda  8 , Sakae Homma  2   9
Affiliations

Differences in Radiological and Pathological Findings by ANCA-Subtype in ANCA-Positive Idiopathic Interstitial Pneumonias

Tetsuro Sawata et al. Clin Respir J. 2025 Mar.

Abstract

Introduction: Anti-neutrophil cytoplasmic antibody (ANCA) seropositivity strongly correlates to ANCA-associated vasculitis. Patients with idiopathic interstitial pneumonias (IIPs) without systemic vasculitis are sometimes ANCA-positive. Radiological and pathological differences between patients with myeloperoxidase (MPO)-ANCA-positive and those with proteinase 3 (PR3)-ANCA-positive IIPs remain unclear. To determine whether high-resolution computed tomography (HRCT) features and pathology findings differ by ANCA subtype in ANCA-positive IIP patients in a national database. Clinical, radiological, and pathological data were examined along with a web-based multidisciplinary discussion.

Methods: We reviewed records of 10 MPO-ANCA-positive and 9 PR3-ANCA-positive IIP patients who underwent HRCT and surgical lung biopsy between April 2009 and March 2014. Pulmonologists, chest radiologists, and pathologists evaluated HRCT scans and pathological findings independently. Patterns were classified using ATS/ERS/JRS/ALAT 2011 guidelines for idiopathic pulmonary fibrosis.

Results: HRCT patterns were definite usual interstitial pneumonia (UIP) (n = 8; 42.1%), possible UIP (n = 6; 31.6%), and inconsistent with UIP (n = 5; 26.3%). Pathological patterns were definite UIP (n = 5; 26.3%), probable UIP (n = 8; 42.1%), possible UIP (n = 4; 21.1%), and not UIP (n = 2; 10.5%). HRCT and pathological patterns did not differ between MPO-ANCA-positive and PR3-ANCA-positive IIPs. Radiological features were reticulation (n = 13; 68.4%), nodules (n = 12; 63.1%), honeycombing (n = 10; 52.6%), and increased attenuation around honeycombing (n = 7; 36.8%). Pathological findings were cysts (n = 12; 63.1%), lymphoid follicles with germinal centers (n = 11; 57.9%), and peribronchiolar wall lymphocytic infiltration (n = 11; 57.9%).

Conclusion: HRCT and pathological patterns did not differ between MPO-ANCA-positive and PR3-ANCA-positive IIPs. This absence of significant differences suggests a similar mechanism underlying both types of interstitial pneumonia.

Keywords: anti‐neutrophil cytoplasmic antibody (ANCA); idiopathic interstitial pneumonias; myeloperoxidase (MPO)‐ANCA; proteinase 3 (PR3)‐ANCA.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Flow diagram of patient enrollment.
FIGURE 2
FIGURE 2
Radiological findings of nodular lesions.
FIGURE 3
FIGURE 3
Radiological findings of increased attenuation around honeycombing.
FIGURE 4
FIGURE 4
Pathological findings of nodular lesions on HRCT image showing lymphocytic infiltration and lymphoid follicles with germinal center around bronchiolar walls.
FIGURE 5
FIGURE 5
Pathological findings of increased attenuation around honeycombing on HRCT image showing lymphocytic infiltration of the bronchiolar walls inducing destructive bronchiolitis resulting cyst formation.
See this image and copyright information in PMC

References

    1. Jennette J. C., Wilkman A. S., and Falk R. J., “Anti‐Neutrophil Cytoplasmic Autoantibody‐Associated Glomerulonephritis and Vasculitis,” American Journal of Pathology 135 (1989): 921–930. - PMC - PubMed
    1. van der Woude F. J., Rasmussen N., Lobatto S., et al., “Autoantibodies Against Neutrophils and Monocytes: Tool for Diagnosis and Marker of Disease Activity in Wegener's Granulomatosis,” Lancet 23 (1985): 425–429. - PubMed
    1. Eschun G. M., Mink S. N., and Sharma S., “Pulmonary Interstitial Fibrosis as a Presenting Manifestation in Perinuclear Antineutrophilic Cytoplasmic Antibody Microscopic Polyangiitis,” Chest 123 (2003): 297–301. - PubMed
    1. Birnbaum J., Danoff S., Askin F. B., and Stone J. H., “Microscopic Polyangiitis Presenting as a “Pulmonary‐Muscle” Syndrome: Is Subclinical Alveolar Hemorrhage the Mechanism of Pulmonary Fibrosis?,” Arthritis and Rheumatism 56 (2007): 2065–2071. - PubMed
    1. Foulon G., Delaval P., Valeyre D., et al., “ANCA‐Associated Lung Fibrosis: Analysis of 17 Patients,” Respiratory Medicine 102 (2008): 1392–1398. - PubMed

MeSH terms

LinkOut - more resources