Clinical Implication of Proteinase-3-antineutrophil Cytoplasmic Antibody in Patients with Idiopathic Interstitial Pneumonias.

Abstract

PURPOSE:

The clinical significance of proteinase-3-antineutrophil cytoplasmic antibody (PR3-ANCA) positivity is not well established in idiopathic interstitial pneumonia (IIP) patients. We aimed to determine the clinical features of PR3-ANCA-positive IIP patients.

METHODS:

We retrospectively reviewed 377 consecutive IIP patients; of these, 360 patients had PR3-ANCA and myeloperoxidase-antineutrophil cytoplasmic antibody test results available. The clinical features of PR3-ANCA-positive IIP patients and control ANCA-negative idiopathic pulmonary fibrosis patients (ANCA-negative IPF) were compared.

RESULTS:

Sixteen patients (4.4 %) were PR3-ANCA-positive IIP and 94 (26 %) were ANCA-negative IPF. The median age at diagnosis (72 vs. 70 years, P = 0.17) and proportion of males (75 vs. 89 %, P = 0.12) in PR3-ANCA-positive IIP and ANCA-negative IPF patients, respectively, were not significantly different. Radiologically, the HRCT patterns of PR3-ANCA-positive IIP patients varied (UIP, n = 3, 18.8 %; possible UIP, n = 3, 18.8 %; NSIP, n = 5, 31.3 %; unclassifiable CT pattern, n = 5, 31.3 %) more than those of ANCA-negative IPF patients (UIP, n = 69, 73.4 %; possible UIP, n = 25, 26.6 %; P < 0.001). No PR3-ANCA-positive IIP patients developed ANCA-associated vasculitis. The 5-year survival rate was 50 % in PR3-ANCA-positive IIP patients and 52 % in ANCA-negative IPF patients with no significant difference (P = 0.96 by log-rank test).

CONCLUSIONS:

The HRCT patterns of PR3-ANCA-positive IIP patients varied more than those of the IPF patients, but the clinical features of high IIP-onset age and male predominance were similar between the groups. Furthermore, PR3-ANCA-positive IIP patients had a poor prognosis similar to that of IPF patients.

• Authors: Hironao Hozumi, Noriyuki Enomoto, Yoshiyuki Oyama, Masato Kono, Tomoyuki Fujisawa, Naoki Inui, Yutaro Nakamura, Takafumi Suda
• Journal: Lung (Lung) Vol. 194 Issue 2 Pg. 235-42 (Apr 2016) ISSN: 1432-1750 [Electronic] United States
• PMID: 26873743 (Publication Type: Journal Article)

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Biomarkers
• Myeloblastin

Topics

• Aged
• Aged, 80 and over
• Antibodies, Antineutrophil Cytoplasmic (blood)
• Biomarkers (blood)
• Biopsy
• Enzyme-Linked Immunosorbent Assay
• Female
• Humans
• Idiopathic Interstitial Pneumonias (blood, diagnosis, immunology, mortality)
• Kaplan-Meier Estimate
• Lung (diagnostic imaging, pathology)
• Male
• Middle Aged
• Myeloblastin (immunology)
• Prognosis
• Retrospective Studies
• Time Factors
• Tomography, X-Ray Computed