Clinical features and long-term outcomes of interstitial lung disease with anti-neutrophil cytoplasmic antibody
BACKGROUND: Patients with interstitial lung disease (ILD) are occasionally positive for anti-neutrophil cytoplasmic antibodies (ANCAs). Differences between ILDs secondary to microscopic polyangiitis (MPA) and isolated ANCA-positive idiopathic interstitial pneumonia (IIP) remain unclear. The aim of this study was to explore the differences in clinical features and outcomes between MPA-associated ILDs and isolated ANCA-positive IIPs.
METHODS: We reviewed 1338 ILDs patients with available ANCA results and retrospectively analysed 80 patients who were ANCA-positive. MPA-associated ILDs (MPA-ILDs group) and isolated ANCA-positive IIPs (ANCA-IIPs group) were compared.
RESULTS: Among 80 patients with ANCA-positive ILDs, 31 (38.75%) had MPA-ILDs, and 49 (61.25%) had isolated ANCA-positive IIPs. Compared with ANCA-IIPs group, patients in MPA-ILDs group had a higher proportion of fever (p = 0.006) and higher neutrophil count (p = 0.011), erythrocyte sedimentation rate (ESR) (p < 0.001) and C-reactive protein (CRP) (p = 0.005). Multivariable analysis showed that ESR level was an independent risk factor for mortality in all 80 ANCA-positive ILDs patients (HR 1.028, p = 0.001). Survival in MPA-ILDs group was lower than that in ANCA-IIPs group, and further stratified analysis revealed that ANCA-IIPs patients with elevated ESR or CRP had a worse prognosis than those with normal inflammation markers, with 5-year cumulative survival rates of 60.00%, 86.90% and 100.00% in MPA-ILDs and ANCA-IIPs with and without elevated inflammation markers, respectively.
CONCLUSIONS: Among patients with ANCA-positive ILDs, the prognoses of ANCA-IIPs with normal inflammation markers, ANCA-IIPs with elevated inflammation markers and MPA-ILDs were sequentially poorer. Therefore, stratified treatment should be considered in the management of ILDs patients positive for ANCAs.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2021 |
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| Erschienen: |
2021 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:21 |
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| Enthalten in: |
BMC pulmonary medicine - 21(2021), 1 vom: 16. März, Seite 88 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Sun, Xin [VerfasserIn] |
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| Links: |
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| Themen: |
Anti-neutrophil cytoplasmic antibody |
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| Anmerkungen: |
Date Completed 12.10.2021 Date Revised 12.10.2021 published: Electronic Citation Status MEDLINE |
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| doi: |
10.1186/s12890-021-01451-4 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| 500 | |a Date Revised 12.10.2021 | ||
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| 500 | |a Citation Status MEDLINE | ||
| 520 | |a BACKGROUND: Patients with interstitial lung disease (ILD) are occasionally positive for anti-neutrophil cytoplasmic antibodies (ANCAs). Differences between ILDs secondary to microscopic polyangiitis (MPA) and isolated ANCA-positive idiopathic interstitial pneumonia (IIP) remain unclear. The aim of this study was to explore the differences in clinical features and outcomes between MPA-associated ILDs and isolated ANCA-positive IIPs | ||
| 520 | |a METHODS: We reviewed 1338 ILDs patients with available ANCA results and retrospectively analysed 80 patients who were ANCA-positive. MPA-associated ILDs (MPA-ILDs group) and isolated ANCA-positive IIPs (ANCA-IIPs group) were compared | ||
| 520 | |a RESULTS: Among 80 patients with ANCA-positive ILDs, 31 (38.75%) had MPA-ILDs, and 49 (61.25%) had isolated ANCA-positive IIPs. Compared with ANCA-IIPs group, patients in MPA-ILDs group had a higher proportion of fever (p = 0.006) and higher neutrophil count (p = 0.011), erythrocyte sedimentation rate (ESR) (p < 0.001) and C-reactive protein (CRP) (p = 0.005). Multivariable analysis showed that ESR level was an independent risk factor for mortality in all 80 ANCA-positive ILDs patients (HR 1.028, p = 0.001). Survival in MPA-ILDs group was lower than that in ANCA-IIPs group, and further stratified analysis revealed that ANCA-IIPs patients with elevated ESR or CRP had a worse prognosis than those with normal inflammation markers, with 5-year cumulative survival rates of 60.00%, 86.90% and 100.00% in MPA-ILDs and ANCA-IIPs with and without elevated inflammation markers, respectively | ||
| 520 | |a CONCLUSIONS: Among patients with ANCA-positive ILDs, the prognoses of ANCA-IIPs with normal inflammation markers, ANCA-IIPs with elevated inflammation markers and MPA-ILDs were sequentially poorer. Therefore, stratified treatment should be considered in the management of ILDs patients positive for ANCAs | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Anti-neutrophil cytoplasmic antibody | |
| 650 | 4 | |a Idiopathic interstitial pneumonia | |
| 650 | 4 | |a Interstitial lung disease | |
| 650 | 4 | |a Microscopic polyangiitis | |
| 650 | 7 | |a Antibodies, Antineutrophil Cytoplasmic |2 NLM | |
| 700 | 1 | |a Peng, Min |e verfasserin |4 aut | |
| 700 | 1 | |a Zhang, Ting |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Zongru |e verfasserin |4 aut | |
| 700 | 1 | |a Song, Lan |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Mengtao |e verfasserin |4 aut | |
| 700 | 1 | |a Shi, Juhong |e verfasserin |4 aut | |
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