Clinical features and prognosis of microscopic polyangiitis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis

© 2019 John Wiley & Sons Ltd..

INTRODUCTION: Pulmonary involvement in microscopic polyangiitis (MPA) is common, little is known about the clinical features of MPA with interstitial pneumonia (MPA-IP).

OBJECTIVES: This study aimed to explore the prevalence of microscopic polyangiitis associated usual interstitial pneumonia (UIP)(MPA-UIP) and compare its clinical features and prognosis with those of MPA-non-UIP and idiopathic pulmonary fibrosis (IPF).

METHODS: A total of 73 patients with MPA-IP were identified and divided into MPA-UIP patients and MPA-non-UIP patients. The clinical characteristics and survival of MPA-UIP patients were analysed and compared with those of MPA-non-UIP patients and 68 patients with IPF.

RESULTS: The results showed that 34/73 (47%) MPA-IP patients were classified as MPA-UIP patients. Compared with MPA-non-UIP patients, MPA-UIP patients tend to have longer duration of symptoms prior to diagnosis and usually have pulmonary involvement as the initial presentation. However, they were less likely to have proteinuria and/or hematuria. Compared with IPF patients, MPA-UIP patients usually had multisystem damage, positive anti-neutrophil cytoplasmic autoantibodies and elevated levels of nonspecific inflammatory markers. MPA-UIP death was concentrated mainly in the first 3 months after diagnosis and resulted in a higher early mortality compared with IPF.

CONCLUSION: UIP is the most frequent type of MPA-IP. These patients tend to have longer duration of symptoms prior to diagnosis and usually have pulmonary involvement as the first presentation. However, they are less likely to have proteinuria and/or hematuria. MPA patients with UIP can be differentiated from IPF patients through comprehensive analysis of clinical and laboratory findings.

Medienart:

E-Artikel

Erscheinungsjahr:

2019

Erschienen:

2019

Enthalten in:

Zur Gesamtaufnahme - volume:13

Enthalten in:

The clinical respiratory journal - 13(2019), 7 vom: 17. Juli, Seite 460-466

Sprache:

Englisch

Beteiligte Personen:

Zhao, Wen [VerfasserIn]
Dai, Huaping [VerfasserIn]
Liu, Yan [VerfasserIn]
Zhu, Min [VerfasserIn]
Bao, Na [VerfasserIn]
Ban, Chengjun [VerfasserIn]
Zhang, Shu [VerfasserIn]
Ren, Yanhong [VerfasserIn]
Ye, Qiao [VerfasserIn]
Wang, Chen [VerfasserIn]

Links:

Volltext

Themen:

ANCA-associated vasculitis
Comparative Study
Idiopathic pulmonary fibrosis
Journal Article
Microscopic polyangiitis
Usual interstitial pneumonia

Anmerkungen:

Date Completed 08.01.2020

Date Revised 08.01.2020

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1111/crj.13032

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM296336246