Poor prognostic factors for relapse of interstitial lung disease in microscopic polyangiitis: the Japanese multicentre REVEAL cohort study
Related Experiment Video
Updated: Dec 24, 2025
Author Spotlight: Exploring the Role of Inflammation in the Co-occurrence of Primary Sjogren's Syndrome and Lung Adenocarcinoma
Published on: September 20, 2024
Shogo Matsuda1, Takuya Kotani2, Ayana Okazaki1
1Department of Internal Medicine IV, Division of Rheumatology, Osaka Medical and Pharmaceutical University, Daigaku-Machi 2-7, Takatsuki, Osaka, 569-8686, Japan.
Background:
This study investigated poor prognostic factors for the relapse of interstitial lung disease (ILD) in patients with microscopic polyangiitis (MPA) after remission induction therapy.
Methods:
We enrolled patients diagnosed with MPA complicated by ILD according to the Chapel Hill Consensus definition from 2001 to 2023 in multiple institutions in the REVEAL cohort. All patients who were treated with immunosuppressive therapy were followed up, and those who relapsed with ILD were extracted in this study. We explored the risk factors for predicting ILD relapse in patients with MPA-ILD by comparing the demographic, clinical, laboratory, and radiological findings and treatments between the relapsed and non-relapsed groups on admission.
Results:
Of 243 patients with MPA, 134 (55.1%) with MPA-ILD were enrolled. Among them, 28 (20.9%) relapsed during a mean follow-up of 4.2 years. The initial serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) levels and the prevalence of usual interstitial pneumonia (UIP) pattern were significantly higher in the relapsed group. The biomarkers were also risk factors for relapse in multivariate Cox regression analysis. The best cut-off values of KL-6, SP-D for predicting ILD relapse were 430 U/mL and 89.5 ng/mL, respectively. We created prediction models based on the best cut-off values for KL-6, SP-D, and the presence of the UIP pattern (KSU model). The 10-year relapse rate was significantly different among patients with MPA-ILD stratified by the number of risk factors based on the KSU model. A higher relapse rate was associated with higher all-cause mortality.
Conclusions:
The initial serum high KL-6 and SP-D levels and the prevalence of the UIP pattern were associated with ILD relapse in patients with MPA-ILD. Our multicentre cohort study indicated that the KSU model, which consists of KL-6 ≥ 430 U/mL, SP-D ≥ 89.5 ng/mL, and the presence of the UIP pattern, is a useful predictor of ILD relapse in patients with MPA after immunosuppressive therapy.
Insights
High initial levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D), along with a usual interstitial pneumonia (UIP) pattern, predict interstitial lung disease (ILD) relapse in microscopic polyangiitis (MPA) patients. The KSU model effectively identifies patients at higher risk for relapse and mortality.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is an autoimmune vasculitis that can affect the lungs, leading to interstitial lung disease (ILD).
- Predicting relapse of ILD in MPA patients after initial treatment is crucial for long-term management and prognosis.
- Identifying poor prognostic factors can guide therapeutic strategies and patient monitoring.
Purpose of the Study:
- To investigate prognostic factors associated with the relapse of interstitial lung disease (ILD) in patients with microscopic polyangiitis (MPA).
- To identify clinical, laboratory, and radiological predictors of ILD relapse following remission induction therapy.
- To develop and validate a predictive model for ILD relapse in MPA patients.
Main Methods:
- A multicentre cohort study enrolled patients diagnosed with MPA complicated by ILD.
- Patients received immunosuppressive therapy, and follow-up data were collected to identify ILD relapses.
- Demographic, clinical, laboratory (including KL-6 and SP-D), and radiological findings were compared between relapsed and non-relapsed groups.
Main Results:
- Of 134 MPA-ILD patients, 20.9% experienced ILD relapse over a mean follow-up of 4.2 years.
- Higher initial serum Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) levels, and the presence of a usual interstitial pneumonia (UIP) pattern were significantly associated with relapse.
- The developed KSU model (KL-6 ≥ 430 U/mL, SP-D ≥ 89.5 ng/mL, UIP pattern) stratified patients by 10-year relapse risk, with higher risk linked to increased all-cause mortality.
Conclusions:
- Initial high KL-6 and SP-D levels, and the UIP pattern are significant predictors of ILD relapse in MPA patients.
- The KSU model demonstrates utility in predicting ILD relapse risk in MPA patients treated with immunosuppressive therapy.
- This predictive model aids in stratifying patients and potentially guiding more intensive management for high-risk individuals.
Related Concept Videos
01:30Acute Kidney Injury IV: Diagnostic Studies and Prevention
01:20COPD: Pathogenesis and Clinical Features
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
01:27Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History
01:17Factors Affecting Renal Clearance: Renal Impairment
One condition associated with renal failure is uremia. Uremia is characterized by impaired glomerular filtration and fluid accumulation in the body. This condition hinders the renal clearance of drugs, resulting in drug accumulation and potential...