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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Clinical features and survival analysis of microscopic polyangiitis-associated interstitial lung disease:a
1Department of Respiratory and Critical Care Medicine, the Affiliated Hospital of Medical School of Ningbo University, Ningbo 315020, China.
Abstract:
Objective: To explore the clinical features, laboratory examination and imaging features of microscopic polyangiitis (MPA)-associated interstitial lung disease (ILD), and to perform survival analysis. Methods: The records of 28 patients with MPA-ILD who were treated at the Affiliated Hospital of Medical School of Ningbo University were reviewed retrospectively from August 2014 to November 2021. The patients' clinical features, laboratory parameters, pulmonary function test, echocardiography, chest CT scan findings and therapeutic regimen were analyzed, and the relevant data were statistically analyzed. Results: There were 18 males and 10 females, with an average age of (70.1±9.3) years. Among them, 13 patients had a history of smoking. The main clinical manifestations were cough (14/28), fever (12/28), chest tightness, shortness of breath (12/28) and hemoptysis (3/28). Sixteen patients had renal involvement, and 78.57% (22/28) and 89.28% (25/28) of the patients had elevated C-reactive protein (CRP) and ESR respectively. Sixteen (16/28) patients had increased rheumatoid factor (RF), and the positive rate of myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) was 82.12% (23/28). 96.43% (27/28) of ILDs were diagnosed before or at the same time as MPA. The chest radiological pattern was mainly usual interstitial pneumonia (UIP) or UIP-like (15/28), followed by nonspecific interstitial pneumonia (NSIP) (8/28). Compared with non-UIP-like patients, UIP or UIP-like patients were older (P=0.018), and had higher serum LDH level (P=0.041), but serum creatinine level was significantly lower (P=0.041). Univariate and multivariate survival analysis showed that inappropriate treatment (HR=9.81, 95%CI: 1.68-57.29, P=0.011) and elevated serum LDH (HR=4.11, 95%CI: 0.99-17.00, P=0.051) were independent risk factors for shortened survival of MPA-ILD, while elevated RF (HR=0.22, 95%CI: 0.06-0.91, P=0.037) was a protective factor for prolonged survival. Conclusions: MPA-ILD patients had fewer systemic vasculitis symptoms. Most of the ILD patients were diagnosed before or at the same time as MPA. The chest radiological pattern was mainly UIP or UIP-like, followed by NSIP. Early use of glucocorticoids combined with immunosuppressant or rituximab could improve the survival rate of MPA-ILD. The elevated serum LDH was an independent risk factor for shortened survival of MPA-ILD, while elevated RF was a protective factor for prolonged survival.
Insights
Microscopic polyangiitis-associated interstitial lung disease (MPA-ILD) often presents with few systemic symptoms and typically shows usual interstitial pneumonia patterns on imaging. Early treatment with immunosuppressants improves survival, while elevated LDH indicates a poorer prognosis.
Area of Science:
- Rheumatology
- Pulmonology
- Internal Medicine
Background:
- Microscopic polyangiitis (MPA) is a systemic vasculitis that can affect the lungs, leading to interstitial lung disease (ILD).
- Understanding the clinical, laboratory, and imaging features of MPA-associated ILD (MPA-ILD) is crucial for diagnosis and management.
- Survival analysis in MPA-ILD can identify prognostic factors and guide therapeutic strategies.
Purpose of the Study:
- To investigate the clinical, laboratory, and imaging characteristics of patients with MPA-ILD.
- To analyze the survival outcomes of MPA-ILD patients.
- To identify independent risk factors influencing survival in MPA-ILD.
Main Methods:
- Retrospective review of 28 patients diagnosed with MPA-ILD.
- Analysis of clinical manifestations, laboratory parameters (CRP, ESR, RF, MPO-ANCA), pulmonary function, echocardiography, and chest CT findings.
- Statistical analysis including univariate and multivariate survival analysis.
Main Results:
- The majority of MPA-ILD patients presented with cough, fever, and shortness of breath, with common renal involvement.
- Elevated CRP, ESR, and MPO-ANCA were frequently observed; chest CT patterns were predominantly usual interstitial pneumonia (UIP) or UIP-like.
- Inappropriate treatment and elevated serum lactate dehydrogenase (LDH) were independent risk factors for shortened survival, while elevated rheumatoid factor (RF) was protective.
Conclusions:
- MPA-ILD patients often exhibit limited systemic vasculitis symptoms, with ILD frequently preceding or coinciding with MPA diagnosis.
- The predominant radiological pattern is UIP or UIP-like, necessitating early diagnosis and intervention.
- Prompt initiation of glucocorticoids with immunosuppressants or rituximab, alongside monitoring LDH and RF levels, can improve MPA-ILD patient survival.
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