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Updated: Feb 6, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Morphologic Aspects of Interstitial Pneumonia With Autoimmune Features
Ellen Caroline Toledo do Nascimento, Bruno Guedes Baldi, Marcio Valente Yamada Sawamura
1Departamento de Patologia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr do Nascimento); Divisao de Pneumologia, Instituto do Coracao, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr Baldi); Departamento de Radiologia, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr Sawamura); and Departamento de Patologia, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil (Dr Dolhnikoff).
Interstitial pneumonia with autoimmune features (IPAF) identifies a distinct patient group. Further research is needed to define prognosis and management for IPAF patients with interstitial lung disease.
Area of Science:
- Pulmonology
- Rheumatology
- Pathology
Background:
- Interstitial lung disease (ILD) is a severe complication of connective tissue diseases.
- A subset of patients presents with ILD and autoimmune features but lacks a specific connective tissue disease diagnosis.
- Previous classifications for this group were inconsistent, leading to the 2015 introduction of interstitial pneumonia with autoimmune features (IPAF).
Purpose of the Study:
- To review diagnostic criteria for IPAF, focusing on morphologic features.
- To understand the diagnostic impact and prognostic significance of morphologic aspects in IPAF.
Main Methods:
- Literature review.
- Analysis of pathologic, radiologic, and clinical data.
- Synthesis of existing experience and evidence.
Main Results:
- IPAF classification appears to distinguish a unique patient subgroup with varying prognoses.
- Nonspecific interstitial pneumonia and usual interstitial pneumonia are common morphologic patterns in IPAF.
- The prognostic impact of the usual interstitial pneumonia pattern on survival in IPAF remains debated.
Conclusions:
- IPAF may represent a distinct clinical entity.
- Further prospective studies are essential for refining IPAF diagnosis and management.
- Clarifying the role of specific morphologic patterns is crucial for patient prognosis.
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