Interstitial Pneumonia With Autoimmune Features (IPAF)

Frontiers in Medicine

article

Ligia Fernandes¹  Mouhamad Nasser²  Kais Ahmad²  Vincent Cottin³ 

[1] Departamento do Tórax, Centro Hospitalar Lisboa Norte;Department of Respiratory Medicine, National Reference Center for Rare Pulmonary Diseases;Claude Bernard Lyon 1 University, University of Lyon

关键词: pulmonary fibrosis;    connective tissue disease;    classification;    autoimmunity;    antibody;   

DOI  :  10.3389/fmed.2019.00209

学科分类：社会科学、人文和艺术（综合）

来源: Frontiers

【 摘 要 】

A significant proportion of patients with interstitial lung disease (ILD) manifest autoimmune features, but do not fulfill the diagnostic criteria for a definite connective tissue disease (CTD). In 2015, the European Respiratory Society (ERS) and American Thoracic Society (ATS) “Task Force on undifferentiated Forms of connective tissue disease-associated interstitial lung disease” proposed classification criteria for a so-called research category of Interstitial Pneumonia with Autoimmune Features (IPAF). These classification criteria were based on a combination of features from three domains: a clinical domain consisting of extra-thoracic features; a serologic domain with specific autoantibodies; and a morphologic domain with imaging patterns, histopathological findings or multi-compartment involvement. Patients meeting IPAF criteria tend to have a history of smoking similar to patients with idiopathic pulmonary fibrosis. The most frequent clinical and serological markers of autoimmune features are Raynaud' phenomenon and positive antinuclear antibodies, respectively. Non-specific interstitial pneumonia is the predominant radiologic and histopathologic pattern, although patients meeting IPAF criteria through the clinical and serologic domains may also have a usual interstitial pneumonia pattern. Management should be carefully individualized on a case-by-case basis in keeping with the wide heterogeneity of IPAF and lack of evidence in this particular subgroup of patients. Prognosis is generally intermediate between that of idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease, but substantially variable according to the predominant histologic and radiologic patterns. As acknowledged by the Task Force, the proposed classification scheme of IPAF is a research concept that will need revision and refinement based on data to better inform prognostication and patient care.

【 授权许可】

CC BY