Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study

Karampeli, Maria; Thomas, Konstantinos; Flouda, S.; Chavatza, Aikaterina; Nikolopoulos, Dionysis; Pieta, Antigone; Tseronis, D.; Aggelakos, M.; Kassara, Dimitra; Tzavara, Vassiliki; Katsimbri, Pelagia; Boumpas, Dimitrios; Karageorgas, Theofanis

doi:10.31138/mjr.31.3.330

Cited by 13 publications

(12 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The inclusion of the seven specific autoimmune features in the definition of IPAF, while excluding other clinical features with a possible autoimmune etiology, has not been well rationalized. In an IPAF cohort from Greece, a morbilliform skin rash was reported as a common finding, while another study from Italy reported sicca features as a common association [23,25]. Although present in a few cases, other autoimmune features such as photosensitive skin rash, nail cuticular changes, subclinical myositis, history of AIHA and immune thrombocytopenia were encountered in our cohort.…”

Section: Discussionmentioning

confidence: 51%

“…A positive ANA was the most common serologic finding in the present study, followed by anti Ro52 and anti tRNA synthetase. ANA positivity and anti Ro52 were also the most common serologic features in the cohort from Greece [25]. ANA, when repeated at 1:320 dilution, also yielded positive results, with slight change in intensity and an occasional unmasking of a different pattern.…”

Section: Discussionmentioning

confidence: 76%

“…Ten patients in our cohort with anti-Ro52 tended to have severe lung disease, with a shorter duration of illness and displayed RP more frequently. Anti-Ro52 in other cohorts was seen to be associated with sicca symptoms, with few patients developing Sjogren's syndrome on follow-up [23,25].…”

Section: Discussionmentioning

confidence: 84%

See 2 more Smart Citations

Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study

et al. 2021

View full text Add to dashboard Cite

The current study aimed to characterize patients from a rheumatology referral center in north India, who satisfied the definition of interstitial pneumonia with autoimmune features (IPAF) as given by the American Thoracic Society and European Respiratory Society (ATS/ERS) consensus committee in 2015. Thirty-five adult patients aged 18 years and above, fulfilling the 2015 ATS/ERS criteria for IPAF were included in the study. The clinical and immunological profile, and radiologic findings on high-resolution computerized tomography thorax were noted. Antinuclear antibody (ANA) by indirect immunofluorescence at 1:320 titer and myositis-specific antibody (MSA) assays were performed. Non-parametric tests were used to compare variables between groups. The study cohort included predominantly female patients with a mean age of 50.6 ± 13 years and mean duration of disease of 38.8 ± 28.4 months. Majority of patients (49%) fulfilled the morphologic and serologic domains as per the IPAF consensus criteria and 31% patients had features in all three domains. Non-specific interstitial pneumonia was the most common pattern observed in 77% patients. Raynaud's phenomenon and inflammatory arthritis were the predominant autoimmune features. Pulmonary arterial hypertension was documented in 60% of patients on echocardiography. Positive ANA at 1:320 dilution was present in all 26 patients tested, whereas extractable nuclear antigen and MSA assays detected autoantibodies in 49% and 51% of patients respectively. IPAF predominantly affected females in the age group of 50 years and above, with varied autoimmune manifestations and autoantibody profile.

show abstract

Section: Discussionmentioning

confidence: 51%

Section: Discussionmentioning

confidence: 76%

See 1 more Smart Citation

Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study

et al. 2021

View full text Add to dashboard Cite

show abstract

“…IPAF patients were included in both trials. Progressive phenotype occurs only in a proportion of IPAF patients [ 62 ]. Therefore, it is essential for the recruitment to antifibrotic treatment to prove the risk of pulmonary fibrosis progression.…”

Section: Discussionmentioning

confidence: 99%

Review: Serum Biomarkers of Lung Fibrosis in Interstitial Pneumonia with Autoimmune Features—What Do We Already Know?

Miądlikowska

Rzepka-Wrona

Miłkowska-Dymanowska

et al. 2021

JCM

View full text Add to dashboard Cite

Interstitial pneumonia with autoimmune features (IPAF) belongs to a group of diseases called interstitial lung diseases (ILDs), which are disorders of a varied prognosis and course. Finding sufficiently specific and sensitive biomarkers would enable the progression to be predicted, the natural history to be monitored and patients to be stratified according to their treatment. To assess the significance of pulmonary fibrosis biomarkers studied thus far, we searched the PubMed, Medline and Cochrane Library databases for papers published between January 2015 and June 2021. We focused on circulating biomarkers. A primary review of the databases identified 38 articles of potential interest. Overall, seven articles fulfilled the inclusion criteria. This review aims to assess the diagnostic and prognostic value of molecules such as KL-6, SP-A, SP-D, circulating fibrocytes, CCL2, CXCL13, CXCL9, CXCL10 and CXCL11. All of these biomarkers have previously been studied in idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated interstitial lung disease (CTD-ILD). IPAF is a disorder of a heterogeneous nature. It explains the lack of coherent observations in terms of correlations with functional parameters. There is still no meta-analysis of pulmonary fibrosis biomarkers in IPAF. This is mainly due to the heterogeneity of the methodology and groups analysed in the research. More research in this area is needed.

show abstract

“…On the other hand, removal of anti-synthetase and MDA-5 antibodies from the criteria has been proposed given that IPAF patients with myositis-specific antibodies (Jo-1, PL7, PL12, EJ, OJ, Mi-2, SRP, NXP2, TIF1y, SAE, and MDA-5 antibodies) behave similarly to IIM-ILD in terms of treatment response and survival [ 9 • ]. In some IPAF cohorts, fever and rash were prevalent [ 10 – 14 ], leading to criticism that these should be added to the clinical domain [ 11 ]. Additionally, sicca and gastroesophageal reflux disease (GERD) have been suggested to be relevant clinical features of IPAF which could be included [ 15 , 16 ].…”

Section: Ipaf Classification Criteria and Limitationsmentioning

confidence: 99%

Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know

et al. 2022

View full text Add to dashboard Cite

Purpose of Review This narrative review will focus on the role of the rheumatologist in evaluating patients with interstitial lung disease (ILD) without a defined rheumatic disease and will outline the current classification criteria for interstitial pneumonia with autoimmune features (IPAF) and describe what is known regarding IPAF pathobiology, natural history, prognosis, and treatment. Lastly, knowledge gaps and opportunities for future research will be discussed. Recent Findings IPAF is a recently defined classification of ILD patients who have features suggesting an autoimmune-mediated process, but do not fulfill current rheumatic disease criteria. The goal of the IPAF criteria is to provide a uniform case definition for the study of autoimmune ILD patients who do not currently fit within standard ILD diagnostic categories, ultimately improving diagnosis and therapy. Many of these patients are referred for rheumatologic evaluation to aid the diagnostic process. Summary The care of the IPAF patient is complex and is multidisciplinary with pulmonology, rheumatology, pathology, radiology, physical therapy, primary care, pulmonary transplant providers all serving vital roles. The rheumatologist has several roles which include classification, disease monitoring, and management.

show abstract

Interstitial Pneumonia with Autoimmune Features (IPAF): A Single-Centre, Prospective Study

Cited by 13 publications

References 18 publications

Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study

Clinical, radiologic and serologic profile of patients with interstitial pneumonia with autoimmune features: a cross-sectional study

Review: Serum Biomarkers of Lung Fibrosis in Interstitial Pneumonia with Autoimmune Features—What Do We Already Know?

Interstitial Pneumonia with Autoimmune Features: What the Rheumatologist Needs to Know

Contact Info

Product

Resources

About