Relevant IPF Research

The CDC identified a statistically significant cluster of IPF patients diagnosed at a Virginia medical center.

When we shared the CDC report and recent research with a medical school professor and family friend of the IPF Foundation’s Executive Director, Dr. Al Masi, MD, DR.PH, responded with the letter below: 

Dear Kristan,

Good to hear from you and was interested in the recent studies you found in IPF, especially the cluster of 8 dentists identified by CDC in 894 patients treated at the Virginia tertiary care center during 1996 – 2017. This 1% frequency is greater than the general population prevalence of less than 0.1%, although the dentists were older age, from 49 – 81 years.  Their exposure to polishing dental appliances and preparing amalgams could possibly have respiratory toxicity as well as exposure to oral micro-organisms.

This cluster in dentists seems to deserve further investigation as does the occurrence in spouses like your mom and dad.  That latter type of study could be done in tertiary care centers such as Virginia and Mayo Clinic, using a control respiratory disease. Other important data would be occurrence in spousal and non-spousal family members, like parents and children.

Your other links are interesting and support an association between lung microbiome and acute exacerbations or progression of IPF.  However, factors which activate the disease or accelerate progression may not be the original initiating risk factors. The causation may also be influenced by underlying genetic factors.

Kristan, you are certainly doing a great job in investigating the many complex factors that could be contributing to IPF and especially those variables pertaining to spousal and familial occurrence and clusters which could reflect causal mechanisms.

Fond best wishes,

Al and Nancy

A.T. Masi, MD, DR.PH
Professor Emeritus of Medicine and Professor of Epidemiology

CDC Report: Dental Personnel Treated for Idiopathic Pulmonary Fibrosis at a Tertiary Care Center — Virginia, 2000–2015
Weekly / March 9, 2018 / 67(9);270–273
See study >

Each research study below represents a step forward in discovering cures for IPF.

“…31% of first-degree relatives of patients with pulmonary fibrosis had interstitial lung abnormalities on CT…” Learn more.

“There was no evidence in multivariable analyses that the prevalence of either ILA (Interstitial Lung Abnormalities) or ILD (Interstitial Lung Disease) differed between the 46 relatives with FPF (Familial Pulmonary Fibrosis) and the 59 relatives with sporadic IPF (Idiopathic Pulmonary Fibrosis). “

“Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis.” American Journal of Respiratory and Critical Care Medicine, Aug. 2019.

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“Among epithelial cells, we identified a population of cells that is transcriptionally distinct from any epithelial cell type previously described in the lung …”

“Among vascular endothelial cells, we identify an ectopically expanded cell population transcriptomically identical to bronchial restricted vascular endothelial cells in IPF.”

“Single-cell RNA-seq reveals ectopic and aberrant lung-resident cell populations in idiopathic pulmonary fibrosis,” Science Advances, July 2020.

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“ … it is to be hoped that the application of these methodologies to large populations, of greatly phenotyped IPF individuals, will help define the role played by viruses and bacteria in the development, progression and acute exacerbations in IPF patients. This should, in turn, enable the development of novel therapeutic approaches for this devastating disease.”

“The role of infection in the pathogenesis of idiopathic pulmonary fibrosis,” Molyneaux, PL, et al., Eur Respir Rev, (2013)

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“These preliminary data suggest progression of idiopathic pulmonary fibrosis is associated with the presence of specific members within the Staphylococcus and Streptococcus genera.”

“Association between lung microbiome and disease progression in IPF: a prospective cohort study,” Meilan Han MD et al., Lancet Respiratory Medicine, 2014.

Investigators asked for more research to identify the specific species and to ascertain if it’s a causal relationship.

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“We found herpesvirus saimiri DNA in the regenerating epithelial cells of 21/21 idiopathic pulmonary fibrosis cases using four separate probe sets but not in the 21 controls.

RT-PCR showed that the source of the cyclin D RNA in active idiopathic pulmonary fibrosis was herpesvirus saimiri and not human.”

“Idiopathic pulmonary fibrosis is strongly associated with productive infection by herpesvirus saimiri,” Folcik et al., Modern Pathology, 2014.

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“Clinical findings, histopathology, and clinical course are indistinguishable between familial IPF and sporadic cases”.

“Idiopathic Pulmonary Fibrosis”, Naftali Kaminski et al., American Journal of Respiratory Cell and Molecular Biology, 29(supplement_1), pp. S1–S105, September 2003.

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“ … our observations challenge the current paradigm for A.E.-IPF and provide a rationale for clinical trials of prophylactic antibiotics as a strategy to prevent acute exacerbations in individuals with IPF.”

“Changes in the respiratory microbiome during acute exacerbations of idiopathic pulmonary fibrosis,” Molyneaux PL et al., Respiratory Research, 2017.

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