Clinical and Histopathologic Features of Interstitial Lung Disease in Erdheim⁻Chester Disease

Journal of Clinical Medicine
Sara G HaroutunianB R Gochuico

Abstract

Limited information is available regarding interstitial lung disease (ILD) in Erdheim⁻Chester disease (ECD), a rare multisystemic non-Langerhans cell histiocytosis. Sixty-two biopsy-confirmed ECD patients were divided into those with no ILD (19.5%), minimal ILD (32%), mild ILD (29%), and moderate/severe ILD (19.5%), based on computed tomography (CT) findings. Dyspnea affected at least half of the patients with mild or moderate/severe ILD. Diffusion capacity was significantly reduced in ECD patients with minimal ILD. Disease severity was inversely correlated with pulmonary function measurements; no correlation with BRAF V600E mutation status was seen. Reticulations and ground-glass opacities were the predominant findings on CT images. Automated CT scores were significantly higher in patients with moderate/severe ILD, compared to those in other groups. Immunostaining of lung biopsies was consistent with ECD. Histopathology findings included subpleural and septal fibrosis, with areas of interspersed normal lung, diffuse interstitial fibrosis, histiocytes with foamy cytoplasm embedded in fibrosis, lymphoid aggregates, and focal type II alveolar cell hyperplasia. In conclusion, ILD of varying severity may affect a high proportion of...Continue Reading

References

Feb 24, 2001·The American Journal of the Medical Sciences·R D ShamburekB R Gochuico
Jul 5, 2005·The European Respiratory Journal·M R MillerUNKNOWN ATS/ERS Task Force
Jul 21, 2007·American Journal of Respiratory and Critical Care Medicine·Ivan O RosasBernadette R Gochuico
Jan 30, 2008·Archives of Internal Medicine·Bernadette R GochuicoIvan O Rosas
Sep 5, 2009·American Journal of Respiratory and Critical Care Medicine·Farshid N RouhaniBernadette R Gochuico
Jun 4, 2010·Blood·Gayane Badalian-VeryBarrett J Rollins
Jun 22, 2010·European Radiology·Anne-Laure BrunPhilippe A Grenier
Mar 11, 2011·The New England Journal of Medicine·George R WashkoUNKNOWN COPDGene Investigators
Feb 18, 2014·Current Rheumatology Reports·Julien HarocheZahir Amoura
Nov 14, 2015·The Journal of Clinical Endocrinology and Metabolism·C CourtillotJ Haroche
Mar 12, 2016·Blood·Jean-François EmileUNKNOWN Histiocyte Society
Nov 2, 2016·The European Respiratory Journal·Anna J PodolanczukDavid J Lederer
May 30, 2017·Blood Advances·Juvianee I Estrada-VerasWilliam A Gahl
Jun 22, 2017·The Annals of Thoracic Surgery·Kohei HashimotoTakahiro Oto
Nov 4, 2017·Lung India : Official Organ of Indian Chest Society·Enambir Singh JosanJayantilal B Mehta
Dec 2, 2017·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Neval OzkayaAhmet Dogan
Feb 6, 2018·American Journal of Hematology·Fleur Cohen-AubartJulien Haroche

❮ Previous
Next ❯

Citations

Aug 30, 2019·Respirology Case Reports·Jun ShiiharaTakashi Ogura
Aug 7, 2019·The British Journal of Radiology·Jeeban Paul DasDarragh F Halpenny
Jun 28, 2020·Leukemia·Francesco PegoraroAugusto Vaglio
Oct 30, 2020·JAMA Network Open·Skand ShekharFady Hannah-Shmouni
Jul 3, 2021·Frontiers in Medicine·Lauren C TestaBernadette R Gochuico
Nov 16, 2021·Journal of the National Comprehensive Cancer Network : JNCCN·Kevin O'BrienJuvianee I Estrada-Veras

❮ Previous
Next ❯

Methods Mentioned

BETA
biopsies
biopsy

Clinical Trials Mentioned

NCT01417520

Software Mentioned

GraphPad Prism
GraphPad

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.