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Review Interstitial Lung Disease
Diffuse Panbronchiolitis is not Restricted to
East Asia—a Mini Literature Review
Ram Kumar Mishra
Epidemiology and HEOR Team, ODC 3, Tata Consultancy Services, Thane (W), Maharashtra, India
D iffuse panbronchiolitis (DPB) is a rare inflammatory lung disease, and is well recognized in East Asian countries like Japan, China, Taiwan
and Korea. Over the years, sporadic DPB cases have been reported worldwide from other countries. This literature review presents an
overview of 48 DPB cases from other regions of the world including the US, European countries and Australia. Identification of DPB cases
from different racial groups across the globe indicates toward a need to educate pulmonologists to correctly diagnose and initiate treatment.
Keywords Diffuse panbronchiolitis, erythromycin, interstitial
lung disease, macrolide therapy, rare disease
Disclosure: Ram Kumar Mishra has nothing to
declare in relation to this article. Opinions expressed
in this article are the author’s own findings and do
not in any manner reflect or represent the view
of the organization to which he is affiliated.
Compliance with Ethics: This study involves a review of
the literature and did not involve any studies with human
or animal subjects performed by any of the authors.
Authorship: All named authors meet the International
Committee of Medical Journal Editors (ICMJE) criteria
for authorship of this manuscript, take responsibility
for the integrity of the work as a whole, and have
given final approval to the version to be published.
Open Access: This article is published under the
Creative Commons Attribution Noncommercial License,
which permits any noncommercial use, distribution,
adaptation, and reproduction provided the original
author(s) and source are given appropriate credit.
Received: October 11, 2017
Accepted: December 7, 2017
Citation: US Respiratory & Pulmonary Diseases,
2017;2(1):30–2 Corresponding Author: Ram Kumar Mishra, Epidemiology
and HEOR Team, ODC 3, Tata Consultancy Services (TCS),
5th Floor, Lexington, Hiranandani Estate, Thane (W),
Maharashtra, India 400 607. E: firstname.lastname@example.org
Support: No funding was received in
the publication of this article.
30 Diffuse panbronchiolitis (DPB) is a rare inflammatory lung disease. It was first identified in 1969 and is
well recognized in East Asian countries such as Japan, China, Taiwan, and Korea. 1 ‘Diffuse’ and ‘pan’
words in the name indicate ‘presence of lesions through both the lungs,’ and inflammation in all layers
At the time of its discovery, DPB had poor prognosis because of recurrent respiratory infections
leading to respiratory failure. In the years following the initial description of DPB in Japan, cases were
also identified in other parts of Asia including China and Taiwan, thus giving it recognition as a distinct
clinical entity. Over the years, sporadic DPB cases have been reported worldwide in countries other
than Japan, China, Taiwan, and Korea (hereafter referred to as rest of the world [ROW]). This stimulated
us to undertake a comprehensive search for available case studies on DPB. The aim of this study
was to present the current clinical evidence captured as DPB case studies across the globe excluding
East Asian countries.
A total of 657 studies regarding DPB were retrieved from PubMed in a systematic manner (Figure 1).
Forty-eight case studies published in ROW and indexed in PubMed were included in the final review.
As indicated in Figure 2, the maximum number of DPB cases among ROW countries were published
from the US (18.75%, n=9), 2–10 jointly followed by Australia, 11–15 France, 16–20 and Italy 21–25 (each 10.41%,
n=5), respectively. There were three DPB cases published each from India 26–28 and Turkey, 29–31 and two
case studies each from Belgium, 32,33 Brazil, 34,35 Germany, 36,37 and Thailand. 38,39 One case report each
was reported from Canada, 40 Ireland, 41 Malaysia, 42 Netherlands, 43 New Zealand, 44 Norway, 45 Portugal, 46
Singapore, 47 Spain, 48 and the UK. 4
It is interesting to note that a majority (50%, n=24) of DPB studies were from four developed countries.
Twelve of 48 studies (25%) reported DPB in patients of East Asian origin (either they immigrated
to other countries or were born to East Asian parents). Patients were reported either by race
(Caucasian, 13,40 Hispanic, African-American 4,9 ) or ethnicity (American citizens, White patients, Oriental
[Asian] immigrant, 3 Cambodian man, White man in Turkey, 29 Turkish girl, 30 Italian man, 21,22 man of Indian
ethnicity, 44 Scandinavian patient, 45 non-Asian Brazilian citizen, 34 and Caucasian man of Canadian
origin 40 ). None of these patients with DPB had ever travelled outside of their country until DPB was
diagnosed, except for one Hispanic patient with an extensive travel history to Far Eastern countries
including Japan. 5 Though most of the cases reported adult patients with DPB, two studies presented
DPB cases in children. One case presented a 10-year-old child of Korean birth, 10 and the other reported
a 12-year-old Turkish girl with DPB. One study reported DPB recurrence in an African-American man
after bilateral sequential lung transplantation. 4
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