2014
DOI: 10.21767/amj.2014.2102
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Follicular Bronchiolitis in Primary Ciliary Dyskinesia

Abstract: Ciliary dysfunction in primary ciliary dyskinesia (PCD) may be associated with bronchiolitis. Diffuse bronchiolitis has been reported in a subset of PCD patients who have Kartagener's syndrome in Japan. We report a case of follicular bronchiolitis (FB) in a case of PCD presenting with recurrent episodes of cough, dyspnea, and bronchiectasis. This may motivate researchers to study rarer variants and presentations in PCD.

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“…B cells may be a key factor for CVID ILD as it is ameliorated by rituximab, potentially explaining its absence in XLA [37]. Infections have been associated with this ILD [31,[38][39][40][41] which did occur more frequently in CVID patients with history of pneumonia or bronchiectasis. However, this result differed from our previous work from a single center that did not find ILD to be associated with bronchiectasis or pneumonia [31].…”
Section: Discussionmentioning
confidence: 97%
“…B cells may be a key factor for CVID ILD as it is ameliorated by rituximab, potentially explaining its absence in XLA [37]. Infections have been associated with this ILD [31,[38][39][40][41] which did occur more frequently in CVID patients with history of pneumonia or bronchiectasis. However, this result differed from our previous work from a single center that did not find ILD to be associated with bronchiectasis or pneumonia [31].…”
Section: Discussionmentioning
confidence: 97%
“…While our understanding of the prevalence of typespecific bronchiolitis other than diffuse bronchiolitis among populations with PCD is insufficient at present, the pathology in PCD-related bronchiolitis has been suggestive of DPB (12). Interestingly, Thalanayar and Holguin reported a case of follicular bronchiolitis in a patient with PCD in which they proposed a revised approach to managing airway disease, including the type-specific bronchiolitis noted in this subset of PCD patients (13). The pathological findings in our four cases were compatible with DPB or PCD, and one patient showed the presence of constrictive bronchiolitis with PCD.…”
Section: Discussionmentioning
confidence: 99%