Sporadic Obliterative Bronchiolitis: Case Series and Systematic Review of the Literature

Abstract

ObjectiveTo describe the clinical characteristics and outcomes of patients diagnosed with obliterative bronchiolitis (OB) not associated with transplantation or point-source exposures to inhaled toxins.Patients and MethodsWe compiled all confirmed diagnoses of OB at our institution and analyzed their demographic characteristics, treatments, and outcomes as defined by pulmonary function tests (PFTs) and transplant-free mortality. The study period ranged from July 2007 to August 2017. Histological diagnosis was confirmed by a pathologist, and high-resolution chest computed tomography (CT) scans were reviewed and scored by chest radiologists. We also performed a systematic literature review of sporadic OB series.ResultsWe identified 19 confirmed cases at our institution and 9 publications in the literature containing 104 patients. In both our series and the literature, patients were disproportionately middle-aged Caucasian women. The disease was idiopathic in 42% and was associated with connective tissue diseases and inhalational exposures in 31% and 15%, respectively. Chest CT showed expiratory air trapping in all patients. Patients were treated with corticosteroids, steroid-sparing agents, and macrolides in 77%, 46%, and 22%, respectively. Over a median follow-up in our series of 1703 days (range, 11-3206 days), PFTs did not change significantly. In all series combined, mortality incidence from any cause was 82/1000 patient-years (95% CI, 65-102). Of 14 patients who died, 3 deaths were due to respiratory failure and 5 were potentially related to complications of immunosuppressive therapy.ConclusionSporadic OB is a rare disease that is uniformly associated with air trapping on high-resolution chest CT. The diagnosis should be established with surgical biopsy if possible. The illness is not typically progressive.

Childhood bronchiolitis obliterans in Hong Kong‐case series over a 20‐year period

“…Bronchiolitis obliterans (BO) is a rare but serious condition characterized by small airway inflammation and fibrosis causing irreversible chronic obstructive lung disease 1–6 . In children, the disease begins with an injury of the small airway epithelium followed by an inflammatory reaction that progresses toward airway obliteration secondary to an aberrant repair process 6,7 .…”

“…Bronchiolitis obliterans (BO) is a rare but serious condition characterized by small airway inflammation and fibrosis causing irreversible chronic obstructive lung disease. [1][2][3][4][5][6] In children, the disease begins with an injury of the small airway epithelium followed by an inflammatory reaction that progresses toward airway obliteration secondary to an aberrant repair process. 6,7 This syndrome can be the result of injury to the respiratory and terminal bronchioles from a variety of causes, which include infectious such as adenovirus, influenza, measles, and noninfectious such as bone marrow or organ transplantation, exposure to inhaled toxins, drug reactions, connective tissue diseases, and gastroesophageal reflux.…”

“…6,7 This syndrome can be the result of injury to the respiratory and terminal bronchioles from a variety of causes, which include infectious such as adenovirus, influenza, measles, and noninfectious such as bone marrow or organ transplantation, exposure to inhaled toxins, drug reactions, connective tissue diseases, and gastroesophageal reflux. 1,3,8 BO can be diagnosed and classified histopathologically. However, due to the invasive nature of lung biopsy, histological diagnosis is not routinely available for all cases.…”

A young lady with dyspnoea: Quest for the cause

“…The literature on cases of cryptogenic OB is very scarce. To the best of our knowledge none has been reported to initially present with severe pulmonary hypertension [13,14]. The management of OB involves bronchodilators and treatment of underlying disorder.…”

Bronquiolite obliterante em paciente pediátrico associada com exposição à cocaína e outras drogas de abuso durante o período gestacional

“…Quanto as possíveis etiologias, quando não idiopática, a BO pode ter como causa a inalação de substâncias tóxicas; síndromes aspirativas; imunodeficiências, síndrome de Sjögren e artrite reumatóide; alterações do metabolismo de colágeno e de tecido conjuntivo; causas do pós-transplante e pós-operatório e; pode também ser induzida por drogas e medicamentos (Terra Filho et al, 2004;Garipo & Capelozzi, 2006;Lin et al, 2018;Callahan et al, 2019). Contudo, durante a infância, a BO pós-infecciosa nas vias aéreas inferiores se destaca, ocorrendo também em crianças imunocomprometidas, sendo causada principalmente pelos adenovírus 3, 7 e 21, sendo citada na pós infecção pelo vírus sincicial respiratório, além de algumas bactérias, como Mycoplasma pneumoniae, Streptococcus tipo B, Legionella pneumophila e Bordetella pertussis (Teper et al, 2002;Lobo et al, 2007;Colom & Teper, 2009a;Colom & Teper, 2009b;Fischer et al, 2010;Champs et al, 2011;Lino et al, 2013).…”