Eberlein Michael H, Drummond Michael B, Haponik Edward F
Department of Medicine, Johns Hopkins Bayview Medical Center, Johns Hopkins School of Medicine, Baltimore, Maryland 21224, USA.
Am J Med Sci. 2008 Feb;335(2):163-9. doi: 10.1097/MAJ.0b013e318068b60e.
Plastic bronchitis is an uncommon and underdiagnosed entity, characterized by recurrent expectoration of large, branching bronchial casts. We describe a 39-year-woman with no prior lung disease who had episodic wheezing, severe dyspnea with expectoration of large and thick secretions, branching in appearance, which she described as resembling squid. A comprehensive evaluation revealed no specific cause and a diagnosis of idiopathic plastic bronchitis was made. In plastic bronchitis the bronchial casts may vary in size from small segmental casts of a bronchus to casts filling the airways of an entire lung. Plastic bronchitis can therefore present as an acute life-threatening emergency if mechanical obstruction of major airways occurs. The casts are differentiated into type I, inflammatory casts, or type II, acellular casts. The type I inflammatory casts are often associated with bronchial disease and often have an acute presentation. The acellular type of cast production is often chronic or recurrent. Numerous systemic illnesses are associated with plastic bronchitis, but often, as in our patient, no underlying cause can be identified. The treatment of plastic bronchitis includes acute therapy to aid the removal and expectoration of casts, and specific short- or long-term treatments attempting to address the underlying hypersecretory process. The therapeutic options are supported only by anecdotal evidence based on case reports as the rarity and heterogeneity of plastic bronchitis confounds systematic investigations of its treatment. Improved understanding of the regulation of mucus production may allow for new treatment options in plastic bronchitis and other chronic lung diseases characterized by hypersecretion of mucus.
塑性支气管炎是一种罕见且易被漏诊的病症,其特征为反复咳出粗大的分支状支气管铸型。我们报告一例39岁女性,既往无肺部疾病史,出现发作性喘息、严重呼吸困难并咳出大量浓稠分泌物,分泌物呈分支状,患者形容其类似鱿鱼。全面评估未发现特定病因,遂诊断为特发性塑性支气管炎。在塑性支气管炎中,支气管铸型大小各异,从小的支气管节段性铸型到充满整个肺气道的铸型都有。因此,如果主要气道发生机械性阻塞,塑性支气管炎可表现为急性危及生命的紧急情况。铸型可分为I型(炎性铸型)或II型(无细胞铸型)。I型炎性铸型常与支气管疾病相关,且通常有急性表现。无细胞型铸型的产生往往是慢性或复发性的。许多全身性疾病都与塑性支气管炎有关,但通常如我们的患者一样,无法确定潜在病因。塑性支气管炎的治疗包括急性治疗以协助清除和咳出铸型,以及针对潜在的高分泌过程的特定短期或长期治疗。由于塑性支气管炎的罕见性和异质性使得对其治疗的系统研究受到困扰,目前的治疗选择仅基于病例报告的轶事证据。对黏液产生调节机制的更好理解可能会为塑性支气管炎及其他以黏液高分泌为特征的慢性肺部疾病带来新的治疗选择。