Wells A U, du Bois R M
Interstitial Lung Disease Unit, Royal Brompton National Heart and Lung Hospital, London, United Kingdom.
Clin Chest Med. 1993 Dec;14(4):655-66.
In general, lung involvement in connective tissue disorders has not been as well defined as has isolated interstitial lung disease; this applies particularly to bronchiolitis, which occurs infrequently. The low prevalence of bronchiolitis may reflect difficulties in making the diagnosis in mild to moderate disease; at present, most reported disease is severe. This is likely to account for the lack of therapeutic success in obliterative bronchiolitis and in many patients with follicular bronchiolitis. There is a need for earlier intervention if treatment is to be effective, and thus there is a need to refine the noninvasive diagnosis of bronchiolitis. This goal is unlikely to be achieved without the systematic noninvasive evaluation and surveillance of large groups of patients with connective tissue diseases. The role of the pulmonary function laboratory in identifying early bronchiolitis remains entirely uncertain; whether silent "small airways disease," defined physiologically, predicts the eventual development of bronchiolitis is unclear. The reversibility of this asymptomatic lesion with inhaled steroid therapy and the role of inhaled treatment in bronchiolitis, in general, have not been evaluated. More work needs to be done to determine the predictive value of CT appearances and BAL findings, to try to identify a subgroup of patients at greater risk of developing severe bronchiolitis. Further histocompatibility studies may serve as a basis for the selection of patients with an increased likelihood of developing airways disease. The role of open lung biopsy requires further clarification. Better noninvasive evaluation should reduce the need for this invasive procedure; in some patients, however, including those with concomitant interstitial lung disease, histologic assessment will remain an essential component of management. In recent years, in contrast to early reports, it has become apparent that organizing pneumonia has a better prognosis than fibrosing alveolitis in the connective tissue diseases; overall, stability or regression of disease, usually with corticosteroid therapy, was documented in 28 of 39 reported cases. In these patients, a tissue diagnosis serves to identify the need for aggressive therapeutic intervention. Finally, the compilation of larger clinical series would improve our understanding of severe bronchiolitis. This is likely to require multicenter collaboration, which often is impracticable; without this approach, however, the description of bronchiolitis will remain anecdotal.
一般来说，结缔组织病累及肺部的情况不像孤立性间质性肺病那样明确；这一点在细支气管炎中尤为明显，细支气管炎并不常见。细支气管炎的低发病率可能反映出在轻至中度疾病中进行诊断存在困难；目前，大多数报道的病例病情严重。这可能是闭塞性细支气管炎以及许多滤泡性细支气管炎患者治疗效果不佳的原因。如果要使治疗有效，就需要早期干预，因此有必要完善细支气管炎的无创诊断。如果不对大量结缔组织病患者进行系统的无创评估和监测，这个目标不太可能实现。肺功能实验室在识别早期细支气管炎方面的作用仍完全不确定；生理学上定义的无症状“小气道疾病”是否能预测细支气管炎的最终发展尚不清楚。这种无症状病变对吸入类固醇治疗的可逆性以及吸入治疗在细支气管炎中的总体作用尚未得到评估。需要做更多工作来确定CT表现和支气管肺泡灌洗结果的预测价值，试图识别出发生严重细支气管炎风险更高的患者亚组。进一步的组织相容性研究可能为选择发生气道疾病可能性增加的患者提供依据。开胸肺活检的作用需要进一步阐明。更好的无创评估应该会减少对这种侵入性检查的需求；然而，在一些患者中，包括那些合并间质性肺病的患者，组织学评估仍将是治疗管理的重要组成部分。近年来，与早期报道不同的是，在结缔组织病中，机化性肺炎的预后似乎比纤维化肺泡炎要好；总体而言，在39例报道病例中有28例记录了疾病的稳定或好转，通常是通过皮质类固醇治疗实现的。在这些患者中，组织诊断有助于确定是否需要积极的治疗干预。最后，汇编更大规模的临床系列将增进我们对严重细支气管炎的了解。这可能需要多中心合作，但这往往不切实际；然而，如果不采用这种方法，对细支气管炎的描述将仍然只是个案。
