OVERVIEW
Respiratory bronchiectasis with interstitial lung disease (RBILD) was recognized in the early 1980s in patients who were smokers or former smokers. RBILD was initially recognized as a disease pathologically similar to desquamative interstitial pneumonitis (DIP); however, it is now considered to be a manifestation of the different severity of small airway and parenchymal damage caused by cigarette smoking, i.e., two different outcomes of the same disease, and has even been advocated as an alternative to DIP.Opinions on this issue are not yet unanimous.
Etiology
RBILD is mainly seen in people who smoke heavily and are exposed to environmental and industrial pollutants. It may be a nonspecific cellular response to inhaled irritants.
Symptoms
Almost all patients with RBILD are smokers, and the onset of the disease is usually in the 30s and 40s, with an insidious onset. Most patients are asymptomatic or have only mild symptoms, including mild cough and shortness of breath. However, cases of severe shortness of breath, active hypoxemia, and hemoptysis have been reported, so severely impaired lung function cannot be used to exclude the diagnosis of RBILD. End-inspiratory bursting rales can be heard at the lung bases. Pestle-like fingers are rare.
Examination
1. Chest X-ray
In more than 2/3 of patients, chest radiographs show diffuse symmetrical reticular shadows or reticular nodular infiltrates, but lung volumes are often normal. A few patients have normal chest radiographs.
2. Chest CT
Chest high-resolution CT examination (HRCT) lacks specific manifestations, the most common manifestations are varying degrees of patchy hairy glass-like changes and centrilobular nodules, which are mainly seen in the lower lungs; pulmonary atelectasis, intra- or interlobular interstitial thickening, emphysema, and peripheral pulmonary pustules can also be seen.
3. Lung function
The symptoms of pulmonary function are often restrictive or mixed ventilatory dysfunction and a mild decrease in diffusion volume. Occasionally, lung function is normal. Increased lung volumes suggest gas trapping in the obstructed bronchi. Mild hypoxemia may be present at rest or with activity.
4. Bronchoalveolar lavage
The total cell count in bronchoalveolar lavage may be elevated, but the cellular classification is similar to that of a regular smoker or a smoker with IPF, with large numbers of macrophages and occasional small numbers of neutrophils.
Diagnosis.
The diagnosis of RBILD is suspected on the basis of the patient’s extensive smoking history, clinical signs and symptoms, and imaging studies; pathologic tests such as lung biopsy are still needed to confirm the diagnosis.
Treatment
Cessation of smoking is essential for patients with RBILD, and clinical symptoms and lung function will improve after smoking cessation. Glucocorticoid therapy is effective. Unnecessary cytotoxic drugs should be avoided as much as possible. About 22% of patients experience partial or complete remission without any treatment.
Questions you may be concerned about
How to treat respiratory bronchiolitis with interstitial lung disease?
Treatment of respiratory bronchiolitis with interstitial lung disease includes smoking cessation and glucocorticoid therapy.
The clinical features of this disease are younger age of onset, slightly more men than women, associated with smoking, and the main symptoms are cough and shortness of breath.
1. Smoking cessation is the most important treatment measure. Almost all of the main groups of the disease have a history of smoking. In addition the presence of irritants such as asbestos, dust and silica dust in the working environment is one of the sources of danger and needs to be detached promptly.
2. Glucocorticoid therapy (e.g., prednisone) is given in a standardized manner. The aim of the treatment is to reduce the inflammatory response, suppress the immune cells, and alleviate the symptoms.
When the disease is diagnosed, early and standardized treatment is recommended to reduce the adverse effects of the disease. The above drugs need to be standardized and rationally applied under the guidance of professional physicians and pharmacists.
Prognosis
RBILD has a favorable prognosis.