Respiratory bronchiolitis interstitial lung diseaseJ68

Katzenstein, 1994

RB-ILD is considered a subgroup of desquamative interstitial pneumonia (DIP) as one of the tobacco smoke-associated idiopathic interstitial pneumonia.

Unknown

RB-IIP also belongs to the group of interstitial lung diseases associated with tobacco smoke. Reliable figures on the frequency of the disease are not available. This is certainly related to the fact that an open or thoracoscopic biopsy is required for an exact diagnosis, which is out of proportion to the favourable prognosis.

Already young patients < 30 years of age are affected. The mean age of the disease is about 43 years. Men are more frequently affected than women.

The disease is undoubtedly caused by smoking. However, since not all excessive smokers develop DIP, it can be assumed that the genetic predisposition also plays a role here.

The symptoms are similar to those of desquamative interstitial pneumonia:

• creeping symptomatology
• persistent, productive cough
• Shortness of breath
• Auscultatory rales are often found to be inspiratory, but occasionally also expiratory.

Pulmonary function: Mostly signs of pulmonary hyperinflation (RV/TLC increased) without - or at most slightly pronounced - airway obstruction. Signs of a significant restriction are absent. The diffusion capacity is restricted to varying degrees.

The diagnosis is made by the HRCT. Here you can find:

• diffusely distributed thickening of the bronchial wall
• centrilobular nodules
• varying degrees of milky glass-like haze, increased in the periphery
• signs of emphysema especially in the upper fields
• Air trapping (bronchial collapse, compression of the small airways) in the subfields with hypodensic zones next to regions with normal or increased density

Abundant macrophages in the peribronchial alveoli.

Any type of tissue removal - sometimes even the BAL - can trigger an acute flare of disease in IPF/UIP. This is not described for the other subgroups. For this reason, however, the indication for biopsy should always be narrowly defined.

• DIP
• Langerhans cell histiocytosis
• Chronic obstructive bronchitis
• Emphysema
• other forms of interstitial pneumonia (IPF/UIP, COP, AIP, DIP, LIP)
• Collagenoses
• Asbestosis
• exogenous allergic alveolitis
• Sarcoidosis of the lungs

• Respiratory insufficiency
• Pulmonary hypertension
• Cor pulmonale
• Lung cancer

Even with pronounced interstitial changes, nicotine withdrawal alone significantly improves the disease. Corticoid treatment is usually not necessary.

The prognosis of the RB-ILD is favourable. No deaths have been observed so far.

However, follow-up examinations are recommended to exclude other IIPs.

1. Baumann A (2015) On the Course of Idiopathic Pulmonary Fibrosis and the Influence of Clinical Exacerbations with Subsequent Inpatient Treatment on Disease Progression and Survival, Inaugural Dissertation. Justus-Liebig-University Giessen
2. Gerok W et al (2007) Internal Medicine 451
3. Goeckenjan G (2003) Respiratory bronchiolitis with interstitial lung disease. Pneumology 57(5) S 278-287
4. Günther A et al (2003) Dtsch Ärztebl 100 (24) A:1676/ B:1389/ C:1305
5. Herold G et al (2018) Internal Medicine 374 and 392-394
6. Kasper DL et al (2015) Harrison's Principles of Internal Medicine 1708-1713
7. Kasper DL (2015) Harrisons Internal Medicine 2089-2096
8. Köhler et al (2010) Pneumology 141-151
9. Kreuter M et al (2016) Rare lung diseases 143-162
10. Lorenz J et al (2016) Checklist Pneumology (Checklists XXL) S 323-330
11. Loscalzo J et al (2011) Harrison's Lung Medicine and Intensive Care S 224-235
12. Müller HM (2003) The classification of interstitial pneumonia from a pathological-anatomical and clinical point of view. Inaugural dissertation. Ruhr University Bochum
13. Travis WD (2013) An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Ideopathic Interstitial Pneumonias. AJRCCM 188 (6) 733-748