DIFFUSE PARENCHYMAL LUNG OSTEODYSTROPHY – IDIOPATHIC INTERSTITIAL LUNG DISEASE (ILD),WITH THE DEFEAT OF THE BRONCHIOLAR ALVEOLAR TRANSITION ZONE (BATZ)

Demura S.A. (1), Kogan E.A. (1,2), Paukov V.S. (1), Fligil D.M. (3), Ziuzya U.R. (3)
1 -Sechenov First Moscow State Medical University,
2 -Academician V.I. Kulakov Research Center of Obstetrics, Gynecology, and Perinatology, Moscow,
3 -Moscow City Scientific-and-Practical Center of Tuberculosis Control

Based on literature data and analysis of own observation the hypothesis of pathogenesis of diffuse parenchymal lung osteodystrophy (DPLOD) is proposed. DPLOD is a very rare disease with unknown etiology. It is characterized by unilateral or bilateral lesions of the lower lobes of the lungs presented by formation of bone tissue foci with the bone marrow cells in the interstitium of the lung. This disease is difficult for life-time diagnosis, chronic and often asymptomatic. Based on data of own observation of DPLOD in the male of 37 years, the authors suggest the possibility that DPOLD may be considered as idiopathic ILD, the development of which is associated with the damage of the bronchiolar alveolar transition zone (BATZ) in a course of chronic inflammation and impaired differentiation of mesenchyme progenitor and stem cells in the course of reparation lung tissue. The study of DPOLD showes that reparation of lung tissue in BATZ, one of the areas of stem cell niches in the lung tissue, can occur with the participation of mesenchyme stem cells, probably of bone marrow origin. In conditions of chronic inflammation and permanent BATZ damage in ILD, the differentiation of stem cells is deteriorated. This leads to fibrosis and, in some cases, to the formation of bone marrow tissue.
Keywords: 
diffuse parenchymal lung osteodystrophy (DPOLD), bone marrow tissue, bronchiolar alveolar transition zone (BATZ), mesenchyme stem cells, interstitial lung disease (ILD)