Golniški simpozij 2011 Zbornik povzetkov

Current concept on pathomechanism
of Idiopathic pulmonary fibrosis
Ema Muøiœ
Previously it was anticipated that idiopathic pulmonary fibrosis (IPF) reflects an inflammatory driven
alveolitis form of lung fibrosis. But the frustrated experience in IPF is that steroids and immunosuppressants
are of little help. A more recent hypothesis puts the alveolar type II cell (P II) at the centre
of the concept, according to which chronic epithelial damage is the underlying trigger mechanism
in IPF. Proliferation of pneumocyte type II cells (P II) is induced to overcome the loss of epithelium
and the factor released may largely induce the uncontrolled proliferation of fibroblasts and the excessive
deposition of extracellular matrix, mostly collagen. IPF patients experience a progressive
dyspnoea due to loss of regularly structured alveolar units and concomitant scarring of the lung.
Quality of life gradually decreases and patients usually die within 3-5 years after diagnosis. No approved
treatment was available, but newly some studies are bringing hope with new approach
against the fibroproliferation.
Figure1. From fibroblast foci with collagen production to honey combing of the lung seen on HRCT.
(courtesy of Izidor Kern and Rok Cesar)
The pathomechanism of IPF is yet not totally understood. Previously, it was anticipated that the disease
reflects another inflammatory driven form of lung fibrosis. However, the frustrating experience
in IPF is that steroids and immunosuppressants are of little help, if at all. A more recent and increasingly
favoured hypothesis puts the alveolar type II cell at the centre of a unifying concept, according
to which chronic epithelial damage is the underlying trigger mechanism for the development
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