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Pneumoconiosis and Progressive Fibrosing Interstitial Lung Disease

Pulmonology
Physify · 10 August 2026

The occupational fibrotic lung diseases (the pneumoconioses) still have no approved disease-modifying therapy, so management rests on removing further exposure and supportive care. The most useful development is the recognition that many of these conditions can follow a "progressive fibrosing" phenotype, for which the antifibrotic nintedanib slows the rate of lung-function decline (as established in progressive fibrosing interstitial lung disease more broadly). Mesenchymal stromal cell therapy has been explored for its anti-inflammatory and potentially reparative effects, but it remains experimental and is not established practice. In short, these restrictive fibrotic diseases increasingly converge on antifibrotic therapy where a progressive phenotype is present, while cell-based and other novel approaches stay investigational.

Further reading: antifibrotics in progressive fibrosing interstitial lung disease (INBUILD trial and reviews); mesenchymal stromal cell therapy in fibrotic lung disease (early-phase studies).

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