Background: Pulmonary siderosis is a well established disorder in welders. Internationally more than 150 cases of interstitial pulmonary siderofibrosis are associated with long-standing and heavy exposure to welding fumes at poorly ventilated working places. Patients: Characteristic job histories, lung function analyses and histological examinations as well as elemental microanalysis by energy dispersive X-ray analysis (EDX) are demonstrated from 3 welders with pulmonary siderofibrosis. Results: Histological examinations show a patchy interstitial fibrosis with accumulations of particulate material typical for welding fumes. EDX disclose an increase of iron-load in activated macrophages as well as in lung tissue and a close topographical relationship of welding fume particles and interstitial fibrotic reactions. Lung function analysis showed predominantly loss of pulmonary performance during spiroergometry. Conclusions: Regarding the actual knowledge about the pathomechanisms of ultrafine particles on lung tissue, the evidence from animal experiments, the histological and electron microscopical results, our own clinical examinations of welders and some epidemiological evidence, we assume a causal relationship of interstitial pulmonary siderofibrosis in welders with long-standing exposure to high concentrations of welding fumes under poor working conditions.