Following a brief mention of the classification of Hammer-Rich disease within idiopathic diffuse pulmonary fibrosis, the main clinical, radiological and laboratory features of these disease are outlined in brief. A personal case, subsequently controlled neroptically and histologically, is then described. From comparison between reported data and findings in the personal case, it is concluded that it was a typical, rare case (less than 150 others have so far been reported in all world literature) of Hamman-Rich disease. Other diagnostic hypotheses can be rejected because they are less correspondent to the typical picture and for various other considerations.

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