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Hamman-Rich syndrome

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Previously used term for acute diffuse idiopathic interstitial pulmonary fibrosis of unknown aetiology. Insidious onset followed by respiratory embarrassment, fatigability, loss of weight, cyanosis, right-sided heart failure and other abnormalities. It is usually fatal. Both sexes affected; slight male predominance; onset over broad age range, but highest incidence between 40 and 70 years of age. Aetiology unknown. Familial, autosomal dominant inheritance.

First described by Sir Dominic John Corrigan (1802-1880) in 1838, in 1897 by Georg Eduard Rindfleisch (1836-1908) as «cirrhosis cystica» and in 1915 by David Paul von Hansemann (1858-1920) as «lymphangitis reticularis».


  • D. J. Corrigan:
    On cirrhosis of the lung.
    The Dublin Journal of Medical Science, 1838, 13: 266-286.
  • G. E. Rindfleisch:
    Ueber Cirrhosis Cystica Pulmonum. Zentralblatt für allgemeine Pathologie und pathologische Anatomie, Jena, 1897, 8: 864–865.
  • L. Hamman, A. R. Rich:
    A clinical-pathological conference. A case of heart failure.
    International Clinics, 43d series, 1933: 1: 197-232
  • L. Hamman, A. R. Rich:
    Fulminant diffuse interstitial fibrosis of the lungs. Transactions of the American Clinical and Climatological Association, 1935, 51: 154-163.
  • L. Hamman, A. R. Rich:
    Acute diffuse interstitial fibrosis of the lungs.
    Bulletin of the Johns Hopkins Hospital, Baltimore, 1944, 74: 177-212.
  • S. Javaheri, D. H. Lederer, J.A. Pella, G. J. Mark and B.W. Levine:
    Idiopathic pulmonary fibrosis in monozygotic twins: the importance of genetic predisposition. Chest, 1980, 78: 591-594.
We thank Jim Loyd for information submitted.

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