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(Chest. 1969;55:7-12.)
© 1969 American College of Chest Physicians

Familial Hamman-Rich Syndrome

Report of Eight Cases

Paul Swaye M.D.1; Howard Scott Van Ordstrand M.D., F.C.C.P.1; Lawrence J. McCormack M.D.1; and Sydney E. Wolpaw M.D., F.C.C.P.2

1 Department of Pulmonary Disease and the Division of Pathology, The Cleveland Clinic Foundation, Cleveland, Ohio
2 Medical Director, Mount Sinai Hospital, Cleveland, Ohio

A family group of eight members is described of which seven members have or had histologically proved Hamman-Rich syndrome. From the onset of symptoms, the age range is from four months to 39 years. The case data of a mother, daughter, and granddaughter with proved disease are presented. The latter two members are alive and well at the present time, being maintained on low doses of steroids. One of these patients was 3frac12 years old when the diagnosis was confirmed by lung biopsy. She probably represents one of the youngest living patients with Hamman-Rich syndrome. Two brothers of this group had coexistent pulmonary fibrosis and bronchogenic cancer; an association in the familial disease not previously recorded, though isolated cases have been reported. It is now firmly established that Hamman-Rich syndrome can occur as a distinct familial entity that is transmitted as an autosomal dominant trait.




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