Diffuse Interstitial Fibrosis of the Lungs (Hamman-Rich Syndrome) Successful Palliation of a Case with Steroid Therapy

In 1944 Hamman and Rich1 described four cases of a unique type of diffuse intersitial fibrosis of the lungs, which they considered to be an entity not previously described. Since then (through June, 1956) 36 cases of the Hamman-Rich Syndrome, as it has come to be known, have been reported,2 all except one ending in death8. The average age at the time of onset of the disease has been 44.5 years, varying from 13 to 72 years, and the average duration of the disease has been two years from the onset of symptoms. The chief and paramount symptom has been dyspnea, usually all out of proportion to the extent of the pulmonary lesions demonstrated by x-ray films. Other symptoms have been inconsistent, but have included cough, cyanosis, fatigability, ankle edema, and those findings usually associated with right heart failure. On physical examination there are no characteristic findings. Reported #{149}have b en dyspnea, cyanosis, finger clubbing and fever, as well as signs of cardiac failure and pneumonia. Laboratory examination has been consistently helpful only insofar as the chest roentgenogram is concerned. All patients have had diffuse bilateral opacities, varying from strandlike to patchy to nodular. Sputum has been negative for tubercle bacilli in all, but the tuberculin reaction has been positive in about one-half of the cases. Polycythemia has been a feature in a few. The EKG has shown evidence of cor pulmonale in 11 instances (30 per cent) with autopsy findings Showing right ventricular hypertrophy in 18 (50 per cent). Multiple biochemical determinations have shown no consistent pattern. Pathologically, the lesions have been fairly specific. The tissue in the great majority of cases has been obtained at autopsy, but in a few by lung biopsy. Opportunities for both sources have been afforded in a few. The findings include (1) Proliferation of the alveolar lining cells-with cuboidal and even columnar cells present (2) Necrosis of alveolar and bronchial epithelium. (3) Hyaline membrane formation around the alveoli. (4) Marked edema of the alveoli. (5) Extensive interstitial fibrosis. (6) Marked perivascular fibrosis. (7) Eosinophilic interstitial infiltration. (8) No demonstrable etiology. Therapeutically, practically all of the antibiotics and antituberculosis drugs have been tried with a marked lack of success. A few have re-