Atrophic polychondritis with the report of a case.

During recent years a number of patients have been described in whom degeneration of cartilage in the ears, nose, respiratory tract, and thoracic cage has been associated with polyarthritis and sometimes also with iritis or episcleritis. The first recorded case appears to be that of Jaksch-Wartenhorst (1923) in Prague: a man aged 32 years became ill with multiple arthritis and fever and this was followed by swelling of both external ears, proceeding to shrinkage and deformity, with partial stenosis of the external auditory canals, deafness, and tinnitus; the nasal cartilage collapsed, producing a saddlenose deformity; the epiglottis became shortened, thick, and nodular; pain and tenderness developed in the costochondral junctions and xiphoid process. Jaksch-Wartenhorst considered that this was a hitherto undescribed degenerative disorder of cartilage and named it polychondropathia. Von Meyenburg (1936), in Zurich, briefly reported a second case and a full account of the same patient was published by Altherr (1936); neither author was aware of Jaksch-Wartenhorst's earlier paper and they described the condition as chondromalacia. This patient was a boy aged 14 years, who died 10 months after the onset of an illness in which a migrating polyarthritis involving the large joints was accompanied by softening of cartilage in the ears, nose, larynx, trachea, and chest wall. Tracheotomy was required to relieve respiratory obstruction. At autopsy, miliary tuberculosis was found with a primary tuberculous complex in the right lung, but the changes in the joints and cartilages were not tuberculous and appeared to be due to a mixture of degenerative and inflammatory processes. Histological evidence of rheumatic myocarditis was also found. Gordon, Perlman, and Shechter (1948), in the United States, reported a third case, again without knowledge of the preceding publications. An American Negress aged 34 years developed arthritis of the knees and ankles, associated with fever, swelling of the ears, hoarseness, and tenderness over the sternum and chest wall. This patient also required tracheotomy, and biopsy specimens of cartilage showed a mixture of degenerative and inflammatory changes. A further feature was bilateral iritis and conjunctivitis. A fourth case, with severe ocular complications leading to blindness, was described by Hilding (1952). In this patient, a woman aged 61 years, progressive polyarthritis was associated with deepening of the voice, deformity of the ears and nose, and softening of the costal cartilages. Harders (1954) recorded a further case, a boy aged 16 years, who also needed tracheotomy and in whom the softening of cartilage involved the nose, ribs, larynx, and trachea, with arthritis and episcleritis. Harders recognized that the disease was similar to that described by Von Meyenburg (1936) and Altherr (1936) and that reported by Gordon, Perlman, and Shechter (1948). Similar cases were published by Rogers and Lansbury (1955), and Bober and Czarniecki (1955), both patients being women, aged 61 and 41 years respectively. Each had ocular manifestations and the patient described by Bober and Czarniecki also required tracheotomy. Harders (1956), in a further publication, mentioned three other cases, diagnosed since his earlier paper, but did not give details. Another typical case was described by Harwood (1958): a man aged 35 years died of respiratory obstruction. Another patient, a woman aged 63 years, was reported by Klatskin and Katzenstein (1958): this patient also died of respiratory infection secondary to chronic respiratory obstruction. Bean, Drevets, and Chapman (1958) reviewed the syndrome and added yet another case, a woman aged 58 years. Two further cases, a man aged 33 years and a woman aged 29 years, were reported by Pearson, Kline, and Newcomer (1960) in a second full review of the syndrome, in which for the first time JakschWartenhorst's original case was quoted. At the Sixth International Congress of Internal Medicine, Bean (1960) reported briefly the details of six additional unpublished cases and Gill (1960) reported another case.