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SARCOIDOSIS
683
might have been responsible.1 Investigations are being pursued by the local health authorities, the Royal Society for the Prevention of Cruelty to Animals, and the National Canine Defence League.
Histology
kill foxes,
Conference SARCOIDOSIS THE third international congress on sarcoidosis was held in Stockholm on Sept. 11-14, under the auspices of the International Union Against Tuberculosis and the presidency of Dr. SVEN LÖFGREN (Stockholm).
Epidemiology The prevalence of pulmonary sarcoidosis, as revealed by mass minature radiography surveys in 20 countries, was approximately 5 per 100,000 in Czechoslovakia, Hungary, and Japan; 10 in Australia, Canada, Finland, France, Italy, Scotland, and Yugoslavia; 15-20 in England, Germany, the Netherlands, New Zealand, Poland, and Switzerland; 26 for Norway; and 60 in Sweden. The highest rates were usually in the 20-40 age-group; and there seemed to be no preponderance in either sex or in rural rather than urban populations. B. POLLACK (Montreal) reported that during the past decade mass surveys of the Eskimo population had revealed 1200 examples of tuberculosis, but no instance of sarcoidosis. J. S. CHAPMAN (Dallas) also emphasised racial disparity: in New York City the prevalence varied from 20 to 80 per 100,000, with the higher figure in the districts where Negroes
predominated. Kveim Test L. SILTZBACH (New York) thought that the Kveim test might be helpful in early detection and in providing more uniform international diagnostic criteria. The behaviour and incidence of positive responses to his Kveim antigen were similar in 21 countries on all continents, thereby supporting a unitarian concept of the disease. The representatives of all countries concerned in this widescale Siltzbach survey were unanimous in their view of the value and specificity of the Kveim test, but all were equally ignorant of its nature. J. D. REID (New Zealand) likened the slowly maturing Kveim test in sarcoidosis to the Mitsuda reaction in leprosy, and the Koch accelerated tubercle reaction in tuberculosis. He thought that these were true late reactions, recognisably different from immediate and delayed responses. T. PUTKONEN (Helsinki) believed that the most potent antigens were obtained from patients who themselves responded weakly to the Kveim test; and this might be a new clue to its mode of action. Sarcoid Granuloma Formation L. E. SILTZBACH showed the first known electron-microscopy preparations of the granuloma, which revealed macrophages in Kveim-test granulomas and in sarcoid lymph-nodes. He has also adopted the fluorescent antibody technique to demonstrate fibrinogen around and sometimes within such granulomas. No abnormal amounts of y-globulin or complement could be demonstrated in 8 Kveim-test sites or 5 sarcoid lymph-nodes. M. M. CUMMINGS (Bethesda) pointed out the acid-fast staining character of pine pollen and its ability to provoke sarcoid granulomas in animals. F. LINELL (Malmö) had studied several different Swedish pine pollens. He also found them to be acid-fast, but they could only provoke foreign-body granulomas in rabbits. A. LINDNER (Oklahoma) confirmed Cummings’ pioneer observations. He had found the biologically active (and epithelioid-cell provoking) substance in the benzene extract of pine pollen. G. TEILUM (Copenhagen) believed that the sarcoid granuloma results from stimulation of immune mechanisms; it began with mononuclear cell infiltration leading to epithelioid cell formation. He placed sarcoidosis in the same category as other mesenychymal disorders, including the collagen diseases. 1. Western
Mail, September 26, 1963.
H. L. ISRAEL (Philadelphia) analysed the biopsy sites of 350 patients with sarcoidosis. He chose skin, lymph-node, or any other site when it was obviously affected and accessible. Otherwise he advocated blind biopsy of mediastinal glands, lung, liver, scalene node, gastrocnemius, or bone-marrow. Like F. MURATORE (Italy), he thought that blind gastrocnemius muscle biopsy was safe, simple, and helpful. J. TURIAF (Paris) preferred bronchoscopic biopsy. He obtained sarcoid tissue from the bronchus in half of a series of 71 patients, even when the bronchial mucosa seemed normal. L. E. SILTZBACH found sarcoid granulomas by random biopsy of the hard palate of 10 of 26 patients with sarcoidosis, even when the hard palate looked normal. E. CARLENS (Stockholm) had developed a relatively safe technique for biopsy with mediastinoscopy. J. PATIALA (Helsinki) obtained histological confirmation in 22 of 25 cases by mediastinoscopy, but in none of 9 attempts to biopsy the scalene node. D. G. JAMES (London) thought this variable experience was due partly to the amount of material submitted to the pathologist and partly to his enthusiasm in cutting serial sections through the block. This extra labour undoubtedly increased the yield of miliary granulomas in aspiration liver biopsy material and the same would probably apply to gastrocnemius muscle biopsies. F. LINELL (Malmö) had obtained histological evidence of disseminated sarcoidosis in 43 of 6706 consecutive necropsies during 1957-62 in Maimo. Only 3 of these 43 had been clinically recognised, and, since most had died from causes other than sarcoidosis, this disease seems even more silent and prevalent than we realise.
Relationship to Tuberculosis SVEN LOFGREN (Stockholm) has found coexistent tuberculosis in only 0-7% of his 700 cases. J. G. SCADDING (London) recognised it in 8% of his 230 cases of pulmonary sarcoidosis. Latterly, there had been a swing away from the view that sarcoidosis and tuberculosis were closely related. Only a small minority of participants favoured a connection between the two diseases or treated sarcoidosis with antituberculous drugs. D. G. JAMES pointed out that the arguments for a tuberculous cause of sarcoidosis had always stemmed from chest physicians, who occasionally found the radiographic changes in both diseases confusingly similar. Modern ophthalmologists and dermatologists used topical corticosteroids for sarcoidosis with blithe disregard for flaring up possible tuberculous infection, and this was never, in fact, a complication of the prolonged steroid treatment of ocular or skin sarcoidosis. All participants were unanimous that B.C.G. vaccination did not lead to sarcoidosis nor did it favour the subsequent development of sarcoidosis.
Prognosis A. C. DOUGLAS (Edinburgh) gave an analysis of the fate of the various pulmonary changes in sarcoidosis. The prognosis of bilateral hilar lymphadenopathy, with or without erythema nodosum, was good; for 89 % had cleared spontaneously within two years. Interestingly, erythema nodosum did not accompany any of the group of 11 % in which hilar adenopathy persisted. By contrast, those with pulmonary infiltration had a worse prognosis, for only 60% achieved spontaneous resolution of the chest X-ray changes within two years. J. TURIAF’S experience of the natural history of early sarcoidosis in Paris was very similar. Miscellaneous Factors G. JÖRGENSEN (Gottingen) saw a significant relationship between sarcoidosis and blood-group A, thereby confirming an observation already made by J. G. LEWIS in London. Jorgensen estimated that persons with blood-group A had a 14% higher chance of acquiring sarcoidosis than patients with blood-group O. He could find no relation between sarcoidosis and rhesus-typing or serum haptoglobins. K. H. FRIED found that had a effect on often beneficial (Berlin) pregnancy the course of sarcoidosis, and thought there was no good argument for termination of pregnancy in this disease.
might have been responsible.1 Investigations are being pursued by the local health authorities, the Royal Society for the Prevention of Cruelty to Animals, and the National Canine Defence League.
Histology
kill foxes,
Conference SARCOIDOSIS THE third international congress on sarcoidosis was held in Stockholm on Sept. 11-14, under the auspices of the International Union Against Tuberculosis and the presidency of Dr. SVEN LÖFGREN (Stockholm).
Epidemiology The prevalence of pulmonary sarcoidosis, as revealed by mass minature radiography surveys in 20 countries, was approximately 5 per 100,000 in Czechoslovakia, Hungary, and Japan; 10 in Australia, Canada, Finland, France, Italy, Scotland, and Yugoslavia; 15-20 in England, Germany, the Netherlands, New Zealand, Poland, and Switzerland; 26 for Norway; and 60 in Sweden. The highest rates were usually in the 20-40 age-group; and there seemed to be no preponderance in either sex or in rural rather than urban populations. B. POLLACK (Montreal) reported that during the past decade mass surveys of the Eskimo population had revealed 1200 examples of tuberculosis, but no instance of sarcoidosis. J. S. CHAPMAN (Dallas) also emphasised racial disparity: in New York City the prevalence varied from 20 to 80 per 100,000, with the higher figure in the districts where Negroes
predominated. Kveim Test L. SILTZBACH (New York) thought that the Kveim test might be helpful in early detection and in providing more uniform international diagnostic criteria. The behaviour and incidence of positive responses to his Kveim antigen were similar in 21 countries on all continents, thereby supporting a unitarian concept of the disease. The representatives of all countries concerned in this widescale Siltzbach survey were unanimous in their view of the value and specificity of the Kveim test, but all were equally ignorant of its nature. J. D. REID (New Zealand) likened the slowly maturing Kveim test in sarcoidosis to the Mitsuda reaction in leprosy, and the Koch accelerated tubercle reaction in tuberculosis. He thought that these were true late reactions, recognisably different from immediate and delayed responses. T. PUTKONEN (Helsinki) believed that the most potent antigens were obtained from patients who themselves responded weakly to the Kveim test; and this might be a new clue to its mode of action. Sarcoid Granuloma Formation L. E. SILTZBACH showed the first known electron-microscopy preparations of the granuloma, which revealed macrophages in Kveim-test granulomas and in sarcoid lymph-nodes. He has also adopted the fluorescent antibody technique to demonstrate fibrinogen around and sometimes within such granulomas. No abnormal amounts of y-globulin or complement could be demonstrated in 8 Kveim-test sites or 5 sarcoid lymph-nodes. M. M. CUMMINGS (Bethesda) pointed out the acid-fast staining character of pine pollen and its ability to provoke sarcoid granulomas in animals. F. LINELL (Malmö) had studied several different Swedish pine pollens. He also found them to be acid-fast, but they could only provoke foreign-body granulomas in rabbits. A. LINDNER (Oklahoma) confirmed Cummings’ pioneer observations. He had found the biologically active (and epithelioid-cell provoking) substance in the benzene extract of pine pollen. G. TEILUM (Copenhagen) believed that the sarcoid granuloma results from stimulation of immune mechanisms; it began with mononuclear cell infiltration leading to epithelioid cell formation. He placed sarcoidosis in the same category as other mesenychymal disorders, including the collagen diseases. 1. Western
Mail, September 26, 1963.
H. L. ISRAEL (Philadelphia) analysed the biopsy sites of 350 patients with sarcoidosis. He chose skin, lymph-node, or any other site when it was obviously affected and accessible. Otherwise he advocated blind biopsy of mediastinal glands, lung, liver, scalene node, gastrocnemius, or bone-marrow. Like F. MURATORE (Italy), he thought that blind gastrocnemius muscle biopsy was safe, simple, and helpful. J. TURIAF (Paris) preferred bronchoscopic biopsy. He obtained sarcoid tissue from the bronchus in half of a series of 71 patients, even when the bronchial mucosa seemed normal. L. E. SILTZBACH found sarcoid granulomas by random biopsy of the hard palate of 10 of 26 patients with sarcoidosis, even when the hard palate looked normal. E. CARLENS (Stockholm) had developed a relatively safe technique for biopsy with mediastinoscopy. J. PATIALA (Helsinki) obtained histological confirmation in 22 of 25 cases by mediastinoscopy, but in none of 9 attempts to biopsy the scalene node. D. G. JAMES (London) thought this variable experience was due partly to the amount of material submitted to the pathologist and partly to his enthusiasm in cutting serial sections through the block. This extra labour undoubtedly increased the yield of miliary granulomas in aspiration liver biopsy material and the same would probably apply to gastrocnemius muscle biopsies. F. LINELL (Malmö) had obtained histological evidence of disseminated sarcoidosis in 43 of 6706 consecutive necropsies during 1957-62 in Maimo. Only 3 of these 43 had been clinically recognised, and, since most had died from causes other than sarcoidosis, this disease seems even more silent and prevalent than we realise.
Relationship to Tuberculosis SVEN LOFGREN (Stockholm) has found coexistent tuberculosis in only 0-7% of his 700 cases. J. G. SCADDING (London) recognised it in 8% of his 230 cases of pulmonary sarcoidosis. Latterly, there had been a swing away from the view that sarcoidosis and tuberculosis were closely related. Only a small minority of participants favoured a connection between the two diseases or treated sarcoidosis with antituberculous drugs. D. G. JAMES pointed out that the arguments for a tuberculous cause of sarcoidosis had always stemmed from chest physicians, who occasionally found the radiographic changes in both diseases confusingly similar. Modern ophthalmologists and dermatologists used topical corticosteroids for sarcoidosis with blithe disregard for flaring up possible tuberculous infection, and this was never, in fact, a complication of the prolonged steroid treatment of ocular or skin sarcoidosis. All participants were unanimous that B.C.G. vaccination did not lead to sarcoidosis nor did it favour the subsequent development of sarcoidosis.
Prognosis A. C. DOUGLAS (Edinburgh) gave an analysis of the fate of the various pulmonary changes in sarcoidosis. The prognosis of bilateral hilar lymphadenopathy, with or without erythema nodosum, was good; for 89 % had cleared spontaneously within two years. Interestingly, erythema nodosum did not accompany any of the group of 11 % in which hilar adenopathy persisted. By contrast, those with pulmonary infiltration had a worse prognosis, for only 60% achieved spontaneous resolution of the chest X-ray changes within two years. J. TURIAF’S experience of the natural history of early sarcoidosis in Paris was very similar. Miscellaneous Factors G. JÖRGENSEN (Gottingen) saw a significant relationship between sarcoidosis and blood-group A, thereby confirming an observation already made by J. G. LEWIS in London. Jorgensen estimated that persons with blood-group A had a 14% higher chance of acquiring sarcoidosis than patients with blood-group O. He could find no relation between sarcoidosis and rhesus-typing or serum haptoglobins. K. H. FRIED found that had a effect on often beneficial (Berlin) pregnancy the course of sarcoidosis, and thought there was no good argument for termination of pregnancy in this disease.