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ENDOMYOCARDIAL FIBROSIS AND FILARIASIS
428 filariasis
in the United Kingdom, we suggest that of the existence of this disease is important. Diagnosis will be made only if we maintain a high index of suspicion whenever we are confronted with a patient who comes from an endemic area. Department of Surgery, J. SPENCER Postgraduate Medical School, M. OWEN-SMITH. London, W.12. now
living
an awareness
ENDOMYOCARDIAL FIBROSIS AND FILARIASIS SiR,—The observations by Dr. Ive and Dr. Brockington (Jan. 22) on the association between endomyocardial fibrosis and certain types of filariasis in Nigeria call for some comment from Uganda. Endomyocardial fibrosis is encountered in Kampala probably as frequently as it is in the environs of Ibadan,l and while we have not specifically investigated our patients for evidence of filariasis, certain general comments can be made. Onchocerciasis in Uganda occurs in relatively small foci and nowhere does it occur in the " truly enormous and diffuse endemic areas which are characteristic of the disease in West Africa ".2 Almost all the province of Buganda, except for the west bank of the Victoria Nile (Jinja focus), is most probably completely free from the disease, judging from the absence of any record of Onchocerca volvulus, and from the unsuitable nature of the terrain for the vectors. When the villages of permanent residence of subjects coming to necropsy with endomyocardial fibrosis are plotted on the map of Buganda, the vast majority come from Bulemezi and Kyagwe in East Mengo and from Kyadondo and Busiro in West Mengo district. None of these areas are listed as proved or even as suspected foci of onchocerciasis, and remarkably few cases come from Bugerere, a very densely populated county on the west bank of the Nile. While all these subjects may have developed their endomyocardial fibrosis elsewhere and then moved to another area, this seems unlikely. Further, when the villages of permanent residence of clinically diagnosed patients with endomyocardial fibrosis are plotted, they follow the same pattern of distribution. Almost all subjects presenting at Mulago Hospital, Kampala, with endomyocardial fibrosis or coming to necropsy with this disorder are by tribal origin from Ruanda.3 Many of these people, however, have been born in Uganda or have spent most of their life here. Onchocerciasis is not recorded as a disease of any major significance in Ruanda, and in a review of African onchocerciasis Kirkdoes not include Ruanda or Burundi in his list of countries in which this infection has been recorded in the Tropical Diseases Bulletin between 1920 and 1957. It would be reasonable to conclude that subjects with endomyocardial fibrosis seen in Ibadan, Nigeria, have evidence of a greater exposure to one or other form of filariasis than subjects in Ibadan without endomyocardial fibrosis. This association has not been apparent in our experience in Uganda, and certainly the presence of clinical onchocerciasis in subjects with endomyocardial fibrosis has not been recorded. If Dr. Ive and Dr. Brockington are postulating a causal relation between filariasis and endomyocardial fibrosis, we would once again have to assume that endomyocardial fibrosis in Africa is the endpoint of many different disease processes,5 and that the disorders seen in East and West Africa have different aetiological backgrounds. On the other hand, it may be more reasonable to look upon the high incidence of filariasis in the Ibadan series as a marker, in the same way as we regard the tribal affiliation of subjects with endomyocardial fibrosis seen in Kampala as a marker of this particular group’s increased susceptibility to this disorder rather than as an indication of some inherent racial susceptibility. These immigrant subjects occupy an inferior economic and social position in Buganda society, even 1.
2. 3. 4. 5.
if they have been born and brought up here, and in our view it is this socioeconomic status which is of significance rather than the particular tribal affiliation or country of origin. We regard this disease not as a nutritional disorder in the purely dietary sense but rather as a disorder in which certain individuals, by virtue of their socioeconomic status, are subject to an increased susceptibility and exposure to the factors, be they bacterial, viral, or toxic, which initiate endomyocardial fibrosis and condition its pathogenesis. We suggest to our Nigerian colleagues that they look upon the filarial infection in their subjects with endomyocardial fibrosis as a marker rather than as an aaiological agent, and examine more closely those factors which are associated with the increased exposure of these patients to filariasis. Our work in this field is supported by the World Health Orgnnigatinn-
Departments of Medicine and Pathology, Makerere University College Medical School, Kampala, Uganda.
A. G. SHAPER R. M. COLES.
COMBINED MEDIASTINAL AND RETROPERITONEAL FIBROSIS SiR,—The article by Professor Morgan and his co-workers (Jan. 8) prompts me to record the following case.
The patient first attended this hospital in 1958 when he was aged 41 years. He was complaining of pain in the left side of his abdomen which he had noted intermittently in the past five years. There was no history of trauma to the abdomen, and he had never had any previous abdominal operations. Physical examination was negative. The following investigations showed no abnormality: culture of urine; estimation of serum electrolytes; Wassermann reaction; X-rays of chest and of lumbar, and lower thoracic, spine; barium meal and enema; intravenous and retrograde pyelography; and cystoscopy. Rectal-lavage cytology revealed no malignant cells. The sole positive findings were an erythrocyte-sedimentation rate of 28 mm. in the first hour, and a positive fsecal occultblood test. No definite cause for his pain could be determined. In 1960 the man was again seen when his pain became more intense, and on this occasion a lump could be felt deep in the left iliac fossa. Laparotomy showed the lump to be a firm " deposit " on the posterior abdominal wall. It was circular in outline (diameter about 4 cm. [11/2 in.]), and had a white shiny surface. There was a large mass in the body of the pancreas, involving the transverse mesocolon. It was thought that the patient probably had a pancreatic neoplasm, and that the lump in the left iliac fossa, which was removed completely, was a secondary deposit. Microscopy showed that the lump was composed of dense, hyalinised fibrous tissue containing a few spicules of bone. After operation he complained of pain in the shoulders, and was found to have bilateral winging of the scapulae, the cause of which remains obscure. No other neurological lesion could be detected. The patient continued much the same for 2 years. The pancreatic mass gradually became palpable in the epigastrium, and at repeat laparotomy in 1962 was found to occupy the greater part of the pancreas, and to have a very hard consistence with an irregular surface. It extended well back into the left loin, was still invading the transverse colon, and now completely surrounded the third part of the duodenum and the superior mesenteric vessels. The left kidney could not be felt as a separate entity. Numerous deposits with the same white shiny surface as that of the original deposit seen in 1960 lay along the line of the ileocolic vessels and the small bowel mesentery, and on the surface of the terminal ileum and ascending colon. A biopsy of the main mass and another of the nodules again showed dense collagenous fibrous tissue. There were foci of lymphocytes and numbers of eosinophils, Shaper, A. G., Williams, A. W. Trans. R. Soc. trop. Med. Hyg. 1960, 54, 12. but no evidence of an epithelial tumour. The pathologists Colbourne, M. J., Crosskey, R. W. Unpublished mimeographed W.H.O. document AFR/Onchocerciasis/30.65. agreed that the appearances were exactly similar to that of Shaper, A. G., Coles, R. M. Br. Heart J. 1965, 27, 121. the specimen removed two years before. They suggested that Kirk, R. Cent. Afr. J. Med. 1959, 5, 233. the entity was microscopically consistent with idiopathic 134. D. W. Br. 1961, ii, Abraham, G., Brigden, med. J.
in the United Kingdom, we suggest that of the existence of this disease is important. Diagnosis will be made only if we maintain a high index of suspicion whenever we are confronted with a patient who comes from an endemic area. Department of Surgery, J. SPENCER Postgraduate Medical School, M. OWEN-SMITH. London, W.12. now
living
an awareness
ENDOMYOCARDIAL FIBROSIS AND FILARIASIS SiR,—The observations by Dr. Ive and Dr. Brockington (Jan. 22) on the association between endomyocardial fibrosis and certain types of filariasis in Nigeria call for some comment from Uganda. Endomyocardial fibrosis is encountered in Kampala probably as frequently as it is in the environs of Ibadan,l and while we have not specifically investigated our patients for evidence of filariasis, certain general comments can be made. Onchocerciasis in Uganda occurs in relatively small foci and nowhere does it occur in the " truly enormous and diffuse endemic areas which are characteristic of the disease in West Africa ".2 Almost all the province of Buganda, except for the west bank of the Victoria Nile (Jinja focus), is most probably completely free from the disease, judging from the absence of any record of Onchocerca volvulus, and from the unsuitable nature of the terrain for the vectors. When the villages of permanent residence of subjects coming to necropsy with endomyocardial fibrosis are plotted on the map of Buganda, the vast majority come from Bulemezi and Kyagwe in East Mengo and from Kyadondo and Busiro in West Mengo district. None of these areas are listed as proved or even as suspected foci of onchocerciasis, and remarkably few cases come from Bugerere, a very densely populated county on the west bank of the Nile. While all these subjects may have developed their endomyocardial fibrosis elsewhere and then moved to another area, this seems unlikely. Further, when the villages of permanent residence of clinically diagnosed patients with endomyocardial fibrosis are plotted, they follow the same pattern of distribution. Almost all subjects presenting at Mulago Hospital, Kampala, with endomyocardial fibrosis or coming to necropsy with this disorder are by tribal origin from Ruanda.3 Many of these people, however, have been born in Uganda or have spent most of their life here. Onchocerciasis is not recorded as a disease of any major significance in Ruanda, and in a review of African onchocerciasis Kirkdoes not include Ruanda or Burundi in his list of countries in which this infection has been recorded in the Tropical Diseases Bulletin between 1920 and 1957. It would be reasonable to conclude that subjects with endomyocardial fibrosis seen in Ibadan, Nigeria, have evidence of a greater exposure to one or other form of filariasis than subjects in Ibadan without endomyocardial fibrosis. This association has not been apparent in our experience in Uganda, and certainly the presence of clinical onchocerciasis in subjects with endomyocardial fibrosis has not been recorded. If Dr. Ive and Dr. Brockington are postulating a causal relation between filariasis and endomyocardial fibrosis, we would once again have to assume that endomyocardial fibrosis in Africa is the endpoint of many different disease processes,5 and that the disorders seen in East and West Africa have different aetiological backgrounds. On the other hand, it may be more reasonable to look upon the high incidence of filariasis in the Ibadan series as a marker, in the same way as we regard the tribal affiliation of subjects with endomyocardial fibrosis seen in Kampala as a marker of this particular group’s increased susceptibility to this disorder rather than as an indication of some inherent racial susceptibility. These immigrant subjects occupy an inferior economic and social position in Buganda society, even 1.
2. 3. 4. 5.
if they have been born and brought up here, and in our view it is this socioeconomic status which is of significance rather than the particular tribal affiliation or country of origin. We regard this disease not as a nutritional disorder in the purely dietary sense but rather as a disorder in which certain individuals, by virtue of their socioeconomic status, are subject to an increased susceptibility and exposure to the factors, be they bacterial, viral, or toxic, which initiate endomyocardial fibrosis and condition its pathogenesis. We suggest to our Nigerian colleagues that they look upon the filarial infection in their subjects with endomyocardial fibrosis as a marker rather than as an aaiological agent, and examine more closely those factors which are associated with the increased exposure of these patients to filariasis. Our work in this field is supported by the World Health Orgnnigatinn-
Departments of Medicine and Pathology, Makerere University College Medical School, Kampala, Uganda.
A. G. SHAPER R. M. COLES.
COMBINED MEDIASTINAL AND RETROPERITONEAL FIBROSIS SiR,—The article by Professor Morgan and his co-workers (Jan. 8) prompts me to record the following case.
The patient first attended this hospital in 1958 when he was aged 41 years. He was complaining of pain in the left side of his abdomen which he had noted intermittently in the past five years. There was no history of trauma to the abdomen, and he had never had any previous abdominal operations. Physical examination was negative. The following investigations showed no abnormality: culture of urine; estimation of serum electrolytes; Wassermann reaction; X-rays of chest and of lumbar, and lower thoracic, spine; barium meal and enema; intravenous and retrograde pyelography; and cystoscopy. Rectal-lavage cytology revealed no malignant cells. The sole positive findings were an erythrocyte-sedimentation rate of 28 mm. in the first hour, and a positive fsecal occultblood test. No definite cause for his pain could be determined. In 1960 the man was again seen when his pain became more intense, and on this occasion a lump could be felt deep in the left iliac fossa. Laparotomy showed the lump to be a firm " deposit " on the posterior abdominal wall. It was circular in outline (diameter about 4 cm. [11/2 in.]), and had a white shiny surface. There was a large mass in the body of the pancreas, involving the transverse mesocolon. It was thought that the patient probably had a pancreatic neoplasm, and that the lump in the left iliac fossa, which was removed completely, was a secondary deposit. Microscopy showed that the lump was composed of dense, hyalinised fibrous tissue containing a few spicules of bone. After operation he complained of pain in the shoulders, and was found to have bilateral winging of the scapulae, the cause of which remains obscure. No other neurological lesion could be detected. The patient continued much the same for 2 years. The pancreatic mass gradually became palpable in the epigastrium, and at repeat laparotomy in 1962 was found to occupy the greater part of the pancreas, and to have a very hard consistence with an irregular surface. It extended well back into the left loin, was still invading the transverse colon, and now completely surrounded the third part of the duodenum and the superior mesenteric vessels. The left kidney could not be felt as a separate entity. Numerous deposits with the same white shiny surface as that of the original deposit seen in 1960 lay along the line of the ileocolic vessels and the small bowel mesentery, and on the surface of the terminal ileum and ascending colon. A biopsy of the main mass and another of the nodules again showed dense collagenous fibrous tissue. There were foci of lymphocytes and numbers of eosinophils, Shaper, A. G., Williams, A. W. Trans. R. Soc. trop. Med. Hyg. 1960, 54, 12. but no evidence of an epithelial tumour. The pathologists Colbourne, M. J., Crosskey, R. W. Unpublished mimeographed W.H.O. document AFR/Onchocerciasis/30.65. agreed that the appearances were exactly similar to that of Shaper, A. G., Coles, R. M. Br. Heart J. 1965, 27, 121. the specimen removed two years before. They suggested that Kirk, R. Cent. Afr. J. Med. 1959, 5, 233. the entity was microscopically consistent with idiopathic 134. D. W. Br. 1961, ii, Abraham, G., Brigden, med. J.