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PULMONARY HÆMOSIDEROSIS REPORT OF A CASE
540
PULMONARY HÆMOSIDEROSIS REPORT OF A CASE
R. E. W. FISHER M.B. Belf., D.P.H., D.I.H. CHIEF MEDICAL MEDICAL
R. MAKIN M.B. Edin. MEDICAL
OFFICER
DEPARTMENT,
SOUTH EASTERN
OFFICER
GAS BOARD
With illustrations on plate RECENT articles by Lendrum, Scott and Park (1950) and Ellman and Gee (1951) have recorded their observations on the secondary effects of cardiac disease on the lungs. We have been studying a case of pulmonary haemosiderosis and mitral stenosis which is of particular interest in that the patient remains comparatively well and continues to perform his normal work, yet the definite due to over a
radiological development of pulmonary changes circulatory back-pressure has been observed period of three years. CASE-REPORT
We first
the patient at a routine medical examination in September, 1947. He was then 22 years of age and he had just completed three years’ service as a stoker in the Royal Navy. He gave no history of serious illness and his parents and he have since asserted quite definitely that he has never had rheumatic fever or chorea. Although an occasional apical systolic murmur was heard at this examination, he was accepted for employment. In July, 1948, he was found to have a definite apical presystolic murmur but his exercise- tolerance was regarded as normal and he was, in fact, managing fairly heavy manual work perfectly well. Soon after this examination he was admitted to hospital complaining of shortness of breath on exertion. Apparently no cardiac lesion was discovered while he was in hospital and the X-ray appearances of his lungs in August and November, 1948, were considered normal. The diagnosis was anaemia and general debility." On discharge from hospital he resumed work and remained well until March, 1949, when he coughed up a small quantity of blood and noticed that he was again somewhat breathless on exertion. He looked pale and ill and the presystolic murmur was still present. Radiography of his chest now showed the bilateral middle zone opacities of pulmonary cedema (fig. 1). A blood examination showed an orthochromic anaemia with a haemoglobin value of 45%. The Wassermann reaction was negative. He was re-examined in November, 1949, having been absent from work for six months. He felt perfectly fit, had no cough, and was not breathless, even on exertion. Radiography, however, showed mottling of both lung fields excluding the apices, and some fluid in the left pleural space seeping into the interlobar fissure (fig. 2). We suggest that these radiological appearances represent further steps in the pathological effects of chronic back-pressure in the pulmonary circulation : they are early evidence of the deposition of iron pigment in the cedematous lung and the transudation of fluid into the pleural cavity. Except for a period of one month off work after a slight haemoptysis in May, 1950, he remained well and continued work as a storekeeper until he was again examined in March, 1951. At that time he felt fairly well but was occasionally short of breath. The presystolic murmur was still present saw
"
and the blood-pressure was 106/64 mm. Hg. X-ray examination on this occasion showed haemosiderosis affecting all areas, except the apices, of both lungs and, in addition, the presence of fluid in the right pleural space (fig. 3). In April, 1951, he had another slight haemoptysis, consisting of blood-streaked sputum, and he rested at home for three
DOLTON, DR. EVERLEY JONES : REFERENCES Apley, J. (1949) Proc. R. Soc. Med. 42, 918. Bayford, D. (1789) 1Hem. med. Soc. -Lond. 2, 271. Edwards, J. E. (1948) Cited by Brown, J. W. (1950) Congenital 2nd ed., London. Heart Disease. Gross, R. E. (1945) New Engl. J. Afed. 233, 586. Neuhauser, E. B. D. (1948) Amer. J. Dis. Child. 75, 570. (1951) Pediatrics, 7, 69. Ware, P. F. (1946) Surg. Gynec. Obstet. 83, 435. Holzapfel, G. (1899) Anat, Hefte. 12, 369. Hunand (1735) Cited by Gross, R. E., Ware, P. F. (1946) Surg. MR.
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—
—
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Gynee. Obstet. 83, 435. Neuhauser, E. B. D. (1946) Amer. J. Roentgenol. 56, 1. BVoJman,1. J. (1939) J. Pcdiat. 14, 527.
weeks. On resuming work in May, he stated that although he was certainly breathless as a result of any excessive exertion, such as running a short distance for a bus, he felt perfectly well. He looked pale but not ill. A well-marked apical presystolic murmur was still present and the pulmonary second sound was greatly accentuated. The blood-pressure A few added moist sounds and slight dullness was 125/80. were present at both bases and pulmonary arterial pulsation There was no oedema of the was audible over both lungs. ankles. Radiography of the chest showed no extension of the hæmosiderosis, but fluid was now present in both pleural cavities. The combination of frequent small hæmoptyses, dyspnœa on exertion, a persistent apical presystolic murmur and the radiographic appearance of pulmonary oedema leave little doubt that this patient suffers from mitral stenosis, which we regard as the basic cause of the various radiological changes described. DISCUSSION
This case is an example of a mitral stenosis compensated, at least temporarily, by a right ventricular hypertrophy, but only at the expense of raising the blood-pressure in the pulmonary arteries above the pressure in the bronchial arteries. Lendrum et al. (1950) point out that this must lead, sooner or later, to haemorrhage from
the
bronchopulmonary anastomoses, sometimes sufficient produce haemoptysis. They attribute hoemosiderosis occurring in mitral stenosis to multiple small hoemorrhages, most of which do not produce frank blood in the sputum. On the other hand, Kerley (1951) thinks that hsemosiderin is deposited in the lungs because it is insoluble in the alkaline medium of the oedematous lung. This means that pulmonary oedema must precede the appearance of hæmosiderin in the lung. Doig and McLaughlin (1948) have shown that the radiological changes in welders’ siderosis may disappear. This is presumably because the inorganic iron causing this disease is removed by phagocytosis. The nodules’ of pulmonary hæmosiderosis have little, if any, tendency to resolve (Prendergrass et al. 1949) ; so presumably, the phagocytes are to
unable to
remove
the iron.
Indeed,
as
Ellman and, Gee
point out, the condition would not occur at all if phagocytosis were effective. The same authors quote the work of Strassman (1944) which showed that, in a normal rabbit lung, phagocytosis of injected red cells was effective and rapid ; but, in the presence of pulmonary oedema, phagocytosis was much delayed. They go on to quote the suggestion of Gumpert (1947) that " oedema and venous congestion cause some obstruction to the normal lymphatic drainage of the alveolar histiocytes responsible for the removal of intra-alveolar red cells." We were interested in this case because the X-ray films showed the presence of pulmonary cedema before the nodular shadows of hsemosiderosis appeared. This is in keeping with the pathological sequence suggested by Ellman and Gee. The case also gives some indication of the time taken for pulmonary hæmosiderosis to become apparent radiologically. Thus, this patient was said to have a normal chest radiograph in November, 1948. In March, 1949, the appearance was that of pulmonary oedema and some eight months later there was evidence of early pulmonary heemosiderosis. After a further sixteen months, the changes were those typical of hæmosiderosis. We wish to express our thanks to Dr. Andrew Meiklejohn for his advice and to the South Eastern Gas Board for permission to publish this case. REFERENCES
Doig, A. T., McLaughlin, A. I. G. (1948) Lancet, i, 789. Ellman, P., Gee, A. (1951) Brit. med. J. ii, 384. Gumpert, T. E. (1947) Ibid, ii, 488. Kerley, P. (1951). In Shanks and Kerley’s Textbook of X-ray Diagnosis. London ; vol. II, p. 179. Lendrum, A. C., Scott, L. D. W., Park, S. D. S. (1950) Quart. J. Med. 19, 249. Prendergrass, E. P., Lane, E. L., Ostrum, H. W. (1949) Amer. J. Roentgenol. 61, 443. Strassman, G. (1944) Arch. Path. 38, 76.
PULMONARY HÆMOSIDEROSIS REPORT OF A CASE
R. E. W. FISHER M.B. Belf., D.P.H., D.I.H. CHIEF MEDICAL MEDICAL
R. MAKIN M.B. Edin. MEDICAL
OFFICER
DEPARTMENT,
SOUTH EASTERN
OFFICER
GAS BOARD
With illustrations on plate RECENT articles by Lendrum, Scott and Park (1950) and Ellman and Gee (1951) have recorded their observations on the secondary effects of cardiac disease on the lungs. We have been studying a case of pulmonary haemosiderosis and mitral stenosis which is of particular interest in that the patient remains comparatively well and continues to perform his normal work, yet the definite due to over a
radiological development of pulmonary changes circulatory back-pressure has been observed period of three years. CASE-REPORT
We first
the patient at a routine medical examination in September, 1947. He was then 22 years of age and he had just completed three years’ service as a stoker in the Royal Navy. He gave no history of serious illness and his parents and he have since asserted quite definitely that he has never had rheumatic fever or chorea. Although an occasional apical systolic murmur was heard at this examination, he was accepted for employment. In July, 1948, he was found to have a definite apical presystolic murmur but his exercise- tolerance was regarded as normal and he was, in fact, managing fairly heavy manual work perfectly well. Soon after this examination he was admitted to hospital complaining of shortness of breath on exertion. Apparently no cardiac lesion was discovered while he was in hospital and the X-ray appearances of his lungs in August and November, 1948, were considered normal. The diagnosis was anaemia and general debility." On discharge from hospital he resumed work and remained well until March, 1949, when he coughed up a small quantity of blood and noticed that he was again somewhat breathless on exertion. He looked pale and ill and the presystolic murmur was still present. Radiography of his chest now showed the bilateral middle zone opacities of pulmonary cedema (fig. 1). A blood examination showed an orthochromic anaemia with a haemoglobin value of 45%. The Wassermann reaction was negative. He was re-examined in November, 1949, having been absent from work for six months. He felt perfectly fit, had no cough, and was not breathless, even on exertion. Radiography, however, showed mottling of both lung fields excluding the apices, and some fluid in the left pleural space seeping into the interlobar fissure (fig. 2). We suggest that these radiological appearances represent further steps in the pathological effects of chronic back-pressure in the pulmonary circulation : they are early evidence of the deposition of iron pigment in the cedematous lung and the transudation of fluid into the pleural cavity. Except for a period of one month off work after a slight haemoptysis in May, 1950, he remained well and continued work as a storekeeper until he was again examined in March, 1951. At that time he felt fairly well but was occasionally short of breath. The presystolic murmur was still present saw
"
and the blood-pressure was 106/64 mm. Hg. X-ray examination on this occasion showed haemosiderosis affecting all areas, except the apices, of both lungs and, in addition, the presence of fluid in the right pleural space (fig. 3). In April, 1951, he had another slight haemoptysis, consisting of blood-streaked sputum, and he rested at home for three
DOLTON, DR. EVERLEY JONES : REFERENCES Apley, J. (1949) Proc. R. Soc. Med. 42, 918. Bayford, D. (1789) 1Hem. med. Soc. -Lond. 2, 271. Edwards, J. E. (1948) Cited by Brown, J. W. (1950) Congenital 2nd ed., London. Heart Disease. Gross, R. E. (1945) New Engl. J. Afed. 233, 586. Neuhauser, E. B. D. (1948) Amer. J. Dis. Child. 75, 570. (1951) Pediatrics, 7, 69. Ware, P. F. (1946) Surg. Gynec. Obstet. 83, 435. Holzapfel, G. (1899) Anat, Hefte. 12, 369. Hunand (1735) Cited by Gross, R. E., Ware, P. F. (1946) Surg. MR.
-
—
—
-
Gynee. Obstet. 83, 435. Neuhauser, E. B. D. (1946) Amer. J. Roentgenol. 56, 1. BVoJman,1. J. (1939) J. Pcdiat. 14, 527.
weeks. On resuming work in May, he stated that although he was certainly breathless as a result of any excessive exertion, such as running a short distance for a bus, he felt perfectly well. He looked pale but not ill. A well-marked apical presystolic murmur was still present and the pulmonary second sound was greatly accentuated. The blood-pressure A few added moist sounds and slight dullness was 125/80. were present at both bases and pulmonary arterial pulsation There was no oedema of the was audible over both lungs. ankles. Radiography of the chest showed no extension of the hæmosiderosis, but fluid was now present in both pleural cavities. The combination of frequent small hæmoptyses, dyspnœa on exertion, a persistent apical presystolic murmur and the radiographic appearance of pulmonary oedema leave little doubt that this patient suffers from mitral stenosis, which we regard as the basic cause of the various radiological changes described. DISCUSSION
This case is an example of a mitral stenosis compensated, at least temporarily, by a right ventricular hypertrophy, but only at the expense of raising the blood-pressure in the pulmonary arteries above the pressure in the bronchial arteries. Lendrum et al. (1950) point out that this must lead, sooner or later, to haemorrhage from
the
bronchopulmonary anastomoses, sometimes sufficient produce haemoptysis. They attribute hoemosiderosis occurring in mitral stenosis to multiple small hoemorrhages, most of which do not produce frank blood in the sputum. On the other hand, Kerley (1951) thinks that hsemosiderin is deposited in the lungs because it is insoluble in the alkaline medium of the oedematous lung. This means that pulmonary oedema must precede the appearance of hæmosiderin in the lung. Doig and McLaughlin (1948) have shown that the radiological changes in welders’ siderosis may disappear. This is presumably because the inorganic iron causing this disease is removed by phagocytosis. The nodules’ of pulmonary hæmosiderosis have little, if any, tendency to resolve (Prendergrass et al. 1949) ; so presumably, the phagocytes are to
unable to
remove
the iron.
Indeed,
as
Ellman and, Gee
point out, the condition would not occur at all if phagocytosis were effective. The same authors quote the work of Strassman (1944) which showed that, in a normal rabbit lung, phagocytosis of injected red cells was effective and rapid ; but, in the presence of pulmonary oedema, phagocytosis was much delayed. They go on to quote the suggestion of Gumpert (1947) that " oedema and venous congestion cause some obstruction to the normal lymphatic drainage of the alveolar histiocytes responsible for the removal of intra-alveolar red cells." We were interested in this case because the X-ray films showed the presence of pulmonary cedema before the nodular shadows of hsemosiderosis appeared. This is in keeping with the pathological sequence suggested by Ellman and Gee. The case also gives some indication of the time taken for pulmonary hæmosiderosis to become apparent radiologically. Thus, this patient was said to have a normal chest radiograph in November, 1948. In March, 1949, the appearance was that of pulmonary oedema and some eight months later there was evidence of early pulmonary heemosiderosis. After a further sixteen months, the changes were those typical of hæmosiderosis. We wish to express our thanks to Dr. Andrew Meiklejohn for his advice and to the South Eastern Gas Board for permission to publish this case. REFERENCES
Doig, A. T., McLaughlin, A. I. G. (1948) Lancet, i, 789. Ellman, P., Gee, A. (1951) Brit. med. J. ii, 384. Gumpert, T. E. (1947) Ibid, ii, 488. Kerley, P. (1951). In Shanks and Kerley’s Textbook of X-ray Diagnosis. London ; vol. II, p. 179. Lendrum, A. C., Scott, L. D. W., Park, S. D. S. (1950) Quart. J. Med. 19, 249. Prendergrass, E. P., Lane, E. L., Ostrum, H. W. (1949) Amer. J. Roentgenol. 61, 443. Strassman, G. (1944) Arch. Path. 38, 76.