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Diffuse proliferative and exudative glomerulonephritis
Pathology (1973), 5, pp. 69-82
ABSTRACTS OF PAPERS PRESENTED AT THE ANNUAL M E E T I N G OF T H E ROYAL COLLEGE OF PATHOLOGISTS OF AUSTRALIA HELD I N ADELAIDE, SOUTH AUSTRALIA October 1972
FlBRlNOlD CHANGE I N RENAL DISEASE
MEADOWS, R. Histopathology Department, The Queen Elizabeth Hospital, Woodville, South Australia Fibrinoid change is seen as part of the histopathology of four disease entities which may present with clear-cut clinical features : (1) In malignant hypertension it is seen most frequently when the blood pressure is poorly controlled and when renal failure is present. It is not seen in all cases and the diagnostic lesion in malignant hypertension is a proliferative endarteritis. (2) Fibrinoid change of the arterial and arteriolar walls is a frequent accompaniment of the intravascular coagulation which characterizes the haemolytic-uraemic syndrome.
(3) Whilst the rather mucoid proliferative endarteritis is the characteristic lesion of scleroderma, fibrinoid change is not uncommon and may be particularly florid in the more acute forms of this disease. (4) Although not particularly common, fibrinoid change may be one of the manifestations of the vascular form of transplant rejection.
One problem in the differential diagnosis of these conditions is that malignant hypertension is frequently a cornplicating factor in other diseases. Although with immunofluorescent studies and electron microscopy some assistance may be obtained in arriving at the correct diagnosis, clinicopathological correlation affords the only reliable way of making a definitive diagnosis. The fact that four diseases with seemingly different aetiologies may have very similar morphological features may reflect the pathological maxim that organs can only react to disease in a limited number of ways. However, it also suggests that the aetiologies may not, in fact, be so diverse as IS usually imagined.
DIFFUSE PROLIFERATIVE A N D EXUDATIVE GLOMERULONEPHRITIS
HERDSON, P. B. Department of Pathology, University of Auckland School of Medicine, Auckland, New Zealand During the past 3 yr, we have studied 380 renal biopsies, of which 60 have been examples of acute diffuse proliferative and exudative glomerulonephritis. In this group, there have been twice as many males as females, with a large proportion of Polynesians, and we have seen a seasonal variation in incidence.
70
THE R O Y A L C O L L E G E O F P A T H O L O G I S T S O F A U S T R A L I A
The biopsies have been examined by light, immunofluorescent and electron microscopy, and the fine structural abnormalities of this type of glomerulonephritis have been further defined. The morphological features of proven post-streptococcal acute diffuse glomerulonephritis appear to be indistinguishable from cases associated with staphylococcal infection, and also from cases in which an association with such infective agents has not been established. The series included some sequential biopsies, which illustrated aspects of the natural history of this type of glomerulonephritis.
OPEN LUNG BIOPSY I N JELIHOVSKY,
THE DIAGNOSIS OF DIFFUSE LUNG DISEASE
TATIANA. Department of Histopathology, Royal Prince Alfred Hospiral,
Sydney, New South Wales
At Royal Prince Alfred Hospital, during the past 18 yr, 56 patients with diffuse lung disease were subjected to thoracotomy to obtain a lung biopsy. Most specimens were taken from the sharp margin of the lung and only a few from the lateral surface. In 9 patients the appearances were suggestive of allergic alveolitis. One of these had farmer’s lung and 3 had been exposed to birds. Six specimens showed desquamative interstitial pneumonia, 2 with severe honeycombing, 2 with early honeycombing and 2 without fibrosis. In one of the latter, the changes were mild and there were also some intranuclear inclusions. Two patients had asbestosis and in both, the histological appearances otherwise resembled desquamative interstitial pneumonia. There were 4 biopsies from patients with eosinophilic granuloma, 3 in the active phase and one healing. Non-specific interstitial pneumonia was found in 5 and was associated with bronchiolitis obliterans in 2. There were single cases of moderately severe emphysema with bronchiolitis, bronchiolitis with muscle hypertrophy and air trapping, lymphosarcoma, eosinophilic pneumonia, probable tuberculosis, probable sarcoidosis, idiopathic pulmonary haemosiderosis, lymphomatoid Wegener’s granulomatosis and probable limited form of Wegener’s granulomatosis. Eleven other biopsies showed some honeycombing with a variety of non-specific features and in the remaining 10 cases the changes were mild or non-specific.
THE MARFAN SYNDROME IN INFANTS, WITH EMPHASIS ON THE P ULM0 NARY PATH 0 LO GY
BALE,PATRICIA M. & REYE,R. D. Institute of Pathology, Royal Alexandra Hospital for Children, Camperdown, New South Wales During the last 10 yr, necropsies have been performed on 4 infants with the Marfan syndrome. Clinically the arachnodactyly was so striking that each baby was admitted soon after birth with a diagnosis by the local practitioner of ‘ ? Marfan’s syndrome’. Each also had hypotonia or hyperextensible joints; other musculoskeletal changes were high arched palate in 3 and eventration of the right hemidiaphragm in 3 patients. Death occurred between 5 wk and 9 mth of age, of what was considered clinically to be cardiac failure. At necropsy, all 4 infants had the characteristic cardiac lesions of large redundant atrioventricular valve cusps, and post-valvular dilatarion of pulmonary trunk or aorta or both. All except the youngest child had left ventricular hypertrophy. Ocular changes, at least 3 of which were present in each infant, included ectopia lentis, spherophakia, deep anterior chamber, glaucoma, focal choroidal proliferation and blue sclera. A finding seldom emphasized but so pronounced as to suggest a non-cardiac mode of death, was the presence of lobar or more widespread emphysema in all cases. This differed from congenital lobar emphysema in that bronchial cartilage and muscle were well developed rather than deficient. On the orher hand, the lungs in the Marfan syndrome all showed an irregularity of elastica in alveolar ducts and sacs, not seen in congenital emphysema or in normal controls.
ABSTRACTS OF PAPERS PRESENTED AT THE ANNUAL M E E T I N G OF T H E ROYAL COLLEGE OF PATHOLOGISTS OF AUSTRALIA HELD I N ADELAIDE, SOUTH AUSTRALIA October 1972
FlBRlNOlD CHANGE I N RENAL DISEASE
MEADOWS, R. Histopathology Department, The Queen Elizabeth Hospital, Woodville, South Australia Fibrinoid change is seen as part of the histopathology of four disease entities which may present with clear-cut clinical features : (1) In malignant hypertension it is seen most frequently when the blood pressure is poorly controlled and when renal failure is present. It is not seen in all cases and the diagnostic lesion in malignant hypertension is a proliferative endarteritis. (2) Fibrinoid change of the arterial and arteriolar walls is a frequent accompaniment of the intravascular coagulation which characterizes the haemolytic-uraemic syndrome.
(3) Whilst the rather mucoid proliferative endarteritis is the characteristic lesion of scleroderma, fibrinoid change is not uncommon and may be particularly florid in the more acute forms of this disease. (4) Although not particularly common, fibrinoid change may be one of the manifestations of the vascular form of transplant rejection.
One problem in the differential diagnosis of these conditions is that malignant hypertension is frequently a cornplicating factor in other diseases. Although with immunofluorescent studies and electron microscopy some assistance may be obtained in arriving at the correct diagnosis, clinicopathological correlation affords the only reliable way of making a definitive diagnosis. The fact that four diseases with seemingly different aetiologies may have very similar morphological features may reflect the pathological maxim that organs can only react to disease in a limited number of ways. However, it also suggests that the aetiologies may not, in fact, be so diverse as IS usually imagined.
DIFFUSE PROLIFERATIVE A N D EXUDATIVE GLOMERULONEPHRITIS
HERDSON, P. B. Department of Pathology, University of Auckland School of Medicine, Auckland, New Zealand During the past 3 yr, we have studied 380 renal biopsies, of which 60 have been examples of acute diffuse proliferative and exudative glomerulonephritis. In this group, there have been twice as many males as females, with a large proportion of Polynesians, and we have seen a seasonal variation in incidence.
70
THE R O Y A L C O L L E G E O F P A T H O L O G I S T S O F A U S T R A L I A
The biopsies have been examined by light, immunofluorescent and electron microscopy, and the fine structural abnormalities of this type of glomerulonephritis have been further defined. The morphological features of proven post-streptococcal acute diffuse glomerulonephritis appear to be indistinguishable from cases associated with staphylococcal infection, and also from cases in which an association with such infective agents has not been established. The series included some sequential biopsies, which illustrated aspects of the natural history of this type of glomerulonephritis.
OPEN LUNG BIOPSY I N JELIHOVSKY,
THE DIAGNOSIS OF DIFFUSE LUNG DISEASE
TATIANA. Department of Histopathology, Royal Prince Alfred Hospiral,
Sydney, New South Wales
At Royal Prince Alfred Hospital, during the past 18 yr, 56 patients with diffuse lung disease were subjected to thoracotomy to obtain a lung biopsy. Most specimens were taken from the sharp margin of the lung and only a few from the lateral surface. In 9 patients the appearances were suggestive of allergic alveolitis. One of these had farmer’s lung and 3 had been exposed to birds. Six specimens showed desquamative interstitial pneumonia, 2 with severe honeycombing, 2 with early honeycombing and 2 without fibrosis. In one of the latter, the changes were mild and there were also some intranuclear inclusions. Two patients had asbestosis and in both, the histological appearances otherwise resembled desquamative interstitial pneumonia. There were 4 biopsies from patients with eosinophilic granuloma, 3 in the active phase and one healing. Non-specific interstitial pneumonia was found in 5 and was associated with bronchiolitis obliterans in 2. There were single cases of moderately severe emphysema with bronchiolitis, bronchiolitis with muscle hypertrophy and air trapping, lymphosarcoma, eosinophilic pneumonia, probable tuberculosis, probable sarcoidosis, idiopathic pulmonary haemosiderosis, lymphomatoid Wegener’s granulomatosis and probable limited form of Wegener’s granulomatosis. Eleven other biopsies showed some honeycombing with a variety of non-specific features and in the remaining 10 cases the changes were mild or non-specific.
THE MARFAN SYNDROME IN INFANTS, WITH EMPHASIS ON THE P ULM0 NARY PATH 0 LO GY
BALE,PATRICIA M. & REYE,R. D. Institute of Pathology, Royal Alexandra Hospital for Children, Camperdown, New South Wales During the last 10 yr, necropsies have been performed on 4 infants with the Marfan syndrome. Clinically the arachnodactyly was so striking that each baby was admitted soon after birth with a diagnosis by the local practitioner of ‘ ? Marfan’s syndrome’. Each also had hypotonia or hyperextensible joints; other musculoskeletal changes were high arched palate in 3 and eventration of the right hemidiaphragm in 3 patients. Death occurred between 5 wk and 9 mth of age, of what was considered clinically to be cardiac failure. At necropsy, all 4 infants had the characteristic cardiac lesions of large redundant atrioventricular valve cusps, and post-valvular dilatarion of pulmonary trunk or aorta or both. All except the youngest child had left ventricular hypertrophy. Ocular changes, at least 3 of which were present in each infant, included ectopia lentis, spherophakia, deep anterior chamber, glaucoma, focal choroidal proliferation and blue sclera. A finding seldom emphasized but so pronounced as to suggest a non-cardiac mode of death, was the presence of lobar or more widespread emphysema in all cases. This differed from congenital lobar emphysema in that bronchial cartilage and muscle were well developed rather than deficient. On the orher hand, the lungs in the Marfan syndrome all showed an irregularity of elastica in alveolar ducts and sacs, not seen in congenital emphysema or in normal controls.