Aus:. RJdiol. (1976). 20,232
Amyloidosis Causing Right Middle Lobe Atelectasis K. R. THOMSON, M.B.,Ch.B., M.R.A.C.R., D. B. SPRING, M.D., and R. E. HANSON,M.D. Department of Diagnostic Radiology, Royal Victoria Hospital, Montreal, Quebec, Canada cytological studies of his sputum were also negative. Protein electrophoresis and immunoelectrophoresis were both normal. Chest roentgenograms (Figures 1 and 2) demonstrated middle lobe atelectasis and loss of the normal right hilar anatomy, best seen on the lateral projection. Lateral tomography (Figure 3) demonstrated severe loss of volume of the middle lobe with patency of thc segmental bronchi. Fiberoptic bronchoscopy revealed complete obstruction of the middle lobe bronchus and
INTRODUCTION The roentgenographic and bronchoscopic findings in a case of diffuse tracheobronchial amyloidosis presenting as middle lobe atelectasis are described. This case is unusual in that 1) the bronchoscopy was unremarkable except for right middle lobe bronchial occlusion, and 2) tomography showed patent segmental middle lobe bronchi in the presence of proximal occlusion of the middle lobe bronchus. CASEREPORT A 6 1-year-old retired male cabinetmaker
presented with a seven-month history of loose cough productive of blood-tinged sputum. He had been a heavy smoker until six years previously. Physical examination and initial laboratory studies were negative. Bacteriological and
FIGURE1-P.A.
232
Chest-Area of consolidation (R) middle lobe.
FIGURE 2-Lateral Chest-Atelectasis of right middle lobe and loss of normal ( R ) hilar anatomy.
A ustrulmian Rudiology, Vol. X X ,No. 3. September. 1976
AMYLOIDOSIS CAUSING RIGHTMIDDLELOBEATELECTASIS
FIGURE 3-Loterul Torriopurii-Note that middle lobe bronchi appear patent (arrows). The loss of volume of the middle lobe involves mostly the medial segment.
minimal deformity of the right upper lobe bronchus. The mucosa appeared normal. Brush biopsy performed at that time was normal. A right bronchogram (Figure 4) confirmed occlusion of the middle lobe bronchus. In addition, there was fusiform narrowing of the intermediate bronchus and proximal stenosis of several segmental bronchi in the right upper and lower lobes (Figure 5). Forceps biopsy on repeat bronchoscopy revealed diffuse submucosal amyloid deposition with typical staining by Congo red and green birefringence when viewed with polarized light. The mucosa was intact.
DISCUSSION Amyloidosis is a disease of uncertain aetiology in which a complex protein-polysaccharide material accumulates in various tissues of the body. Osserman and Isobe (1972) classified it as a plasma cell dyscrasia because, at least in some cases, the amyloid fibrils are composed of complete or fragmented light chain immunoglobulins. Amyloidosis of the upper respiratory tract, especially the larynx, is not uncommon, but localization in the lower respiratory tract is rare (Domm er ul., 1965). To date, 30 cases have been described, but this is only the eighth case with bronchographic findings. Arrstmlusiari Rodiolo,cp. Vol. XX. N o . 3 . September. 1976
FIGURE +Right Bronchogranr-Note the lack of middle lobe filling and fusiform narrowing of the intermediate bronchus (arrows).
Lower respiratory tract amyloid is classified into three groups (Prowse, 1958): Group I-Parenchymal amyloid tumours without tracheobronchial involvement. Group 11-Localized tracheobronchial amyloidosis. Group Ill-Diffuse tracheobronchial amyloidosis. Diffuse tracheobronchial amyloidosis is three times more common in males than in females, and the cases are equally distributed in all decades from the third to the seventh. Symptoms other than hemoptysis, cough, and hoarseness usually result from secondary atelectasis or pneumonia. Respiratory function tests mav show a mild obstructive pattern, with evidence of proximal airway obstruction in helium How studies. 233
K. R.THOMSON, D. B. SPRING A N D R. E. HANSON
FIGURE Sn-Right
Bronchogram-Proximal stenosis of upper lobe bronchus.
Treatment is largely symptomatic. Some authors describe piecemeal removal of obstructing lesions (Kamberg et al., 1962), but fatal haemorrhage has been reported following this procedure (Komschnabel and Landis, 1962; Mainwaring et al., 1969). More recently, a single case of primary renal amyloidosis without myeloma was resolved with combination drug therapy over a period of four years (Cohen et al., 1975). This method may prove useful in tracheobronchial amyloidosis. Bronchoscopic findings are those of extensive submucosal nodules with or without bronchial obstruction. The appearance is said to be characteristic (Prowse, 1958; Antunes and Vierra de Luz, 1969; Attwood ef al., 1972), but it is of interest that no submucosal nodules were seen during the two bronchoscopic examinations of our case. Cooke et al. (1973) consider the bronchographic appearances virtually diagnostic. These consist of widespread irregular narrowing of the tracheobronchial tree with segmental bronchial occlusion. Similar appearances might be seen in infiltrating malignancy, aspiration of caustics, and tracheobronchiopathia osteochondroplastica (Fraser and Pare, 1970). The final diagnosis, of course, rests upon biopsy. Another feature of interest in this case was tomographic patency of the middle lobe segmental bronchi even though the middle lobe 234
5 b-Right Bronchogra,n-Proximal stenosis of FIGURE anterior basal bronchus.
bronchus was occluded. This apparent paradox may be explained by collateral air drift across an incomplete horizontal fissure (Fraser and Pare, 1970). SUMMARY Diffuse tracheobronchial amyloidosis is a rare condition with characteristic bronchoscopic and bronchographic findings. A case in which only the bronchographic findings were characteristic is described. ACKNOWLEDGEMENTS We are grateful to our colleagues for referring this patient for examination, to Mrs. A. Hoskins for typing the manuscript, and to Mr. W. Czerwinski for the illustrations. REFERENCES Antunes. M. I... and Vierra de Luz. J. M. (1969): “Primary Diffuse Tracheo-Bronchial Amyloidosis.” Thorax. 24, 307. Attwood, H. D.. Price. C. G., and Riddell, R. J. (!972): “Primary Diffuse Tracheo-Bronchial Amyloidosis.” Thorax, 27, 620. Cohen, H. J.. Lessen, L. S., Hallal. I., and Burkholder, P. (1975) : “Resolution of Primary Amyloidosis.” Ann. Intern. Med., 82, 466. Cooke. A. J.. Weinstein, M., and Powell, R. D. (1973): “Diffuse Amyloidosis of the Tracheobronchial Tree.” Radiology. 107, 303. Australasian Radiology. Vol. X X . No.3. September. 1976
AMYLOIWSIS CAUSING RIGHTMIDDLELOBEATELECTASIS I h m m . B. M.. V;tssallo, C. L.. and Adams, C. I.. ( 196s): “Amyloid Deposition Localized to the I.ower Respiratory Tract.” Am. 1. Med.,38, 151. Fraser, R. G.. and Pare. J. A. P. ( 1970) : “Diagnosis of Disease of the Chest.” W. B. Saunders, Philadelphia. ktmberg., S., 1-oitmun. B. S., and Holtz, S. (1962): “Amyloidosis of the Tracheobronchial Tree.” N. Eiigl. I . Med., 266, 587. Kornschnabel. E. F., and Landis, F. B. ( 1962): “Primary Amyloid Tumour.” Arch. Otoloryti,gol., 76, 233.
Mainwring, A. R., Williams, (3.. Knight. E. 0.W., and Bassett, H. F. M. (1969): ”Localized Arnyloidosis of the Lower Respiratory Tract.” Thornx, 24. 441. Osserman, E. F., and Isobe, T. (1972): “Plasma Cell Dyscrasins - General Considerations.” Huentutdos\ (Williams, W. J., Buetler. E., Ersler, A. J.. et ul., Eds.). McGraw-Hill. New York. Prowse, C. B. ( 1 9 5 8 ) : “Amyloidosis of the Lower Respiratory Tract.“ Thoruw, 13, 308.
NOTICE The
2nd Congress of the European Society of Cardio-VascularRadiology will be held on May 25-27, The topics of the Congress will be “Contrast Agents in Angiology-Modern Trends”, “Densitometry and Videodensitometry”, “Cardiac Tumours97and a‘Diagnostic Yield of Angiograph y”.
1977, in Uppsala, Sweden.
Preliminary Programme Wednesday, May 25 afternoon Registration Opening of the Congress evening Reception Thursday, May 26 morning Registration Scientific Sessions afternoon Scientific Sessions evening Music Entertainment
A second circular with forms for application for participation and accommodation will be distributed in December 1976.
Friday, May 27 morning afternoon
scientific Secretariat: Uno Erikson, M D Department of Diagnostic Radiology University Hospital S-750 14 UPPSALA, Sweden
evening
Scientific Sessions Scientific Sessions Closure of the Congress Banquet
A programme for accompanying family members will be arranged. A Post Congress Tour to Dalecarlia including a visit to Dr. Seldinger at Mora will be arranged.
Secretariat: 2nd Congress of the ESCR c / o RESO Congress Servicc S-105 24 STOCKHOLM Sweden
235
Amyloidosis Causing Right Middle Lobe Atelectasis K. R. THOMSON, M.B.,Ch.B., M.R.A.C.R., D. B. SPRING, M.D., and R. E. HANSON,M.D. Department of Diagnostic Radiology, Royal Victoria Hospital, Montreal, Quebec, Canada cytological studies of his sputum were also negative. Protein electrophoresis and immunoelectrophoresis were both normal. Chest roentgenograms (Figures 1 and 2) demonstrated middle lobe atelectasis and loss of the normal right hilar anatomy, best seen on the lateral projection. Lateral tomography (Figure 3) demonstrated severe loss of volume of the middle lobe with patency of thc segmental bronchi. Fiberoptic bronchoscopy revealed complete obstruction of the middle lobe bronchus and
INTRODUCTION The roentgenographic and bronchoscopic findings in a case of diffuse tracheobronchial amyloidosis presenting as middle lobe atelectasis are described. This case is unusual in that 1) the bronchoscopy was unremarkable except for right middle lobe bronchial occlusion, and 2) tomography showed patent segmental middle lobe bronchi in the presence of proximal occlusion of the middle lobe bronchus. CASEREPORT A 6 1-year-old retired male cabinetmaker
presented with a seven-month history of loose cough productive of blood-tinged sputum. He had been a heavy smoker until six years previously. Physical examination and initial laboratory studies were negative. Bacteriological and
FIGURE1-P.A.
232
Chest-Area of consolidation (R) middle lobe.
FIGURE 2-Lateral Chest-Atelectasis of right middle lobe and loss of normal ( R ) hilar anatomy.
A ustrulmian Rudiology, Vol. X X ,No. 3. September. 1976
AMYLOIDOSIS CAUSING RIGHTMIDDLELOBEATELECTASIS
FIGURE 3-Loterul Torriopurii-Note that middle lobe bronchi appear patent (arrows). The loss of volume of the middle lobe involves mostly the medial segment.
minimal deformity of the right upper lobe bronchus. The mucosa appeared normal. Brush biopsy performed at that time was normal. A right bronchogram (Figure 4) confirmed occlusion of the middle lobe bronchus. In addition, there was fusiform narrowing of the intermediate bronchus and proximal stenosis of several segmental bronchi in the right upper and lower lobes (Figure 5). Forceps biopsy on repeat bronchoscopy revealed diffuse submucosal amyloid deposition with typical staining by Congo red and green birefringence when viewed with polarized light. The mucosa was intact.
DISCUSSION Amyloidosis is a disease of uncertain aetiology in which a complex protein-polysaccharide material accumulates in various tissues of the body. Osserman and Isobe (1972) classified it as a plasma cell dyscrasia because, at least in some cases, the amyloid fibrils are composed of complete or fragmented light chain immunoglobulins. Amyloidosis of the upper respiratory tract, especially the larynx, is not uncommon, but localization in the lower respiratory tract is rare (Domm er ul., 1965). To date, 30 cases have been described, but this is only the eighth case with bronchographic findings. Arrstmlusiari Rodiolo,cp. Vol. XX. N o . 3 . September. 1976
FIGURE +Right Bronchogranr-Note the lack of middle lobe filling and fusiform narrowing of the intermediate bronchus (arrows).
Lower respiratory tract amyloid is classified into three groups (Prowse, 1958): Group I-Parenchymal amyloid tumours without tracheobronchial involvement. Group 11-Localized tracheobronchial amyloidosis. Group Ill-Diffuse tracheobronchial amyloidosis. Diffuse tracheobronchial amyloidosis is three times more common in males than in females, and the cases are equally distributed in all decades from the third to the seventh. Symptoms other than hemoptysis, cough, and hoarseness usually result from secondary atelectasis or pneumonia. Respiratory function tests mav show a mild obstructive pattern, with evidence of proximal airway obstruction in helium How studies. 233
K. R.THOMSON, D. B. SPRING A N D R. E. HANSON
FIGURE Sn-Right
Bronchogram-Proximal stenosis of upper lobe bronchus.
Treatment is largely symptomatic. Some authors describe piecemeal removal of obstructing lesions (Kamberg et al., 1962), but fatal haemorrhage has been reported following this procedure (Komschnabel and Landis, 1962; Mainwaring et al., 1969). More recently, a single case of primary renal amyloidosis without myeloma was resolved with combination drug therapy over a period of four years (Cohen et al., 1975). This method may prove useful in tracheobronchial amyloidosis. Bronchoscopic findings are those of extensive submucosal nodules with or without bronchial obstruction. The appearance is said to be characteristic (Prowse, 1958; Antunes and Vierra de Luz, 1969; Attwood ef al., 1972), but it is of interest that no submucosal nodules were seen during the two bronchoscopic examinations of our case. Cooke et al. (1973) consider the bronchographic appearances virtually diagnostic. These consist of widespread irregular narrowing of the tracheobronchial tree with segmental bronchial occlusion. Similar appearances might be seen in infiltrating malignancy, aspiration of caustics, and tracheobronchiopathia osteochondroplastica (Fraser and Pare, 1970). The final diagnosis, of course, rests upon biopsy. Another feature of interest in this case was tomographic patency of the middle lobe segmental bronchi even though the middle lobe 234
5 b-Right Bronchogra,n-Proximal stenosis of FIGURE anterior basal bronchus.
bronchus was occluded. This apparent paradox may be explained by collateral air drift across an incomplete horizontal fissure (Fraser and Pare, 1970). SUMMARY Diffuse tracheobronchial amyloidosis is a rare condition with characteristic bronchoscopic and bronchographic findings. A case in which only the bronchographic findings were characteristic is described. ACKNOWLEDGEMENTS We are grateful to our colleagues for referring this patient for examination, to Mrs. A. Hoskins for typing the manuscript, and to Mr. W. Czerwinski for the illustrations. REFERENCES Antunes. M. I... and Vierra de Luz. J. M. (1969): “Primary Diffuse Tracheo-Bronchial Amyloidosis.” Thorax. 24, 307. Attwood, H. D.. Price. C. G., and Riddell, R. J. (!972): “Primary Diffuse Tracheo-Bronchial Amyloidosis.” Thorax, 27, 620. Cohen, H. J.. Lessen, L. S., Hallal. I., and Burkholder, P. (1975) : “Resolution of Primary Amyloidosis.” Ann. Intern. Med., 82, 466. Cooke. A. J.. Weinstein, M., and Powell, R. D. (1973): “Diffuse Amyloidosis of the Tracheobronchial Tree.” Radiology. 107, 303. Australasian Radiology. Vol. X X . No.3. September. 1976
AMYLOIWSIS CAUSING RIGHTMIDDLELOBEATELECTASIS I h m m . B. M.. V;tssallo, C. L.. and Adams, C. I.. ( 196s): “Amyloid Deposition Localized to the I.ower Respiratory Tract.” Am. 1. Med.,38, 151. Fraser, R. G.. and Pare. J. A. P. ( 1970) : “Diagnosis of Disease of the Chest.” W. B. Saunders, Philadelphia. ktmberg., S., 1-oitmun. B. S., and Holtz, S. (1962): “Amyloidosis of the Tracheobronchial Tree.” N. Eiigl. I . Med., 266, 587. Kornschnabel. E. F., and Landis, F. B. ( 1962): “Primary Amyloid Tumour.” Arch. Otoloryti,gol., 76, 233.
Mainwring, A. R., Williams, (3.. Knight. E. 0.W., and Bassett, H. F. M. (1969): ”Localized Arnyloidosis of the Lower Respiratory Tract.” Thornx, 24. 441. Osserman, E. F., and Isobe, T. (1972): “Plasma Cell Dyscrasins - General Considerations.” Huentutdos\ (Williams, W. J., Buetler. E., Ersler, A. J.. et ul., Eds.). McGraw-Hill. New York. Prowse, C. B. ( 1 9 5 8 ) : “Amyloidosis of the Lower Respiratory Tract.“ Thoruw, 13, 308.
NOTICE The
2nd Congress of the European Society of Cardio-VascularRadiology will be held on May 25-27, The topics of the Congress will be “Contrast Agents in Angiology-Modern Trends”, “Densitometry and Videodensitometry”, “Cardiac Tumours97and a‘Diagnostic Yield of Angiograph y”.
1977, in Uppsala, Sweden.
Preliminary Programme Wednesday, May 25 afternoon Registration Opening of the Congress evening Reception Thursday, May 26 morning Registration Scientific Sessions afternoon Scientific Sessions evening Music Entertainment
A second circular with forms for application for participation and accommodation will be distributed in December 1976.
Friday, May 27 morning afternoon
scientific Secretariat: Uno Erikson, M D Department of Diagnostic Radiology University Hospital S-750 14 UPPSALA, Sweden
evening
Scientific Sessions Scientific Sessions Closure of the Congress Banquet
A programme for accompanying family members will be arranged. A Post Congress Tour to Dalecarlia including a visit to Dr. Seldinger at Mora will be arranged.
Secretariat: 2nd Congress of the ESCR c / o RESO Congress Servicc S-105 24 STOCKHOLM Sweden
235
