Amyloidosis with pleural involvement D.R. Graham, D. Ahmad* CASE

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Amyloidosis with pleural involvement D.R. Graham, D. Ahmad* CASE
Eur RespirJ
1988, 1, 571- 572
Amyloidosis with pleural involvement
D.R. Graham, D. Ahmad*
Amyloidosis with pleural involvement. D .R. Graham, D. Ahmad.
ABSTRACT: Although amyloidosis of the respiratory tract is well
recognized, pleural involvement Is very rare with only tw o cases being
reported In the past. We report a case of primary amyloidosis with pleural
effusion and suggest that pleural Involvement and pleural effusion be
added to the classification of pulmonary amyloidosis, and that amyloidosis
be added to the list of causes of a pleural effusion.
Eur Respir J ., 1988, 1. 571-572.
*Sir Adam Beck Chest Disease Unit, University
Hospital, London, Ontario, Canada.
Correspondence: D. Ahmad, Sir Adam Beck Chest
Disease Unit, University Hospital, 339 Win dermere Road, London, Ontario, Canada N6A
Keywords: Amyloidosis; pleural effusion; pleural
infiltration; thoracoscopy.
Received: June 6, 1987; accepted after revision:
February 15, 1988
Amyloidosis of the lung is rare though its occurrence is well recognized [1, 2]. Involvement of the
lower respiratory tract occurs in both the tracheobronchial tree and lung parenchyma. Pleural involvement
is very rare, there has only been a single case report
of pleural effusion [3] and pneumopleural amyloid
'tumour' [4].
We report the case of a patient with primary amyloidosis who developed a pleural effusion and had
amyloid deposition identified in both lung and pleura
at thoracoscopy.
Case Report
A 52-yr old man presented to a cardiologist with a
nine month history of dull central chest pain which
was associated with exercise and was relieved by rest.
The pain often radiated to his throat, was worse after
meals and was accompanied by breathlessness. There
was no relief with sublingual nitrate preparations. He
had undergone a cholecystectomy and vagotomy ten
years earlier and two years before presentation he had
severe post-operative dyspnoea following reduction of
a wrist fracture. Although no definite diagnosis was
made, he was thought to have had a pulmonary embolus
or aspiration pneumonia. There was no family history
of heart disease nor was there any relevant occupational history. He was a life long non-smoker, drank
no alcohol and his only medication was cholestyramine for slightly elevated blood cholesterol levels.
Physical examination at presentation was unremarkable apart from an intermittent 4th heart sound.
The chest radiograph was normal. Electrocardio&rram
showed T-wave inversion in the lateral chest leads.
Exercise ECG using the Bruce protocol was negative.
Cardiac catheterization showed normal coronary arter-
ies, the right coronary artery being dominant. There
was some diffuse narrowing following ergometrine
which was associated with chest pain and S-T segment
It was concluded that there was no evidence of
coronary artery disease and there was functional
overlay to his symptoms. Over the next twelve months
he was treated with nitrates, beta-blockers and calcium
slow-channel antagonists, with no effect. He was referred to the pain relief clinic, and acupuncture was
also unsuccessful.
One year after presentation he was admitted with
severe breathlessness on exertion. He was found to
have a tachycardia of 110 b·min· ', an elevated jugular
venous pressure, bilateral ankle oedema, a 4th heart
sound and signs consistent with a pleural effusion at
the right base. Chest radiography showed cardiomegaly
and confirmed the pleural effusion, there was no evidence of parenchyma! lung disease. Echocardiogram
showed both ventricles to be hypokinetic. Cardiac catheterization was repeated and again showed normal
coronary arteries with global hypokinesis. Cardiac biopsy was performed (fig. 1) and showed obliteration of
interstitial and myocardial fibres by an eosinophilic
amorphous material, which was orange with Congo red
and apple green under polarization. There was also
amyloid infiltration of vessel walls. Electron microscopy was performed and confirmed the presence of
The pleural fluid was tapped and biochemically was
an exudate. The effusion reaccumulated two days later,
by which time the cardiac failure had been resolved
following diuretic treatment. Because the fluid was an
exudate and thought to be due to an inflammatory
process rather than cardiac failure, thoracoscopy was
performed. The procedure was carried out using a Storz
thoracoscope under local anaesthesia. Following induc-
We suggest that pleural involvement be added to
the classification of amyloidosis of the lower respiratory tract and that amyloidosis be added to the list
of rare causes of a pleural effusion. Furthermore, the
thoracoscope should be considered in making the diagnosis in cases of unusual infiltrative disease which
may involve the lung and pleura. One must be aware,
however, of the possibility of bleeding tendencies which
may occur in amyloidosis. These in turn have been
ascribed to amyloid involvement of the blood vessels
and in a few cases a deficiency of factor X [9, 10].
Acknowledgement: We thank Mrs. E. McGowan for
typing this manuscript.
Fig. I. -Pleural biopsy. Internal scale bar equals 10 j.t. Congo Red Stain
with polari7-ation, showing amyloid material in the pleural interstitium.
tion of pneumothorax, inspection revealed the lungs to
have oedema of the interlobular septa, the pleural surfaces were hyperaemic and the chest cavity contained
500 ml of a brown turbid fluid. Biopsies were taken of
the pleura and lung and both had been infiltrated by
Amyloidosis affecting the lung was first described
in a case of secondary amyloidosis by VIRCHow in
1857 [5]. Amyloid localized to the lower respiratory
tract was reported by LEssER in 1877 [6]. Since that lime
the occurrence of amyloid in the lung has been well
documented, indeed it has been suggested that in generalized primary amyloidosis, particularly when there is
cardiac involvement, infiltration of the lung parenchyma
is common [7, 8].
Most authorities agree that amyloidosis, when it
affects the lung, can be classified into two main types;
the tracheobronchial type which can be multifocal submucosal plaques or a tumour-like mass, and the parenchyma} type in which the deposition may be nodular,
either multiple or solitary, or may be that of a diffuse
alveolar septal infiltration [2]. A review of the literature shows that pleural involvement is very rare. Pleural
effusion with pleural amyloidosis was the subject of
a case discussion at the Massachusetts General Hospital [3] and a pneumonopleural tumour was described
in 1961 [4].
1. Celli BR, Rubinow A, Cohen AS, Brody JS. - Patterns of
pulmonary involvement in systemic amyloidosis. Chest, 1978,
2. Thompson PJ, Citron KM. - Amyloid and the lower
respiratory 1ract. Thorax, 1983, 38, 8~87.
3. Case reports of the MGH. Case 48-1977. N Engl J Med,
1977, 297, 1221.
4. Lundin P, Simonsson B, Winberg T. - Pneumonopleural
amyloid tumour. Acla Radio, 1961, 155, 139-144.
5. Virchow R. - Neue beobachtungen Uber amyloide degeneration. Virchow'sArch(PaJhAnat), 1857,11,188.
6. Lesser A. - Ein fall von enchondroma osteoides mix rum
der lunge mit partieller amyloid entortung. V irehow's Arch
(Palhol Anat), 1877, 69, 404-408.
7. Smith RRL, Hutchins GM, Moore GW, Humphrey RL.Type and distribution of pulmonary parenchyma! and vascular
amyloid. Correlation with cardiac amyloidosis. Am J Med, 1979,
66,96- 105.
8. Kline LR, Disc CA, Ferro TJ, Hansen Flaschen JH. Diagnosis of pulmonary amyloidosis by transbronchial biopsy.
Am Rev Respir Dis, 1985, 132, 191-194.
9. Yood RA, Skinner M, Rubinow A, Talarico L, Cohen AS. Bleeding manifestations in lOO patients with amyloidosis. JAm
MedAssoc, 1983,10, 1322- 1324.
10. Greipp PR, Kyle RA, Bowie EJW.- Factor X deficiency in
amyloidosis, a critical review. Am J Hematol, 1981, 11,
RESUME: Quoique l'amyloi'dose du tractus respiratoire soiL
bien reconnue, l'atteinte pleuralc est tres rare, avec seulement
deux cas rapportes dans la litterarure. Nous presentons un cas
d'amyloldose primaire avec epanchement pleural et nous
suggerons que l'atteinte pleurale et l'epanchement pleural
devraient etre ajoutes a la classification de l'amyloldose
pul.monaire, et que l'amyloldose devrait etre jointe a la lisle
des causes d'epanchement pleural.
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