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  • Thorax (1975), 30, 178.

    Multiple nodular pulmonary amyloidosisA case report and comparison with

    diffuse alveolar-septal pulmonary amyloidosisSIONG-CHUAN LEE and HORTON A. JOHNSON

    Department of Pathology, Indiana University School of Medicine, 1100 W. Michigan Street,Indianapolis, Indiana 46202, USA

    Lee, S-C. and Johnson, H. A. (1975). Thorax, 30, 178-185. Multiple nodular pulmonaryamyloidosis. A case of multiple nodular pulmonary amyloidosis in a 54-year-oldCaucasian man is presented. Discrete symptomless radiodensities had developed in thispatient's lungs within a period of three years, leading to a suspicion of a neoplasticprocess. The amyloid nature of these nodules was demonstrated by biopsy. In this case,as in others previously reported, there was no evidence of systemic disease, and immuno-globulins were normal. Local factors probably play an important part in the patho-genesis of this disease.

    This entity is to be distinguished from the diffuse type of pulmonary amyloidosis,which has a far graver prognosis. Diffuse alveolar septal amyloidosis is usually associatedwith primary systemic amyloidosis or multiple myeloma and leads rapidly to respiratorydistress.

    Amyloidosis, either generalized or localized, canaffect the upper or lower respiratory tract.Amyloidosis of the larynx, trachea, and bronchiis well known and has been the subject of manyreports (Whitwell, 1953; Prowse, 1958; Heiner,1968; McGurk, 1968; Spencer, 1968; Attwood,Price, and Riddell, 1972). Amyloidosis of the lungparenchyma occurs in two different forms, thenodular type and the diffuse alveolar septal type.Although postmortem examinations frequentlyreveal small foci of amyloid in lung parenchymawhich are of no clinical significance, amyloidosiscan occasionally form single or multiple nodulesor a diffuse pulmonary infiltrate that will drawspecial attention and will require medical man-agement. Thus far, about 30 to 40 cases ofisolated multiple or solitary nodular pulmonaryamyloidosis (Whitwell, 1953; Prowse, 1958; Duke,1959; Condon, Pinkham, and Hames, 1964;Cotton and Jackson, 1964; Fors and Ryden, 1964;Firestone and Joison, 1966; Hayes and Bernhardt,1969; Zundel and Prior, 1971; Dyke et al., 1974)and less than 15 cases of diffuse alveolar septalamyloidosis (Larsen, 1930; Sappington, Davie,and Horneff, 1942; Bruno and Ober, 1968;Thomsen, 1968; Gonzalez-Cueto et al., 1970;Crosbie et al., 1972) have been reported in the

    literature. The following is a report of a case ofisolated multinodular pulmonary amyloidosiswhich was investigated at Indiana UniversityMedical Center following discovery by routinechest radiography. This case is reported not onlybecause of the rarity of the condition, but alsobecause of its striking difference from the diffusealveolar septal type of pulmonary amyloidosis inits clinical aspects, prognosis, and histopathologi-cal picture.

    CASE REPORT

    CLINICAL FINDINGS A 54-year-old Caucasian manwas admitted to Indiana University MedicalCenter because of a chest radiograph whichshowed multiple nodular lesions in both lungfields strongly suggestive of neoplasm (Figs 3 and4). The patient had been in good health exceptfor mild gout, hypertension, and chronic inter-mittent diarrhoea. Review of previous chest filmsshowed the nodules to have been present, al-though of smaller size, on 15 October 1971 (Fig.2), but entirely absent from a chest film takenon 3 October 1969 (Fig. 1). Physical examinationwas within normal limits, showing no evidenceof respiratory distress, hepatosplenomegaly, or

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  • Multiple nodular pulmonary amyloidosis 179

    FIG. 1. Chest radiograph in 1969 showing no evidence of nodular lesions.

    ..4. 5S #

    FIG. 2. Chest radiograph in 1971 showing multiple nodular lesions in both lung fields.

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  • S-C. Lee and H. A. Johnson

    FIG. 3. Chest radiograph in 1973 before exploratory thoracotomy showing increase in sizeand density of the nodular lesion in both lung fields.

    FIG. 4. Lateral view showing nodular lesions distri-buted in the anterior and posterior parts of the lungs.

    macroglossia. Diagnostic tests, includingcreatinine clearance, liver function tests, pul-monary function tests, intravenous pyelography,and liver and spleen scans, were also withinnormal limits. The bone marrow examinationshowed no evidence of multiple myeloma or otherneoplastic disease. No Bence-Jones protein waspresent in the urine. Serum electrophoresis andimmunodiffusion studies showed normal immuno-globulins with no evidence of a monoclonalgammopathy. An exploratory thoracotomy wascarried out, and multiple discrete nodules werefound in the subpleural areas. A representativewedge biopsy was taken.

    PATHOLOGICAL FINDINGS The surgical specimenmeasured 5 X 5 X 1-0 cm. The pleural surfacewas smooth but elevated by underlying nodules.There were several discrete, well-circumscribed,but unencapsulated nodules measuring 0-5 to1-0 cm in diameter immediately beneath thepleura. The cut surface of these nodules had atranslucent, homogeneous, gray, waxy appear-ance. The surrounding lung parenchyma wasslightly compressed. On examination by light

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  • Multiple nodular pulmonary amyloidosis

    microscopy the nodules were well demarcatedfrom the lung parenchyma (Fig. 5). Each masswas composed of amorphous, homogenous,eosinophilic material, which stained red to pinkwith Congo Red and metachromatically withCrystal Violet. Within the nodules foci of calcifi-cation and dystrophic bone formation were seen(Fig. 6). At the periphery of each nodule therewere variable numbers of lymphocytes, plasmacells, histiocytes, and a few multinucleated giantcells (Fig. 7).

    HOSPITAL COURSE The postoperative course wasuneventful, and the patient was discharged ingood condition without specific treatment. He hasremained free of symptoms since that time.

    DISCUSSION

    Although isolated nodular pulmonary amyloidosisis a very rare type of localized amyloidosis, thisentity was reported as early as 1877 by Lesser.Since then some 30 to 40 cases have appeared inthe literature. Most of these cases have beentabulated and reviewed by Condon et al. (1964),Firestone and Joison (1966), Weiss (1960), andFors and Ryden (1964), and so a further reviewof these cases is not necessary here. In brief, thedisease tends to affect patients in an older age

    group. The average age of the 30 well-documentedcases was 68 years. Two-thirds of those patientswere over 60, one-third were older than 70, andthe overall age range was 38 to 95. No sexpredilection has been noted. The majority ofpatients were free of symptoms, and pulmonaryfunction tests have shown no evidence of impair-ment of gas transfer. These patients were gener-ally discovered by routine chest radiographswhich were interpreted as either metastatic neo-plasm or miliary tuberculosis. The histopathologyhas been presented in detail by Weiss (1960). Themost prominent features are the presence ofamyloid, calcification, dystrophic bone formationwith plasma cells, histiocytes, lymphocytes, andmultinucleated giant cells. The intimate relation-ship between amyloid and plasma cells is wellknown, and it is thought that plasma cells play arole in the production and perhaps also in thedegradation of immunoglobulins (Glenner, 1973).The role of the multinucleated giant cells, whichare also present in the diffuse alveolar septal typeof amyloidosis and in amyloid tumours of thelarynx, is not clear, but they may represent ahost reaction to abnormal protein. The commonfinding of calcification and ossification of thesenodules indicates their long duration. In the caseson which necropsies have been carried out, nonehas shown amyloidosis of other visceral organs.

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    FIG. 5. Low magnification showing discrete well-demarcated amyloid nodule(H and E X10).

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  • S-C. Lee and H. A. Johnson

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    FIG. 7. Magnification showing close relationship of plasma cells and giant cell to theamyloid (H and E X400).

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  • Multiple nodular pulmonary amyloidosis

    In the present case, intensive investigation showedno evidence of extrapulmonary amyloidosis.The isolated nodular type of amyloidosis is an

    entity quite distinct from the diffuse alveolarseptal type of pulmonary amyloidosis. The latteris usually associated with overt multiple myeloma(Crosbie et al., 1972) or primary amyloidosis(Larsen, 1930; Sappington et al., 1942; Brunoand Ober, 1968; Th