Chest
Volume 90, Issue 6, December 1986, Pages 827-831
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Clinical Investigations
Amyloidosis of the Lower Respiratory Tract: Clinical and Pathologic Features in a Series of 21 Patients

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Twenty one cases of amyloidosis of the lower respiratory tract were seen at a single center. In three patients, multifocal bronchial amyloid plaques led to stenosis and atelectasis, and in two, small pseudotumor masses were an incidental bronchoscopic finding. Two patients had nodular parenchymal amyloidosis, in one of whom the lesions were progressive and in the other static. Fifteen patients had diffuse parenchymal amyloidosis. Two of these had severe interstitial involvement and died in respiratory failure; eight had congestive cardiac failure, and parenchymal amyloidosis was a post-mortem finding; two had senile cardiorespiratory amyloidosis, also found at autopsy; and in three, the amyloidosis was associated with malignancy. The degree of respiratory embarrassment seemed to be related to the amount of amyloid in the gas diffusion zones, irrespective of the etiology of amyloidosis.

Section snippets

MATERIALS AND METHODS

We studied all patients with amyloidosis seen in bronchopulmonary specimens examined by our Pathology Department between 1970 and 1985 and for whom sufficient clinical details were available. Amyloidosis was diagnosed on seeing typical amorphous eosinophilic extracellular material with a green birefringence under the polarizing microscope after staining with Congo red. Sections were also pre-treated with potassium permanganate (except for cases 4,6,9,12,14). This abolishes the uptake of Congo

RESULTS

Our series included 21 patients (14 men and 7 women), with a mean age of 59.8 years (range 35–90). They were classified as follows: tracheobronchial amyloidosis: multifocal submucosal plaques, three; pseudotumor mass, two (one associated with nodular parenchymal amyloidosis). Parenchymal amyloidosis: nodular, two; diffuse, 15. Pretreatment with potassium permanganate did not abolish the uptake of Congo red in any of the sections tested.

DISCUSSION

The varied pattern of involvement of the lower respiratory tract by amyloidosis gives rise to several different clinical and radiologic presentations.3, 5, 6, 7 Our three patients with multifocal submucosal amyloid plaques had atelectasis. Bronchial biopsy led quickly to the diagnosis. In one of these patients, ossification associated with the amyloid deposits suggested a relationship between amyloidosis and tracheobronchopathia osteoplastica.3 Tracheobronchial amyloidosis is an indolent form

ACKNOWLEDGMENTS

We thank our colleagues who contributed to this study: Prs J. C. Bertrand, J. Normand, R. Tête, P. Touboul; Drs. H. Benier, P. Chapuy, M. Gauthier, A. Gressard, B. Saugier, M. Silie, L. Van Straaten, J. M. Vergnon, B. Veyre, M. Vincent and S. Peyrol. We also thank L. D. Gruer for reviewing the translation and M. C. Thévenet for secretarial assistance.

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    Citation Excerpt :

    Amyloidosis can be systemic or localized in one organ with little or no spread, with very different prognosis and treatment implications. It is estimated that about 50% of amyloidosis cases are localized in the respiratory system and are categorized in 3 forms: (1) tracheobronchial amyloidosis, (2) nodular parenchymal amyloidosis, and (3) diffuse parenchymal or alveolar septal amyloidosis [1-6]. The diagnosis of respiratory amyloidosis is challenging and often missed since the clinical symptoms, when present, are non-specific and extremely common.

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Manuscript received April 14; revision accepted July 1.

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