Pulmonary Manifestations of Amyloidosis

11/12/01

Question: What is the incidence of pulmonary presentations of amyloidosis?

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Unique Identifier: 21165534 / PubMed Identifier: 11264765

Authors: Howard ME. Ireton J. Daniels F. Langton D. Manolitsas ND. Fogarty P. McDonald CF.

Institution: Department of Respiratory Medicine, Austin Repatriation Medical Centre, Heidelberg, Victoria, Australia. m.howard@pgrad.unimelb.edu.au

Abstract: Respiratory tract involvement with amyloid is rare. We report eight cases of lower respiratory tract amyloidosis including a case of isolated pulmonary interstitial amyloidosis treated with chemotherapy, two cases of recurrent endobronchial amyloid with airway obstruction successfully treated with laser therapy and three cases of localized nodular pulmonary amyloidosis. The subjects with endobronchial and nodular amyloid demonstrated good long-term survival, while those with systemic or interstitial pulmonary amyloid had progressive disease and poor survival. Circulating monoclonal immunoglobulins were identified in five of the eight cases as the likely cause of the amyloid.

Link Directly to Fulltext article in Ovid

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[Link Directly to Fulltext Article in OVID]

Unique Identifier: 96151944 / PubMed Identifier: 8554249

Authors: Utz JP. Swensen SJ. Gertz MA.

Institution: Mayo Medical School, Rochester, Minnesota, USA.

Abstract: OBJECTIVE: To define the prognosis for and radiographic presentation of patients with pulmonary amyloidosis. DESIGN: Retrospective review of the Mayo Clinic experience with biopsy-proven pulmonary amyloidosis from 1980 to 1993. SETTING: Tertiary care center. PATIENTS: Patients with pulmonary biopsy specimens showing amyloid deposition. MEASUREMENTS: Medical records were reviewed, and pertinent information was recorded, including demographic data, type of pulmonary biopsy, results of biopsies of nonpulmonary sites and of immunoelectrophoresis, and other clinical, radiographic, and laboratory information necessary for distinguishing localized pulmonary amyloidosis, primary systemic amyloidosis, secondary amyloidosis, and familial amyloidosis. RESULTS: 35 of 55 patients with pulmonary amyloidosis had primary systemic amyloidosis that presented radiographically as an interstitial or reticulonodular pattern with or without pleural effusion. The median survival after diagnosis was 16 months. Nodular pulmonary "amyloidomas" (nodular amyloid lesions) were not associated with systemic disease and were associated with a benign prognosis. Three of 4 patients with localized tracheobronchial amyloidosis required Nd:YAG (neodymium:yttrium-aluminum-garnet) laser therapy for obstructive symptoms. "Senile" amyloid deposition was an incidental finding in some patients at autopsy. CONCLUSIONS: Localized amyloidomas are characterized by a benign course and are not associated with systemic amyloidosis. Despite its localized nature, tracheobronchial amyloid deposition may be asymptomatic or may result in significant morbidity due to obstructive phenomena. Pulmonary amyloidosis associated with primary systemic amyloidosis generally presents as a diffuse interstitial pattern with or without pleural effusion. Complete survival data indicate that long-term outcome is poor after diagnosis. We describe the largest series of patients diagnosed by bronchoscopic lung biopsy. Despite reports to the contrary, we have found bronchoscopic lung biopsy to be a safe and effective diagnostic technique.