Case 2--Diffuse Nodular Amyloidosis

Clinical summary: A 55-year-old man, who presented in 1989 with a 1-year history of dyspnea on exertion underwent an open lung biopsy, which showed diffuse, nodular amyloidosis, in 12/89. Serum protein electrophoresis showed an IgG lambda monoclonal gammopathy. The bone marrow showed no evidence of multiple myeloma. Despite chemotherapy (vincristine, melphalan, cyclophosphamide, and prednisone), the pulmonary disease progressed. By 9/92 his FVC was 38% and TLC was 32% of predicted. As part of evaluation for lung transplantation, biopsies of liver, colon, bone marrow, and small bowel were performed and showed no amyloid deposition. Also, a transthoracic echocardiogram did not suggest cardiac amyloidosis. Transplantation of the left lung was performed in 10/94. Large hilar lymph nodes were noted at surgery. Postoperatively, the paraprotein and IgG levels declined with immunosuppression that included cyclosporine, azathioprine, and prednisone [1,2].

This frontal chest x-ray taken prior to transplantation shows small lung volumes and multiple, confluent, small nodules predominantly in the mid and lower lung zones and lung periphery. Note the pleural thickening adjacent to the most heavily involved lung bilaterally.

A HRCT (12/95, post-transplantation) shows in the native right lung a combination of parenchymal lines, interlobular septal thickening, and small nodules, up to several centimeters in diameter. Confluent subpleural consolidation is also present. These changes had progressed since the first HRCT in 1989, whereas the chest radiograph showed little change.

A mediastinal window shows calcification in the opacities. What is the differential diagnosis of these changes? Answer

References:

1. Graham C, Stern E, Finkbeiner W, Webb W. High-resolution CT appearance of diffuse alveolar septal amyloidosis. AJR 1992; 158:265-267.

2. Ware L, Keith F, Gordon R, Ries C, Seitz R, Gold W, Golden J. Lung transplantation for pulmonary amyloidosis: a case report. J Heart Lung Transplant 1998; 17:1129-1132.

Clinical summary Case 2 continued

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Answer: The differential diagnosis includes sarcoidosis, lymphangitic metastatic tumor, silicosis and other pneumoconioses (eg, coal workers' pneumoconiosis), hypersensitivity pneumonia, and lymphoma. However, only the pneumoconioses are likely to calcify [1].

References:

1. Graham C, Stern E, Finkbeiner W, Webb W. High-resolution CT appearance of diffuse alveolar septal amyloidosis. AJR 1992; 158:265-267.

2. Ware L, Keith F, Gordon R, Ries C, Seitz R, Gold W, Golden J. Lung transplantation for pulmonary amyloidosis: a case report. J Heart Lung Transplant 1998; 17:1129-1132.

Clinical summary Case 2 continued

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