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Obstructive Lung Diseases |

Sjögren's Related Pulmonary Amyloidosis

Matthew Hammar, DO; Khalid Malik, MD
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Allegheny General Hospital, Pittsburgh, PA


Chest. 2013;144(4_MeetingAbstracts):677A. doi:10.1378/chest.1704712
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Abstract

SESSION TITLE: Interstitial Lung Disease Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Amyloidosis (AL) results from extracellular accumulation of monoclonal immunoglobulin light chain proteins. Amyloid deposition can be local or systemic. Pulmonary amyloid is usually found in the setting of systemic AL. Systemic disease typically portends unfavorable outcomes; consequently, the lung involvement of systemic AL is clinically insignificant in most patients as cardiac symptoms dominate. Isolated pulmonary AL confined to the lung is uncommon. We report a case of isolated nodular parenchymal pulmonary amyloidosis secondary to Sjögren's symdrome (SS).

CASE PRESENTATION: A 61 year-old female with SS obtained a chest radiograph for routine preoperative clearance. The film revealed pulmonary nodules (Fig. 1). Subsequent chest CT confirmed multiple small nodular opacities in the bilateral mid and lower lungs (Fig. 2). Despite being asymptomatic, there was concern for malignancy, vasculitis or organizing pneumonia. Therefore a CT guided biopsy was done. Pathology confirmed AL. Serum protein electrophoresis, urine protein electrophoresis and bone marrow biopsy failed to demonstrate any lymphoproliferative disorder or systemic amyloidosis. An immunosuppressive regimen was recommended for treatment of SS complicated by isolated pulmonary AL. The patient declined treatment and was lost to followup. The patient returned 18 months later after a chest CT demonstrated interval progression. She remained asymptomatic and resistant to immunosuppressive therapy. The patient sought a second opinion at Boston University’s amyloid clinic. Aggressive treatment with immunosuppression was recommended. Patient was initiated on treatment and the pulmonary nodules remain radiographically stable.

DISCUSSION: Amyloid induced organ dysfunction occurs secondary to monoclonal plasma cell immunoglobulin light chain protein deposition. AL can be categorized by etiology, such as primary or secondary, or by location, such as systemic or local. In the lung, amyloid has histopathologic forms based on location and distribution: tracheobronchial with localized and diffuse distribution, parenchymal with nodular and diffuse distribution, and senile with a diffuse scattered distribution. Localized pulmonary AL is rare and often discovered as an incidental lower lobe finding. The natural history for SS associated lung disease is not well established. Mainstay of treatment is SS control.

CONCLUSIONS: Review of the literature reveals a paucity of data mostly in the form of case reports. As such, there is no recommended treatment strategy for pulmonary nodular amyloidosis occurring in the absence of lymphoma.

Reference #1: Gertz, M., Greipp, P., Philip, R. Clinical aspects of pulmonary amyloidosis. CHEST 1986; 190:790-1.

Reference #2: Podbielski, F., Nelson, D., Pearsall, G., Marquez, G., Connolly, M. Nodular pulmonary amyloidosis. J Thorac Cardiovasc Surg 1997;114:289-91.

Reference #3: Utz, J., Swensen, S., Gertz, M. Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993. Ann Intern Med 1996;124:407-13.

DISCLOSURE: The following authors have nothing to disclose: Matthew Hammar, Khalid Malik

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