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A Rare Case of Primary Amyloidosis in a Patient With Stage 2 Pulmonary Sarcoidosis FREE TO VIEW

Amitesh Agarwal*, MD; Raghav Govindrajan, MD
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Cleveland Clinic Florida, Weston, FL

Chest. 2012;142(4_MeetingAbstracts):1019A. doi:10.1378/chest.1390920
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SESSION TYPE: Miscellaneous Student/Resident Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Sracoidosis is classically associated with secondary amyloidosis (AA type) induced by the chronic indolent inflammatory process of sarcoid. There is sparse literature on the co-existence of primary amyloid (AL type) with sarcoidosis. We report a case of a 60 year old lady with history of pulmonary sarcoidosis who presented with dysphagia and was found to have AL type esophageal amyloidosis.

CASE PRESENTATION: A 60 year old woman with a 15 year history of stage 2 pulmonary sarcoidosis on low dose oral prednisone presented with progressive dysphagia, weight loss and failure to thrive. Her primary care physician treated her for gastro-esophageal reflux initially. But when the symptoms persisted she was referred to a gastroenterologist. An esophaogastrodudenoscopy was done which showed smooth velvety appearance in the lower third of the esophagus. Biospy with Hand E stain followed by congo red staining confirmed the presence of amyloid deposition the submucosal and muscularis layers of the eosophagus. Serum protein electrophoresis and urine protein electrophoresis showed M spike with immunofixation demonstrating IgG kappa light chain monoclonal proteins. Bone marrow biopsy did show a clonal plasma cell expansion. Patient expired 5 years from the initial diagnosis of amyloidosis due to restrictive cardiomyopathy.

DISCUSSION: In sarcoidosis the most frequently involved organs are the lungs and the hilar lymph nodes.The pathogenesis between AL amyloidosis and active sarcoidosis is not clear. A few prior reports suggest a coincidental association between cardiac sarcoidosis and AL amyloidosis. There is no evidence of direct relationship between active untreated sarcoidosis and development of AL amyloidosis , however the likely cause of amyloidosis in our patient appears to be active sarcoidosis.

CONCLUSIONS: Further studies are needed to establish if indeed there is a common triggering etio-pathogenic agent to our clinical observation of overlapping pulmonary sarcoidosis and primary amyloidosis. Given the latency of 15 years between the diagnosis if sarcoidosis and amyloidosis the question remains whether there was an ongoing exposure to the triggering agent.

1) Diana O Treaba :Sarcoidosis and immunoglobulin lambda II light-chain amyloidosis diagnosed after orthotopic heart transplantation: a case report and review of the literature Modern Pathology (2005) 18, 451-455.

2) Stather D Sarcoid:amyloid, and acute myocardial failure. Mod Pathol. 1998 Sep;11(9):901-4

DISCLOSURE: The following authors have nothing to disclose: Amitesh Agarwal, Raghav Govindrajan

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Cleveland Clinic Florida, Weston, FL




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