SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Cystic lung disease in Sjögren’s Syndrome (SS) is classically associated with Lymphocytic interstitial pneumonia (LIP). However, as our case illustrates, pulmonary amyloidosis is a rare entity, that can result into similar cystic and nodular lung disease in patients with SS.
CASE PRESENTATION: 59 y/o female with 10 pack year history of smoking, presented for evaluation of chronic worsening exertional dyspnea. Patient had long-standing history of SS with symptoms of dry mouth/eyes, arthralgia and fatigue; successfully treated with prednisone and hydroxychloroquine. CT chest showed bilateral diffuse cystic disease with several spiculated nodules, largest one measuring 5.5X5.2cm in RLL with eccentric calcification. Biopsy was consistent with pulmonary amyloidosis. Based on pathology and CT appearance, RLL mass was thought to be Amyloidoma.
DISCUSSION: SS is recognized as chronic lymphoproliferative disorder resulting into lymphocytic infiltration, monoclonal gammopathy, light chain amyloid deposition in various organs and lymphoma. Pulmonary manifestation in SS include ILD, bronchiectasis, bronchiolitis, pleuritis and rarely pulmonary vascular disease. Cystic lung disease in SS is usually associated with LIP & follicular bronchiolitis, however pulmonary amyloidosis has occasionally been implicated. Amyloid deposition is believed to be related to the benign lymphoproliferative process seen in the lung. Release of metalloproteinase from macrophages in lung causes elastolysis and cystic lung disease. Nodular amyloidosis or Amyloidoma radiographically mimics broncho-pulmonary carcinoma and is characterized by local immunoglobulin deposition. It is frequently associated with indolent neoplasm in the spectrum of MALT lymphoma. Natural progression and management is not entirely known due to rarity of cases reported.
CONCLUSIONS: Cystic and nodular lung lesions seen in patients with primary SS can represent pulmonary amyloidosis. The association of cysts in the lung with amyloid is of particular interest since pulmonary cysts in patients with SS are generally assumed to be due to LIP and not biopsied. There is an association between amyloidoma and underlying lymphoma which can be obscured by extensive amyloid deposition. Hence, strong clinical suspicion is required for timely diagnosis and treatment,
Reference #1: Baqir et al. Amyloid-associated cystic lung disease in primary Sjo ̈gren’;Respiratory medicine (2013)107;616-621
Reference #2: Rajgopala et al.Pulmonary amyloidosis in Sjogren's syndrome Respirology 2010 Jul;15(5):860-6
DISCLOSURE: The following authors have nothing to disclose: Kinjal Dave, Steven Nathan, A.W. Brown, C. King
No Product/Research Disclosure Information