Abstract 14814: By Stanza or Story, the Structure of a Poem
A 63-year-old male with recent diagnosis of idiopathic pericarditis presented to the hospital with atrial fibrillation and rapid ventricular rates. He reported one week of palpitations and subacute lower extremity weakness. On exam he was hemodynamically stable with evidence of intra- and extravascular fluid overload. Lab analysis showed elevated creatinine, inflammatory markers, and serum protein gap. Chest radiograph showed bilateral pleural effusions and a focal sclerotic thoracic vertebra. TTE revealed mildly reduced biventricular function, elevated RVSP, and moderate pericardial effusion. The presence of pericardial and pleural effusions with elevated inflammatory markers suggest serositis, and a differential of malignant, autoimmune, and infectious etiologies were considered. Hepatosplenomegaly was noted on CT imaging. Blood, pleural, and pericardial cultures revealed no growth and autoimmune serologies were negative. Given these findings- the differential was narrowed to lymphoma, myeloma with or without amyloidosis, and paraneoplastic syndromes. Electrophoresis revealed presence of an M protein, fat pad biopsy was negative for amyloid fibrils. Bone marrow biopsy revealed an abnormal plasma cell population and biopsy of sclerotic vertebra showed no malignancy. Whole body FDG PET showed no focal uptake in the heart nor elsewhere. VEGF levels were significantly elevated. On the basis of the plasma cell dyscrasia, polyneuropathy, sclerotic bone lesion, elevated VEGF level, hepatosplenomegaly, and pericardial effusion the patient was diagnosed with POEMS syndrome- an extremely rare paraneoplastic syndrome with multisystemic end-organ effects. Cardiac manifestations are infrequent, but include pericardial effusion, myocardial edema and dysfunction, pulmonary hypertension, and volume overload. No specific cardiac therapy exists, but involvement may regress with chemotherapy for treatment of the underlying malignancy. This case demonstrates the importance of recognizing cardiac manifestations as the initial or cardinal presentation of underlying systemic disease, where accurate diagnosis may rely on identification of subtle non-cardiac symptoms, laboratory, and imaging findings as demonstrated here.