Disorders of the Mediastinum: Student/Resident Case Report Poster - Disorders of the Mediastinum |

Encephalopathy and Pancreatitis: Unusual Presentation of Pulmonary Sarcoidosis FREE TO VIEW

Agazi Gebreselassie, MD; Rahel Dagne, MD; Babak Shokrani, MD; Alem Mehari, MD
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Howard University Hospital, Washington, DC

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):553A. doi:10.1016/j.chest.2016.08.641
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SESSION TITLE: Student/Resident Case Report Poster - Disorders of the Mediastinum

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: The usual presentation of sarcoidosis is hilar adenopathy, pulmonary reticular opacities, skin, joint or eye lesions. There are only few case reports of sarcoidosis presenting with severe hypercalcemia and pancreatitis. We describe a case of sarcoidosis presenting for the first time with metabolic encephalopathy and severe pancreatitis.

CASE PRESENTATION: A 53-year-old female with past medical history of hypertension, type 2 diabetes mellitus and dyslipidemia presented with a 3-day history of generalized body weakness and 1-day history of vomiting, diffuse abdominal pain and altered metal status (AMS). Investigations revealed serum creatinine 3.7 mg/dL, calcium 16.7 µg/dL, amylase 678 U/L, lipase 912 U/L. Intact PTH levels of 23 pg/mL (normal), PTH related protein 49 pg/mL (elevated), Vitamin D 1,25 89 mg/dL (elevated), serum ACE level 175 U/Liter (elevated). CT chest showed diffuse mediastinal/subcarinal/hilar adenopathy. CT abdomen revealed enlarged pancreatic head. The patient was admitted with the impression of acute metabolic encephalopathy, severe pancreatitis and acute kidney injury all ascribed to severe hypercalcemia. She was started on aggressive intra venous fluid hydration and intravenous calcitonin after which the AMS resolved and hypercalcemia improved. Further work up for hypercalcemia with bronchoscopy guided FNA cytology from hilar lymphnodes revealed non caseating granuloma, suggestive of sarcoidosis. The patient was started on prednisone 20 mg po daily and serum calcium levels normalized.

DISCUSSION: Clinical manifestations of sarcoidosis tend to vary with age, sex and ethnicity (1). Tuberculosis and sarcoidosis are the most common granulomatous diseases causing hypercalcemia. This patient most likely presented with newly diagnosed sarcoidosis. Biopsy of hilar lymph nodes revealed noncaseating granulomatous lesion on cytology. The elevated 1,25 vitamin D3 levels and ACE inhibitor levels also support the diagnosis of sarcoidosis.The central nervous system manifestation and the pancreatitis are most likely the consequences of the severe hypercalcemia as imaging studies did not reveal lesions in the brain and pancreas.The hypercalcemia in sarcoidosis is as the result of the increased production of calcitriol (Vitamin D 1,25) by macrophages in granuloma. PTHrP is also involved in causing hypercalcemia in sarcoidosis as seen in our patient (2).

CONCLUSIONS: Sarcoidosis might rarely present with severe hypercalcemia resulting in AMS, acute kidney failure and pancreatitis. It is always prudent to consider the possibility of sarcoidosis in a patient presenting with hypercalcemia. Bisphosphonates or calcitonin are used to acutely treat hypercalcemia. Long term management is steroid.

Reference #1: 1. Baughman RP, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001; 164:1885.

Reference #2: 2. Zeimer HJ et al. Parathyroid-hormone-related protein in sarcoidosis. Am J Pathol 1998; 152:17.

DISCLOSURE: The following authors have nothing to disclose: Agazi Gebreselassie, Rahel Dagne, Babak Shokrani, Alem Mehari

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