Case Presentation: A 63-year-old Caucasian female with a medical history of type 2 DM, HTN, and cholecystectomy presented with a 3-days history of abdominal pain, nausea, and vomiting. She has no history of alcohol intake and has a pertinent family history of colon cancer. Physical exam and vital signs were normal except for epigastric tenderness.Labs were significant for Cr 8.9 mg/dl, lipase 1,723 U/L (11- 82), Calcium 11.9 mg/dl (8.4 -10.2), Alb 3.9 mg/dl, Triglyceride 121 mg/dl and normal IgG4. CT abdomen and pelvis showed inflammation in the tail of the pancreas. Investigations for hypercalcemia showed normal bone survey, SPEP, PTH, PTHrP, and vitamin D. After standard management with IV fluids, Cr improved to 1.5 mg/dl and Ca to 8.6 mg/dl. EUS and MRCP did not provide an etiology of acute pancreatitis. Two months later, she presented again with abdominal pain, constipation, and weight loss. Labs showed Cr 2.7 mg/dl, Ca 13.5 mg/dl, ionized Ca 7.4 mg/dl (4.6- 5.3), 1,25-OH Vit D (Calcitriol) 101 pg/mL (20 -79) and high ACE level 104 U/L (9 – 67). Hypercalcemia resolved after IV fluids and Calcitonin. CT chest revealed non-specific mediastinal lymphadenopathy. Transbronchial needle aspiration was nondiagnostic. PET scan showed hypermetabolic lymphadenopathy above and below the diaphragm. Excisional biopsies through Mediastinoscopy showed multiple granulomas with giant cells and focal hyalinization without necrosis. Fungal and mycobacterial stains were negative. Immunophenotyping revealed no evidence of abnormal lymphocytes or myeloid blasts. The diagnosis of Sarcoidosis was made based on the above findings. Ca level was slowly increasing again but responded well to high dose Prednisone. Hydroxychloroquine 200 mg daily was added to facilitate prednisone tapering.
Discussion: Sarcoidosis is a multisystem inflammatory disorder of unknown etiology that is characterized pathologically by the presence of noncaseating granulomas. Approximately 90 percent of patients with Sarcoidosis have lung involvement and are often associated with hilar and mediastinal lymphadenopathy. The diagnosis of Sarcoidosis, in this case, was established based on the criteria of having compatible clinical and radiographic findings along with histopathologic detection of noncaseating granulomas and after the exclusion of other differential diagnoses. Only a few cases of Sarcoidosis presenting as pancreatitis have been reported. Hypercalcemia occurs in about 10% of the patients with Sarcoidosis due to increased extrarenal and PTH-independent production of Calcitriol by activated macrophages in pulmonary alveoli and granulomatous inflammation. Additionally, hypercalcemia-induced acute pancreatitis is not common; its reported prevalence in primary hyperparathyroidism is 1.5 – 8%. Besides standard management of acute pancreatitis, hypercalcemia-induced pancreatitis in sarcoid patients would benefit from corticosteroids that usually cause a prompt reversal of the metabolic defect. Hydroxychloroquine can be used if the patient fails to respond or develops serious side effects from corticosteroids.
Conclusions: Sarcoidosis is a diagnosis of exclusion and requires a constellation of clinical, radiographic, and histopathological manifestations. Although hypercalcemia-induced pancreatitis is a rare presentation of Sarcoidosis, it is important to consider it in the differential diagnosis due to its management implications since corticosteroids are not usually recommended but are very effective in this case.