Sarcoidosis Manifested as Hypercalcemic Pancreatitis.

ABSTRACT: Sarcoidosis is a multisystem disease of unknown etiology and with protean manifestations. Pancreatic involvement is unusual, with only a few case reports in the literature. Granulomatous infiltration is the usual cause. This case report describes an unusual presentation of sarcoidosis as hypercalcemic pancreatitis.

HYPERCALCEMIA is a well-established cause of acute pancreatitis. In most reported cases, hypercalcemia is due to either hyperparathyroidism or malignancy. This is the first case report clearly describing hypercalcemia-induced pancreatitis as the initial presentation of sarcoidosis.

CASE REPORT

A 49-year-old African American woman came to our institution because of abdominal pain, nausea, and vomiting. She had a medical history of type II diabetes mellitus, hypertension, and bronchial asthma. Her medications included metformin, enalapril, and salbutamol inhaler. She had had a cholecystectomy 18 years earlier for gallstones. She was a nonsmoker and denied use of alcohol.

Physical examination showed an obese female in significant distress due to abdominal pain. Her vital signs, with the exception of resting tachycardia, were stable. Multiple non-tender, firm, freely mobile lymph nodes were present in the neck, the largest being 2 x 2 cm. Chest examination showed decreased air entry bilaterally, with inspiratory and expiratory wheezing. The abdomen was tender in the epigastric and periumbilical regions. There were no peritoneal signs. The remainder of the physical examination was within normal limits. The serum amylase value was 810 IU/L (normal, 16 to 108 IU/L), serum lipase value 642 IU/L (normal, 23 to 300 IU/L), and serum calcium value 11.6 mg/dL (normal, 8.5 to 10.4 mg/dL). Other routine laboratory values, including serum triglycerides, were within normal range. Contrastenhanced computed tomography (CT) of the abdomen confirmed non-necrotizing pancreatitis. No pancreatic calcification was detected. The patient was admitted with a diagnosis of hypercalcemia-induced pancreatitis and treated with intravenous hydration and narcotic analgesics.

Further investigations of the hypercalcemia showed a serum parathyroid hormone (PTH) level (intact) of 4 ng/L (normal, 12 to 82 ng/L), and an elevated serum angiotensin converting enzyme (ACE) level of 375 IU/L (normal, 8 to 52 IU/L). Serum electrophoresis revealed polyclonal gammaglobulinemia. Cervical lymph node biopsy showed multiple noncaseating granulomas. A highresolution CT scan of the chest showed pulmonary fibrosis, with no hilar adenopathy. Pulmonary function tests were consistent with moderately severe interstitial and obstructive disease. Flexible bronchoscopy showed a nodular, erythematous airway mucosa. Biopsy of these lesions revealed noncaseating granulomas. Stains of biopsy specimens were negative for fungi and acid-fast bacilli. A tuberculin skin test was nonreactive, and controls with candida and mumps antigen were positive. On the basis of histopathologic findings and elevated ACE levels and by excluding other granulomatous diseases, a diagnosis of sarcoidosis was made. Treatment was star ted with oral steroids and outpatient medications. Abdominal pain resolved, and respiratory symptoms significantly improved. Two months later, the patient remained symptom free, with serum calcium and ACE levels back to normal.

DISCUSSION

The diagnosis of acute pancreatitis in this patient was established on the basis of symptoms, elevated amylase and lipase levels, and the CT demonstration of pancreatic inflammation. The patient denied any alcohol use, and a cholecystectomy had been done 18 years earlier. Triglyceride levels were within normal limits. Although enalapril has been associated with pancreatitis, this patient had been taking it for 5 years without complications. With the exception of hypercalcemia, she had no other precipitating factor for pancreatitis. The presence of cervical lymphadenopathy along with a low level of serum PTH led to the initial suspicion of malignant hypercalcemia. However, the histopathologic presence of noncaseating granulomas, exclusion of other granulomatous diseases, and the presence of elevated ACE levels established the diagnosis of sarcoidosis.

Chronic pancreatitis as a complication of sarcoidosis has been reported in the literature. [1,2] Infiltration of the pancreas by noncaseating granulomas is the usual cause. Cases of acute idiopathic pancreatitis in patients previously found to have sarcoidosis have also been reported. [3] In general, these patients had normal serum calcium levels and responded dramatically to steroids. They probably also had granulomatous infiltration of the pancreas. Hypercalcemia-induced pancreatitis has been reported in patients with sarcoidosis after sun exposure.[4,5] However, some of these patients had additional risk factors for pancreatitis in the form of alcohol abuse. Also, sarcoidosis had been previously diagnosed in these patients before development of abdominal symptoms. We were able to find only one reported case in which acute hypercalcemic pancreatitis was believed to be the initial manifestation of sarcoidosis.[4] However, that report did not mention whether other causes of pancreatitis and noncaseating gra nulomas were excluded.

In summary, we have reported what is probably the first case in the English language literature of sarcoidosis manifested as hypercalcemic pancreatitis.

From the Department of Internal Medicine, Wright State University School of Medicine, Dayton, Ohio.

References

(1.) Boruchowicz A, Hachulla E, Cortot A, et al: Chronic pancreatitis: a complication of sarcoidosis? Eur J Gastroenterol Hepatol 1996; 8:1125-1127

(2.) McCormick PA, Malone D, FitzGerald MX, et al: Pancreatitis in sarcoidosis. BMJ 1985; 290:1472-1473

(3.) Boruchowicz A, Wallaert B, Cortot A, et al: Idiopathic acute pancreatitis and sarcoidosis. Gastroenterol Clin Biol 1995; 19:439-441

(4.) Cronin CC, Dineen SF, O'Mahony MS, et al: Precipitation of hypercalcemia in sarcoidosis by foreign sun, holidays: report of four cases. Postgrad Med J 1990; 66:307-309

(5.) Friedman HZ, Weinstein RA: Sarcoidosis of the pancreas. Arch Intern Med 1983; 143:2182-2183

KEY POINTS

* Hypercalcemia is a well-established cause of acute pancreatitis.

* Hypercalcemia-induced pancreatitis has been reported in patients with sarcoidosis after sun exposure.

* Some patients have additional risk factors for pancreatitis in the form of alcohol abuse.