Primary hyperparathyroidism (PHPT) is rarely associated with the occurrence of acute or chronic pancreatitis. Hypercalcemia plays a major role in the pathogenesis. We report five cases of pancreatitis revealing PHPT.
The causes of pancreatitis are largely dominated by gallstones and alcohol. Primary hyperparathyroidism (PHPT) is rarely associated with the development of pancreatitis. It is considered as well a cause of acute or chronic pancreatitis (AP or CP). Hypercalcemia secondary to the secretion of parathyroid hormone (PTH) plays a major role in the pathogenesis, but other mechanisms may be involved. The manifestations are polymorphic. We report five cases of pancreatitis revealing PHPT.
This is a retrospective and descriptive study from January 2011 to December 2014, including all patients admitted to intensive care units or gastroenterology department at Hôpital Principal de Dakar (HPD) for an acute or chronic pancreatitis revealing a PHPT.
The diagnosis of pancreatitis was retained in the presence of at least 2 of the 3 following elements: abdominal pain, levels of serum amylase, or lipase greater than 3 times the normal, or characteristic aspects of AP (oedema or pancreatic necrosis, acute necrosis collection) or CP (pancreatic atrophy or hypertrophy, pancreatic calcifications, ductal abnormalities, and cysts) at imaging. For PHPT, hypercalcemia associated with increased PTH levels posed diagnosis. A search of the secretory lesion was made by imaging.
Other causes of acute or chronic pancreatitis were sought, including cholelithiasis, alcohol consumption, or hypertriglyceridemia. Patients with another etiology that could explain pancreatitis were not retained. The epidemiological, clinical, and paraclinical outcomes of the patients were noted.
During the study period, 61 patients were hospitalized for pancreatitis (54 AP and 7 CP). Among them, 5 patients had a pancreatitis revealing PHPT (8%). Hospital prevalence of this association was 1.25 cases per year. All patients were female, with mean age 54 years [40–76 years]. One patient was diabetic and hypertensive, with stroke sequelae like left hemiparesis, another was diabetic, and a third was hypertensive. No patient had consumed alcohol and one was smoking 40 pack-years.
Revealing clinical symptoms were abdominal pain in all cases associated with vomiting in two patients. One patient was overweight with a body mass index (BMI) at 27.34 kg/m2 and two were obese with BMI, respectively, at 32 and 34 kg/m2. Clinical examination found in all cases a sensitive but flexible abdomen. One patient had hypovolemic shock.
AP was suspected and confirmed by dosage of pancreatic enzymes and imaging. Lipasemia was high in four cases with a rate of more than 40 times normal in the case of AP that had occurred on a normal pancreas. Amylasemia was also high in both cases where it was performed. Abdominal CT scan showed a homogeneous pancreatic hypertrophy without necrosis (
PHPT was in all cases revealed by an AP. Searching for an hyperparathyroidism was achieved after eliminating the most common causes of AP. No patient was taking alcohol. Cholelithiasis or early change in liver function tests was not found enabling us to eliminate a biliary etiology. Serum calcium was high in all cases with an average of 119.6 mg/L (104–130). There was no context of neoplasia and serum protein electrophoresis showed no monoclonal peak or hypogammaglobulinemia. The diagnosis of PHPT was confirmed by the dosage of PTH which was high in 4 cases with a mean value of 331 ng/L (199–536) and normal in a patient. Patients characteristics are summarized in Table
Patients, pancreatitis, and PHPT characteristics.
|
Sex | Age | Amylase | Lipase | Type of pancreatitis | Calcemia | PTH | Secretory parathyroid lesion |
---|---|---|---|---|---|---|---|---|
1 | F | 40 | 640 (7, 8 N) | Benign AP on CP | 130 | 536 (8, 2 N) | Right parathyroid adenoma of 25 × 23 mm | |
2 | F | 43 | 3694 (61 N) | Severe AP | 119 | 199 (3 N) | Left parathyroid adenoma of 1.6 cm × 1.4 cm | |
3 | F | 57 | 2466 (41 N) | Benign AP | 128 | 61 | Right lateroesophageal parathyroid adenoma at the cervicothoracic hole of 27 × 12 mm | |
4 | F | 76 | 419 (7 N) | Benign AP on CP | 104 | 231 (4, 8 N) | Left ectopic (adrenal?) parathyroid adenoma of 31 × 35 × 63 mm | |
5 | F | 55 | 1114 (13, 6 N) | 279 (4, 7 N) | Benign AP | 117 | 360 (5, 5 N) | Right parathyroid adenoma of 7 mm |
The secreting lesion was found in all cases by ultrasound or computed tomography. This was a parathyroid nodular lesion in 3 patients. In one case, the nodule was lateroesophageal right at the cervicothoracic hole whose character of hyperfunctional parathyroid was confirmed by MIBI-Tc-99m scintigraphy (Figure
Hyperfunctional parathyroid adenoma in retrotracheal and right paraesophageal position (arrow).
Furthermore, two patients who had an AP on CP had bilateral renal lithiasis. These lesions were responsible for a deterioration of renal function with a renal clearance, respectively, to 19 and 20 mL/min.
Therapeutically, all patients initially received symptomatic management of the AP. Only one patient received bisphosphonates awaiting surgery. An explorative cervicotomy was performed in three patients with resection of the parathyroid adenoma. The patient who is 74 years old did not have surgery due to significant comorbidities. The death was noted in one who had severe AP, following a superinfection of necrosis flows.
The outcome was favorable in 3 patients operated on, with normalization of PTH and serum calcium and no recurrence of the AP. The other one with CP and PHPT, who was not operated on, presented recurrent minor abdominal pain relieved by simple painkillers.
The occurrence of pancreatitis secondary to PHPT is not so rare, with a prevalence of 3.6% (1.5 to 15.3%) [
Pancreatitis occurs at an advanced stage of parathyroid disease [
The occurrence mechanism of pancreatitis during PHPT remains controversial but may be related to hypercalcemia, only statistically significant factor associated [
Hypercalcemia would act by several mechanisms: increased level of calcium in pancreatic juice at the origin of activation of trypsinogen to trypsin; activation of pancreatic enzymes through the lysosomal system and hydrolases; calcium precipitation and formation of protein plugs responsible for upstream pancreatitis. The direct toxic action of PTH on the pancreas is mentioned, but pancreatitis is not usually found in dialysis patients with elevated PTH [
Thus, viewing these different mechanisms, the association of PHPT and pancreatitis can take many forms. So, Jacob et al. proposed a classification of this association which can be in 4 forms [
The circumstances of discovery are dominated by acute pancreatitis which is revealing in 75% of cases [
The PHPT was diagnosed in all of our patients in the course of etiological research of this pancreatitis and hypercalcemia. The diagnosis is made by the PTH dosage which was elevated in 4 of our patients. PTH in patients with pancreatitis on PHPT is not higher than among those with only PHPT [
The discovery of PHPT imposes the research of other events related to hypercalcemia. Due to the advanced parathyroid disease, these lesions are more often found in patients with pancreatitis on PHPT than those having only PHPT. Thus, nephrolithiasis is present in 42–46% of cases [
Treatment in patients with pancreatitis on PHPT first rests on a management of the AP which can be fatal. Indeed, one of our patients who had a severe AP died before benefiting from parathyroidectomy. Pending parathyroid surgery, the management of serum calcium may be necessary based on rehydration and bisphosphonates as in our third patient.
Surgical resection of the secreting lesion causes a collapse of postoperative PTH levels [
The occurrence of pancreatitis during a hyperparathyroidism is rare. Normal or higher calcemia during acute or chronic pancreatitis should always draw attention and be subject to complementary explorations in search of endocrine or malignant cause.
The authors declare that there are no competing interests regarding the publication of this paper.