Acute pancreatitis in pregnancy and familial chylomicronemia syndrome: case report and literature review
Division of Endocrinology and Metabolism, Department of Internal Medicine, American University of Beirut Medical Center, Beirut 1107 2020, Lebanon.
Correspondence to: Dr. Jessica Abou Chaaya, Department of Internal Medicine, American University of Beirut, Cairo St., Beirut 1107 2020, Lebanon. E-mail:
Acute pancreatitis rarely occurs in pregnancy, with hypertriglyceridemia being the fourth leading cause during pregnancy. Hypertriglyceridemia, of which Familial Chylomicronemia Syndrome is the most severe form, ranks among the four principal causes of pancreatitis in pregnancy. Total Plasma exchange (TPE) has been found to be an effective and safe intervention both as a therapeutic and a prophylactic act. A 22-year-old female with FCS presented at the 21st week of gestation with acute hypertriglyceridemia pancreatitis. Despite medical management, she was then started on TPE at the two-week follow-up after serum triglyceride level was out of control. The triglyceride dropped from 55.0 % to 77.5 % during these sessions. Despite these interventions, pancreatitis recurred in week 34. An emergency C-section was carried out after a drop in the fetal heart rate. Postpartum triglycerides dropped by 57 % but remained above 1,000 mg/dl. FCS is difficult to manage during pregnancy, and it frequently fails to respond to various pharmacologic lines. TPE can help prolong a pregnancy, but it is not a definite treatment. Novel therapies for hypertriglyceridemia in pregnancy await additional safety testing.
Acute pancreatitis rarely occurs in pregnancy, with an estimated incidence rate of 1 per 1,000 to 10,000 pregnancies[1-3]. Delay in recognition and/or proper management is associated with high maternal and fetal mortality rates. Due to the rarity of the disease and the wide range of different medical and obstetric etiologies of epigastric pain, the diagnosis is frequently missed. Acute pancreatitis in pregnancy can be secondary to different etiologies, with gallstones being the most common cause[3-5] [Table 1]. The risk of pancreatitis associated with hypertriglyceridemia (HTG) rises with duration of pregnancy: 19% in the first trimester, 26% in the second trimester, 53% in the third trimester, and 2% in the postpartum period[5,6].
Most common causes of acute pancreatitis in pregnancy
|Causes of pancreatitis in pregnancy||Percentage %|
Women with hereditary anomalies in lipoprotein metabolism, such as Familial Chylomicronemia Syndrome (FCS), are more likely to develop severe HTG later in pregnancy. In addition, according to Fredrickson classification, familial HTG (type 4), familial combined hyperlipidemia (type 2B), familial dysbetalipoproteinemia (type 3), and inherited lipodystrophy syndromes are all known primary genetic disorders that cause hypertriglyceridemia. FCS (OMIM 238600), commonly known as type 1 hyperlipoproteinemia and previously as lipoprotein lipase deficiency (LPLD), is a rare autosomal recessive disorder with an incidence of 1-2 per million. It is the most severe form, with serum chylomicron accumulation, severe HTG (> 1,000 mg/dL), and recurrent pancreatitis occurring in childhood. Patients can have hepatosplenomegaly, lipemia retinalis, and eruptive xanthomas[8-10]. Bi-allelic mutations in the LPL gene are the most common cause of this disorder, followed by mutations in other genes that contribute to the function of LPL, such as Apolipoprotein C2 (APOC2), Apolipoprotein A5 (APOA5), Lipase Mutation Factor 1 (LMF1), and glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 (GPIHBP1). Genetic testing can be helpful in establishing the diagnosis when clinical or biochemical data are inconclusive.
Based on small case series and case reports[5,11], the mainstream therapy for FCS has consisted of a very low-fat diet (10% to 15% of daily caloric intake) to lower plasma triglyceride (TG) levels below 750-880 mg/dL and abort the attacks of pancreatitis. Due to the absence of a standard prophylactic regimen, additional therapies have included fibrates, marine omega-3 fatty acids, eicosapentaenoic acid, nicotinic acid, and therapeutic plasma exchange (TPE). Nil per Os (NPO or nothing by mouth) and hydration with or without artificial feeding, heparin, insulin, and TPE have all been described as treatments for acute bouts of pancreatitis. TPE has become more widely adopted due to promising results, with the first case described in the literature being that of Swoboda et al. in 1993[4,7,13-15].
Here, we describe a pregnant patient with uncontrolled FCS who experienced two episodes of pancreatitis during the second and third trimesters and delivered a healthy premature baby at week 33. The aim of reporting this case is to emphasize the challenges faced upon treatment of acute pancreatitis secondary to FCS-induced HTG. Multiple therapies have been used, including a very low-fat diet, fibrates, icosapent ethyl, intravenous insulin, and frequent elective TPE sessions.
We present the case of a 22-year-old primigravid woman with a medical history of poorly managed FCS diagnosed at the age of three months. Her family background reveals a history of severe
The initial presentation to our Emergency Department was at 21 weeks of gestation with acute epigastric pain radiating to the back that was relieved by leaning forward, as well as frequent episodes of vomiting. Two weeks prior to her Emergency department (ED) presentation, she visited the Endocrinology Clinic for tighter control of her TG (TG 4,707 mg/dL), (Total Cholesterol 376 mg/dL, HDL 13 mg/dL). She was only taking Icosapent ethyl 2 g twice daily because her physician had discontinued her fenofibrate due to its potential teratogenic adverse effect. During this visit, Niacin (500 mg/day) was added, and she was referred to a registered dietitian for diet counseling. Given the risk of gestational diabetes in this high-risk patient and the low level of shortage, niacin was stopped a few days later. Her vital signs were stable at the time of presentation, with a blood pressure of 90/54 mmHg, a heart rate of 85 bpm, a temperature of 36.7 oC, a respiratory rate of 20 bpm, and a SO2 level of 99%. On physical examination, a gravid soft abdomen and epigastric discomfort were noted. She did not have any eruptive xanthomas, and her slit lamp eye exam came back normal. Venous blood was extremely lipemic. Blood studies showed elevated TG 4,273 mg/dL
Upon this diagnosis, the patient began adhering to NPO instructions and started on a regimen of continuous IV Insulin Glulisine (0-3 U/hr), daily 40 mg subcutaneous molecular weight heparin, and continuous aggressive intravenous (IV) fluid r (D10W 120 ml/h) with the aim to keep blood sugar levels between 100 mg/dl and 200 mg/dl. Her blood glucose ranged from 76 to 282 mg/dL; thus, she did not experience any episodes of hypoglycemia (75 mg/dL or below). Thromboprophylaxis was indicated in a high coagulable state with HTGP and bed rest. She had significant symptomatic hypocalcemia
Figure 1. Changes in serum triglyceride level during the first hospitalization for pancreatitis. TG: triglycerides.
TPE was selected to possibly decrease the serum level of TG and eventually decrease the risk of acute pancreatitis. All TPE sessions were done using 5% albumin as a replacement fluid and heparin as an anticoagulant through a right femoral Central venous catheter. Before and after each session, a Nonstress test was performed. From week 26 to week 33, she received a total of 12 TPE treatments, with 2 sessions during each admission every 7-13 days, resulting in a serum TG reduction of 55.0 percent to 77.5 percent after each visit [Figure 2 and Table 2]. Note that the patient did not undergo screening for gestational diabetes by oral glucose tolerance test because, despite the decrease in TG level after each session, the patient presented on week 34 of pregnancy with recurrent pancreatitis.
Changes in TG levels pre- and post-TPE after each admission (2 sessions per admission), TG level and percentage decrease; mean and standard deviation for pre- and post-TPE TG levels, for TG level and percentage decrease
|Admission||Pre-TPE TG (mg/dl)||Post-TPE TG (mg/dl)||Amount TG decrease (mg/dl)||Percentage decrease (%)|
On week 34, she presented to the Emergency room with a second episode of HTGP with early contractions. Studies revealed serum TG 4,719 mg/dl, calcium 6.6 mg/dl, amylase 704 IU/L, and lipase 519 U/L. Oral intake was suspended, and TPE was started according to the same protocol as before. An emergency C-section was performed a few hours later for fetal distress. A yellowish milky intra-abdominal fluid (approximately 500 cc) was discovered intra-operatively. She delivered a healthy female baby weighing 2,165 grams, with an Apgar score of 4 at 1 min and 9 at 5 min. The neonate was admitted to the neonatal ICU for 2 days because of prematurity. TG level dropped by 29% just after delivery, and by 57% (2,029 mg/dl) on the second day from the initial serum level on presentation. A very low-fat diet was initiated along with IV insulin, aiming to reduce TG below 1,000 mg/dL. Insulin was stopped after 4 days at a TG level of
We presented the case of a 22-year-old female patient with poorly controlled FCS presenting with acute pancreatitis secondary to FCS-induced HTG. This case revealed the challenges faced during the treatment of pancreatitis caused by severe HTG. Acute pancreatitis in pregnancy is an uncommon complication that affects 1 in 1,000 to 1 in 10,000 women[1-3,5]. Its fetal and maternal morbidity burden was traditionally significant until recently, owing to early diagnosis and better management. The considerable fall in maternal and fetal mortality rates in current reports (approximately 0% to 3%, respectively) compared to what was previously recorded (37% and 60%, respectively) demonstrates this. Maternal adverse outcomes include pancreatic pseudocyst formation, pancreatic necrosis, shock, hypocalcemia, preeclampsia, eclampsia, chylous ascites, pleural effusion, and chronic pericarditis. The fetal risks arise from preterm labor, which may result in prematurity and, in severe cases, lead to fetal death in utero[5,17]. On the other hand, there is evidence supporting the association between mild to moderate HTG, low-grade inflammation, and risk of acute pancreatitis. This correlation has been substantiated in multiple studies, including the work of Hansen et al., which involved a sample size of approximately 118,000 individuals with HTG. The incidence of primary HTGP in pregnancy is estimated to be 1 in 25,000 individuals. It is still uncertain at what exact serum TG level pregnant women might develop pancreatitis. However, women with serum TG levels above 1,000 mg/dL are at an increased risk of developing severe pancreatitis (4%), and at a median TG level of 2,600 mg/dL, the risk has been observed to rise to 14%[2,4,7,9].
Primary HTG-induced pancreatitis was shown to be more severe than other etiologies, and patients had worse outcomes[2,3,5,7]. In women with primary HTG, the risk of developing pancreatitis increases during pregnancy and becomes more pronounced as the pregnancy advances[2,3]. This is due to physiologic hormonal changes that occur in the last two-thirds of pregnancy, which are principally manifested by a rise in serum estrogen levels and an insulin-resistant state. This is attributed to the downregulation of the LPL gene expression, which reduces LPL function and, as a result, the clearance of both VLDL and TG. The placental lactogen, on the other hand, increases TG substrates, free fatty acids (FFA), and adipose tissue lipolysis. Despite a 2.5-fold increase in TG levels, pregnant women without primary HTG seldom develop pancreatitis because TG levels rarely exceed 300 mg/dL by the third trimester; this is of minimal clinical consequence[2,3,5].
FCS (OMIM 238600) is an extremely rare autosomal recessive disorder caused by biallelic mutations of either the LPL or apo C-II, which induce lipoprotein lipase (LPL) and apo C-II deficiencies, resulting in defective TG hydrolysis. It is characterized by severe HTG that begins in childhood and manifests as hepatosplenomegaly, lipemia retinalis and eruptive xanthomas, and recurrent pancreatitis[7-10]. In fact, many cases of FCS syndrome in women are discovered in the third trimester of pregnancy as there are marked changes in the lipid profile, often resulting in a 2- to 4-fold increase in triglyceride level; therefore, pregnancy is an exacerbating factor for uncontrolled FCS. In addition, according to the “reset hypothesis”, weaning, rather than delivery, will terminate the maternal metabolic effects of pregnancy. Throughout literature, few cases are reported of pregnant women presenting with acute pancreatitis secondary to FCS-induced HTG[9,17,19,20-24] [Table 3]. Acute pancreatitis is hypothesized to be caused by the lipolysis of TG by pancreatic lipase. This process results in the generation of free fatty acids, which, in turn, have a direct cytotoxic effect on the acinar cells[4,7,11]. FCS is a severe form of primary genetic HTG that manifests during infancy and is characterized by extremely high blood TG levels (>1,000 mg/dl), resulting in recurrent HTGP[3,10]. The majority of HTGP cases occur when TG levels exceed 3,000 mg/dl, and most guidelines recommend commencing therapy at a TG level of 2,000 mg/dl.
Cases of pregnant women with acute pancreatitis secondary to FCR-induced HTG and treatment modalities used
|Author||Age||Peak TG (mg/dl)||Pancreatitis episodes||Time onset of pancreatitis||Treatment||Number of TPE sessions|
|Shi et al. (2020)||26||1,777||2 (1 in each pregnancy)||40 + 1
37 + 2
|Emergent C-section, NPO||0|
|Kim et al. (2020)||19||3,667||1||25||Insulin
SC Heparin Marine Oil
|Gupta et al. (2014)||32||12,570||1||38 + 4||Emergent C-section
|Tsai et al. (2004)||23||6,000||1||28||Gemfibrozil||0|
|Lennertz et al. (1999)||34||9,410||1||31||TPE||1|
|Sanderson et al. (1991)||33||12,000||2 (1 in each pregnancy)*||33
32 + 5
|Low-fat diet and Hydration||0|
|Our case||22||5,000||2 (same pregnancy)||21 & 34||NPO
So far, there are no established guidelines for managing HTG during pregnancy[2,5,11]. The primary objective of treatment is to lower serum TG levels and prevent HTGP. Most pharmacological agents used in pregnancy are classified as pregnancy class C and should only be used when other conservative approaches have failed. Furthermore, most TG-lowering drugs are ineffective in FCS as compared to other primary HTG disorders, which is also related to LPL deficiency. The safe TG limit for pregnant women with HTGP is clear: less than 1,000 mg/dl. TG levels of less than 500 mg/dl may be safer[4,7,11]. However, the safe limit for patients without pancreatitis is debatable. Safe limits should be based on patient characteristics, attainable TG levels without significant side effects by therapeutic apheresis, duration of pregnancy, and presence of symptoms.
In our patient, we had limited treatment options due to the scarcity of studies in the literature regarding the management of FCS during pregnancy. While various novel therapies were studied in the general population, they are rarely investigated in pregnant women. Given the failure of our patient to respond to nutritional recommendations, we were restricted to conventional therapy options. In addressing her acute pancreatitis and recognizing the imperative of preventing recurrence, we decided to employ TPE. Throughout literature, different treatment techniques were studied and used in such cases, but on the other hand, novel therapies required further testing. The basic lifestyle approach is a very low-fat diet and medium-chain fatty acids. The core therapy is to limit saturated and unsaturated fat intake to 10%-15% of total daily calories, or 20-40 g[2,5,9-12]. To promote compliance, patients are advised to seek the instructions of a registered dietitian. It is necessary to take fat-soluble vitamin supplements. It is also critical to limit alcohol consumption and avoid certain medications that might exacerbate HTG. In addition, the patient was advised to use medium-chain fatty acids since they decrease postprandial rise in TG level by decreasing the synthesis of chylomicrons. Our patient's TG level was not controlled despite following a very low-fat diet as directed by a registered dietician and documented by a food frequency questionnaire, and additional therapy was indicated.
Some conventional and novel therapy options include: (1) Fibric acid derivatives: They have a limited role in FCS because they promote TG-rich particle clearance by acting on the peroxisome proliferator-activated receptor alpha (PPAR- ), increasing LPL activity, and reducing apoC3 levels[7,10]. Because they are classified as category C by the Food and Drug Administration, there is minimal information about their usage during pregnancy (FDA). According to certain case studies, they can be used after the second trimester with no documented side effects, including teratogenicity[27-29]. Similarly, our patient was prescribed fenofibrate but did not respond, and no teratogenic effects were observed; (2) Niacin: Because it is designated as pregnancy class C, there is limited data on its utilization in FCS and its safety during pregnancy. It reduces TG through lowering VLDL secretion, peripheral lipolysis, and apoC-3 levels. With dosages of 2 to 3 grams per day, it can lower plasma TG levels by around 20%, which is normally seen in 6 weeks[10,12]. We were not able to assess its potency in our patient because she had to stop using it after only a few days due to a shortage of supply. It is also worth mentioning that niacin is associated with an increased risk of thrombocytopenia, and therefore, its use is questionable and its safety during pregnancy is now well established[30-33]; (3) Volanesorsen: It is a second-generation antisense oligonucleotide (ASO) to apoC3. As a novel licensed therapy, it was proven to be effective in patients with FCS, with a 50% decrease in TG and no episodes of pancreatitis. It was not tested in pregnant women; however, animal trials revealed no maternal-fetal harm. Furtherly, this drug was inaccessible due to its high cost in our case.
On the other hand, some treatment techniques require hospitalization, including: (1) Insulin: It is effective at lowering serum TG levels by increasing the activity of the LPL enzyme. It can be given intravenously or subcutaneously, with identical outcomes; however, the IV method is more practical and easier to titrate. It acts synergistically with fasting, leading to an 87% reduction in TG when employed in combination, compared to 40% when used alone. When IV insulin was combined with TPE in a study, it resulted in a 92.6% decrease in 24 h at the expense of adverse effects such as respiratory distress and acute kidney injury. Shaka et al. described its use in FCS in a non-diabetic patient, where it was an effective therapy, but the result was transient, and the patient eventually needed elective TPE. Similarly, IV insulin proved a temporary therapy in our patient since once it was withdrawn and feeding resumed, TG went up again;
Our patient's post-treatment TG level was virtually always over 1,000 mg/dl, even though the sessions were done practically every week. Furthermore, pharmaceutical therapy for decreasing TG throughout her pregnancy was ineffective. Despite regular TPE treatments, she developed a second HTGP in her third trimester, and the TPE attempt failed due to premature contractions and abnormalities in fetal heart rate, necessitating an urgent C-section. TPE's use as a conventional therapy is hampered by the high cost of treatment, its temporary nature, and its lack of availability in all healthcare settings[7,9]. Compared to TPE, double filtration lipid apheresis was shown to be less successful in reducing TG and have greater side effects, including higher infection rates, hemorrhage, hemolysis, anemia, and technical issues owing to tube blockage. This case is of importance to be reported since only rare subjects are reported in the literature and the challenge of managing acute pancreatitis in patients with FCS-induced HTG. In addition, our patient is the first to develop pancreatitis twice during a single pregnancy, even after prophylactic TPE. The limitation is the lack of genetic testing due to the low socioeconomic status of the patient, and the unavailability of novel drugs in the region.
In conclusion, FCS is a very rare primary hypertriglyceridemia disorder that can manifest itself in pregnancy as severe hypertriglyceridemia and acute pancreatitis. A metabolic disease that is manifested by disruption of chemical reactions leading to accumulation of excessive metabolites. Our patient, diagnosed in infancy, was uncontrolled throughout adolescence, and upon pregnancy, the complications started to occur. Acute pancreatitis develops in uncontrolled FCS patients, but in pregnancy, this remains a challenging approach with limited studies. Pharmacological agents are often ineffective, and a very low-fat diet may not be enough to keep HTG under control. To prevent HTGP and unfavorable maternal-fetal outcomes, affected women should be followed closely and frequent TPE is advised. Safety studies on novel therapies for hypertriglyceridemia due to FCS in pregnancy are required.
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The authors thank the patient for her participation.
Participated in literature review, collecting data, and writing the manuscript: Jaafar B, Abou Chaaya J, Ammar S
Reviewed the manuscript: Salti I
Availability of data and materials
The data that support the findings of this study are available from the corresponding author, [B.J], upon reasonable request.
Financial support and sponsorship
Conflicts of interest
All authors declared that there are no conflicts of interest.
Ethical approval and consent to participate
An oral informed consent was taken from the patient to participate since it is an anonymous case report.
Consent for publication
An oral informed consent was taken from the patient to publish since it is an anonymous case report.
© The Author(s) 2023.
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Cite This Article
Jaafar B, Abou Chaaya J, Ammar S, Salti I. Acute pancreatitis in pregnancy and familial chylomicronemia syndrome: case report and literature review. Metab Target Organ Damage 2023;3:21. http://dx.doi.org/10.20517/mtod.2023.12
Jaafar B, Abou Chaaya J, Ammar S, Salti I. Acute pancreatitis in pregnancy and familial chylomicronemia syndrome: case report and literature review. Metabolism and Target Organ Damage. 2023; 3(4): 21. http://dx.doi.org/10.20517/mtod.2023.12
Jaafar, Batoul, Jessica Abou Chaaya, Shahed Ammar, Ibrahim Salti. 2023. "Acute pancreatitis in pregnancy and familial chylomicronemia syndrome: case report and literature review" Metabolism and Target Organ Damage. 3, no.4: 21. http://dx.doi.org/10.20517/mtod.2023.12
Jaafar, B.; Abou Chaaya J.; Ammar S.; Salti I. Acute pancreatitis in pregnancy and familial chylomicronemia syndrome: case report and literature review. Metab Target Organ Damage. 2023, 3, 21. http://dx.doi.org/10.20517/mtod.2023.12
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