P1507 - Systemic Lupus Erythematous in Disguise: Acute Pancreatitis and Macrophage Activation Syndrome as Initial Presentation

Monday, October 23, 2023

10:30 AM – 4:15 PM PT

Location: Exhibit Hall

Presenting Author(s)

Saeed Abughazaleh, MD

St. Elizabeth’s Medical Center, Tufts University School of Medicine
Brighton, MA

Saeed Abughazaleh, MD¹, Saqr Alsakarneh, MD², Majd Khadra, MD³, Mohammad Almeqdadi, MD⁴
¹St. Elizabeth’s Medical Center, Tufts University School of Medicine, Brighton, MA; ²University of Missouri-Kansas City, Kansas City, MO; ³Detroit Medical Center/Wayne State University, Detroit, MI; ⁴Lahey Medical Center, Lexington, MA

Introduction: Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease that can manifest with various gastrointestinal disorders, affecting 8-40% of adult SLE patients. Acute pancreatitis and macrophage activation syndrome (MAS) are rare initial presentations of SLE. Here, we present a case of 20-year old female who presented with acute pancreatitis and later was diagnosed with SLE.

Case Description/Methods: A 20-year-old female presented with 1 day history of stabbing epigastric abdominal pain, nausea, and vomiting. On presentation, Blood pressure was 85/56, pulse 129, temperature 100.2 F, respiratory rate 22, and oxygen saturation 95%. Table 1 exhibits laboratory investigations which revealed pancytopenia, elevated liver enzymes, elevated ferritin, lipase, and triglycerides. Chest and abdominal CT scans showed bilateral axillary lymphadenopathy, patchy lower lobe consolidations, small pleural effusions, ascites, splenomegaly, and signs of acute pancreatitis (Figure 1). Patient was admitted for management of acute pancreatitis and further workup. She was kept NPO and started on IV fluids. Immunology workup showed positive results for ANA, Anti Ds-DNA, Anti-SSA, Anti-SSB, and Anti-cardiolipin. Further workup revealed high IgG titer and low complement levels of C3 and C4. Surprisingly, serology indicated positive CMV IgG AB, EBV IgG AB, and HSV type 1 IgG AB. Peritoneal fluid analysis revealed a serum ascites albumin gradient (SAAG) of 0.8, consistent with an exudative fluid, and cytology exhibited multiple hemophagocytic changes with abundant lymphocytes and histiocytes (Figure 2). Patient was treated with a total of three days of 1000 mg methylprednisolone in addition to IV fluids. Five days later, patient clinical status has improved, and she was discharged home with outpatient follow up in 2 weeks.

Discussion: Lupus-related pancreatitis carries a poor prognosis, with a mortality rate reaching 45%. Early detection and prompt intervention are essential in achieving favorable outcomes and preventing complications. Further research is warranted to better understand the pathogenesis and optimal management strategies for SLE patients presenting with acute pancreatitis and MAS.

Figure: Image 1: A CT Abdomen with contrast showing (1) an edematous pancreas with significant peri-pancreatic fluid, (2) enlarged lymph nodes in the celiac axis and gastro-hepatic region, (3) ascites. B CT Chest with contrast showing bilateral pleural effusion and patchy lower lobes consolidations (Red). C Ascites fluid sample showing hemophagocytic cells

Complete Blood Count	WBC	3.1 x103/ul	Immunology	ANA	1:1280 Positive
	Hgb	7.7 g/dl		Anti-Ds-DNA	>300 Positive
	Platelet	68 x103/ul		Anti-SSA	>8 Positive
Chemistry	BUN	24 mg/dl		Anti-SSB	>8 Positive
	Creatinine	0.7 mg/dl		Anti-Cardiolipin	36 Low Positive
	AST	226 U/L		Complement C3	14 Low
	ALT	93 U/L		Complement C4	< 2 Low
	ALP	223 U/L		IgG	2066 mg/dl
	Total Bilirubin	0.5 mg/dl	Serology	CMV IgG AB	136 Positive
	Albumin	1.8 g/dl		EBV IgG AB	>10 Positive
	Fibrinogen	221 mg/dl		HSV type 1 IgG	51.5 Positive
	LDH	417 U/L
	CRP	35.7 mg/L
	Ferritin	3508 ng/ml
	Lipase	1618 U/L
	Triglycerides	260 mg/dl

Disclosures:

Saeed Abughazaleh indicated no relevant financial relationships.

Saqr Alsakarneh indicated no relevant financial relationships.

Majd Khadra indicated no relevant financial relationships.

Mohammad Almeqdadi indicated no relevant financial relationships.

Saeed Abughazaleh, MD¹, Saqr Alsakarneh, MD², Majd Khadra, MD³, Mohammad Almeqdadi, MD⁴. P1507 - Systemic Lupus Erythematous in Disguise: Acute Pancreatitis and Macrophage Activation Syndrome as Initial Presentation, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.