Acute pancreatitis results in inflammation and autodigestion of pancreatic acinar cells leading to the elevation of pancreatic enzymes, namely, amylase and lipase. Serum lipase levels have long been considered a hallmark of acute pancreatitis. However, pancreatitis is not always the cause of elevated serum lipase levels. This series presents four patients who had elevated serum lipase levels without any demonstrable damage to the pancreas on imaging. On further evaluation, one of the patients was found to have acute on chronic kidney disease (CKD) whose lipase levels settled later. A patient presenting with an episode of acute gastroenteritis, later diagnosed to have Crohn's disease, also had hyperlipasemia, which improved after a course of initial antibiotics. Non-gastrointestinal causes, such as lupus nephritis and organophosphate (OP) poisoning, also had elevated lipase levels on presentation, in which the hyperlipasemia settled with supportive treatments. It is important to remember other causes of elevated lipase levels in patients with a normal pancreas on imaging studies.

Systematic lupus erythematosus (SLE) is a multisystem disease, including the gastrointestinal system in about half of SLE patients. As a rare complication of SLE, acute pancreatitis presents as generalized flare-ups in most cases of patients previously diagnosed with SLE. Here we report a rare case of acute pancreatitis as the initial presentation with later diagnosis of SLE.

Exceptionally, acute pancreatitis and reactive hemophagocytic syndrome (RHS) are observed in the course of systemic lupus erythematosus (SLE). However, the association of the two conditions has never been reported before. A 31-years-old woman with a 7-year history of SLE was admitted for abdominal pain and fever. Elevated serum amylase and pancreatic enlargement on computerized tomography confirmed the diagnosis of pancreatitis. Laboratory examinations revealed pancytopenia, abnormal hepatic tests, and elevation of serum LDH and triglyceride levels. Bone marrow aspiration showed hemophagocytosis. The patient responded well to high dose corticosteroids. About eighty cases of pancreatitis have been reported in patients with SLE. The mechanisms are still unclear: SLE as the primary etiologic factor, drug toxicity, especially steroids which play a controversial role, or infection. About 40 cases of RHS have been reported in patients with SLE, sometimes associated with active infection. Overall mortality is 38.5%. When RHS occurs as an initial manifestation of SLE, or in the course of active SLE, it responds well to immunosuppressive therapy.

Involvement of the pancreas in systemic lupus erythematosus is rare. The purpose of this article is to provide a detailed review of lupus-associated pancreatitis.

We describe 3 patients with lupus-associated pancreatitis and review the English literature of the last 30 years, including the demographic, clinical, therapeutic, and prognostic aspects of this disorder.

There were detailed descriptions of 77 patients, 88% were females. Median age was 27 years. In 44% of the patients pancreatitis developed within 1 year of the diagnosis of lupus, and 84% had active lupus at the time of pancreatitis. Abdominal pain was the most frequent pancreatitis-related symptom (88%), followed by nausea or vomiting (67%). In 97% the diagnosis of pancreatitis was based on laboratory evidence of elevated serum amylase or lipase. Abdominal computerized tomography and ultrasonography did not show signs of pancreatic involvement in 24 and 45%, respectively. The mortality rate was 27%; active lupus and some biochemical abnormalities were significantly associated with increased mortality. Treatment with corticosteroids or azathioprine was not associated with increased mortality. On the contrary, mortality was decreased in patients who were treated with these agents after the onset of pancreatitis (20% mortality, compared with 61% among those who were not treated with steroids for their pancreatitis, P = 0.005).

Pancreatitis should be suspected in any SLE patient with abdominal pain. Mortality rate is related to both active lupus and some biochemical markers. In most cases, the onset of pancreatitis appears unrelated to previous treatment with steroids or azathioprine. Moreover, treatment with these medications improves prognosis.

The possibility of nicotine toxicity, although rare, should be considered in cases of acute edematous pancreatitis.

A 30-year-old woman was hospitalized to identify the cause of an initial episode of acute edematous pancreatitis. The observation of native anti-DNA and antiphospholipid antibodies suggested lupus pancreatitis and/or an antiphospholipid syndrome, both subsequently ruled out. The final diagnosis was nicotine poisoning induced by the combination of a nicotine patch and tobacco smoking.

Although a nicotine patch has never been reported in connection with an episode of acute pancreatitis before, this case suggests that such an event might be a rare complication of an overdose of nicotine.

Symptoms of leukoencephalopathy led to hospital admission of a 59-year-old woman. In addition, a tumor of unknown nature in the pancreas was identified by abdominal ultrasound and CT scan. Following explorative laparotomy and pancreas tail resection, histopathologic analysis revealed a pancreatic pseudotumor with chronic fibrotic pancreatitis. Systemic lupus erythematosus (SLE) was diagnosed due to the presence of antinuclear antibodies (ANA) in serum, antiphospholipid antibodies, and involvement of the central nervous system. Leukoencephalopathy related to anticardiolipin antibodies in serum is a known but rare manifestation of SLE. The concomitant occurrence of chronic pancreatitis can be caused by the development of SLE-induced vasculitis in the pancreas. Subsequent complications of pancreatitis are responsible for the critical, life-threatening state of these patients and may be prevented by early identification of anticardiolipin antibodies and therapy for SLE.

The aim of this study was to evaluate whether corticosteroid use is the etiological agent in acute pancreatitis in patients with systemic lupus erythematosus, or whether it is related to the underlying connective tissue disorder. Hospital admissions at Thomas Jefferson University Hospital between 1982 and 2002 that carried the dual diagnosis of systemic lupus erythematosus and pancreatitis were identified, and demographic data, clinical interventions and parameters of clinical progression of their disease were identified. From 2947 admissions with systemic lupus erythematosus 25 (0.85%) were diagnosed as having acute pancreatitis; 76% of cases had active systemic lupus erythematosus on presentation, with an average of 4.4 organ involvement, and a clustering of renal disease (56%), pleural effusion (48%) and arthritis (44%) in these patients. Fifteen patients with active disease and three whose disease was inactive received increased doses of corticosteroids, and four active cases and one inactive one stayed on the same doses. Two inactive patients received no corticosteroids before or after the diagnosis of pancreatitis. Eighty-two percent of patients had clinical and laboratory improvement on the higher or maintenance dose of corticosteroids. We therefore concluded that acute pancreatitis is a rare manifestation of systemic lupus erythematosus, and corticosteroids do not appear to be the etiological agent.

There are many causes of acute abdominal pain, or abdominal "crises," in patients with systemic lupus erythematosus (SLE), most frequently the causes are serositis or vasculitis. Vasculitis generally causes small vessel abnormalities and may present with symptoms owing to mucosal damage, such as pain, diarrhea, or bleeding. We present a patient with SLE who had the acute onset of severe abdominal pain while hospitalized for a lupus flare and who was found to have a ruptured ileocolic aneurysm with intraperitoneal bleeding. She was successfully managed with angiographic embolization, without further complications. Although angiography is well established as a therapeutic intervention for mesenteric aneurysms of various etiologies, this is the first case of an SLE-related ileocolic aneurysm so managed. This entity should be considered in the differential diagnosis of abdominal pain in patients with lupus, and angiographic embolization should be considered in its management.

Vasculitis can affect every organ of the digestive system. In many cases, it may first present with gastrointestinal symptoms. In several forms of vasculitis, including Churg Strauss syndrome, Henoch-Schönlein purpura, and lupus, the majority of patients have gastrointestinal involvement. The astute gastroenterologist should consider vasculitic causes of the symptoms seen in many patients. Making the correct diagnosis requires a thorough understanding of the potential role of vasculitis in causing these symptoms and the appropriate path to making a diagnosis. This article reviews the variety of manifestations of vasculitis on the digestive system, and emphasizes diagnosis and clinical manifestations.

We analyzed the clinical and laboratory characteristics of 50 patients with catastrophic antiphospholipid syndrome (APS) (5 from our clinics and 45 from a MEDLINE computer-assisted review of the literature from 1992 through 1996). Thirty-three (66%) patients were female and 17 (34%) were male. Twenty-eight (56%) patients had primary APS, 15 (30%) had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 1 (2%) had rheumatoid arthritis. Mean age of patients in this series was 38 +/- 14 years (range, 11-74 yr). Three (6%) patients developed the clinical picture of catastrophic APS under the age of 15 years, and 11 (22%) were 50 years old or more. In 11 (22%) patients, precipitating factors contributed to the development of catastrophic APS (infections in 3, drugs in 3, minor surgical procedures in 3, anticoagulation withdrawal in 2, and hysterectomy in 1). The presentation of the acute multi-organ failure was usually complex, involving multiple organs simultaneously or in a very short period of time. The majority of patients manifested microangiopathy--that is, occlusive vascular disease affecting predominantly small vessels of organs, particularly kidney, lungs, brain, heart, and liver--with a minority of patients experiencing only large vessel occlusions. Thrombocytopenia was reported in 34 (68%) patients, hemolytic anemia in 13 (26%), disseminated intravascular coagulation in 14 (28%), and schistocytes in 7 (14%). The following antibodies were detected: lupus anticoagulant (94%), anticardiolipin antibodies (94%), anti-dsDNA (87% of patients with SLE), antinuclear antibodies (58%), anti-Ro/SS-A (8%), anti-RNP (8%), and anti-La/SS-B (2%). Anticoagulation was used in 70% of the patients, steroids in 70%, plasmapheresis in 40%, cyclophosphamide in 34%, intravenous gammaglobulins in 16%, and splenectomy in 4%. Most patients, however, received a combination of nonsurgical therapies. Death occurred in 25 of the 50 (50%) patients. In most, cardiac problems seemed to be the major cause of death. In several of these, respiratory failure was also present, usually due to acute respiratory distress syndrome and diffuse alveolar hemorrhage. Among the 20 patients who received the combination of anticoagulation, steroids, and plasmapheresis or intravenous gammaglobulins, recovery occurred in 14 (70%) patients. The use of ancrod and defibrotide appeared to be effective in the 2 respective patients in whom they were used.

We report five cases of pancreatitis in systemic lupus erythematosus. Three patients died. The cause of death was directly related to pancreatitis in two cases. Several causes of pancreatitis may be suspected: vasculitis, thrombosis when associated to antiphospholipid syndrome, biliary calculi, infection, metabolic abnormalities or adverse effects of therapy. About seventy cases of pancreatitis have been reported in systemic lupus erythematosus in the literature Pancreatitis was the inaugural sign of lupus in six cases. In 12 cases, besides lupus, no other cause of pancreatitis was found. However, pancreatic vasculitis or thrombosis was rarely demonstrated even in post-mortem examinations. The role of corticosteroids in pancreatitis in lupus is controversial since the evolution of pancreatitis in lupus is generally good with corticosteroids. We think that diagnosis of pancreatitis in lupus should not implicate discontinuation or decrease of corticosteroids dosage, unless their responsibility is absolutely demonstrated.

Four patients with systemic lupus erythematosus visiting an emergency department were reported with clinical presentation of acute pancreatitis. All had high levels of anticardiolipin antibodies (aCL); at autopsy, one had multiple thrombi within the blood vessels of the pancreas. The aCL antibody might play an important role in the pathogenesis of acute pancreatitis. Three patients had multiple organ involvement, especially the central nervous system, concomitant with an acute attack of pancreatitis; all died from these complications. Thus it was suggested that initial acute pancreatitis is an omnious prognostic factor in lupus patients visiting an emergency department. When evaluating lupus patients visiting an emergency department, clinicians should be alert to the status of aCL and the possibility of pancreatitis in these patients.

Systemic lupus erythematosus (SLE), an autoimmune disease with multisystem involvement, has been reported to be associated with a number of gastrointestinal complications. Acute pancreatitis is an unusual manifestation of this disorder. This report presents two cases of patients with SLE who developed chronic pancreatitis with no other identifiable etiology.

A patient with connective tissue disease presenting with both protein-losing enteropathy and pancreatic involvement is reported. A 52-year-old female was admitted because of mild epigastralgia, anasarca and ascites. Serum albumin, transferrin and zinc, showed low levels. An Upper G.I. series and endoscopy showed thickened folds of the duodenum and the jejunum. Biopsy specimens revealed lymphangiectasia in edematous villi. 99mTc-labeled human serum albumin scintigram showed abnormal radioactivity in the small intestine 90 minutes after intravenous injection, indicating protein-losing enteropathy. Hypoalbuminemia was ameliorated by glucocorticoid therapy, but recurred twice when glucocorticoid treatment was tapered. Hypoalbuminemia has not occurred since intestinal lymphangiectasia was improved with glucocorticoid treatment. Levels of elastase 1 and lipase were high in serum and ascites on admission. Endoscopic retrograde pancreatogram showed no abnormalities. Serum pancreatic enzymes were also ameliorated by glucocorticoid therapy, but slightly high levels continued for about one year and a half. This case might have been diagnosed as systemic lupus erythematosus although mixed connective tissue disease was also suspected. There are few reports of protein-losing enteropathy and pancreatic involvement associated with connective tissue diseases. Protein-losing enteropathy and pancreatic involvement were ameliorated with glucocorticoid treatment, suggesting participation of immunological mechanisms.

We report a case of recurrent acute pancreatitis in a 57-year-old man with reactivation of Wegener's granulomatosis. An association between acute pancreatitis and Wegener's granulomatosis has not been reported previously. Six episodes of abdominal pain and hyperamylasemia occurred and were complicated by development of a pancreatic pseudocyst. New cavitary lung lesions typical of Wegener's granulomatosis led to treatment with increased glucocorticoid dosage, resulting in rapid resolution of the pancreatitis and pulmonary lesions. Acute pancreatitis may be a clinical manifestation of the histologic pancreatic vasculitis observed in Wegener's granulomatosis. Reactivation of Wegener's granulomatosis should be considered when a patient with the disorder develops otherwise unexplained acute pancreatitis.