Acute pancreatitis in juvenile systemic lupus erythematosus: a manifestation of macrophage activation syndrome?

06:00 EDT 15th September 2010 | BioPortfolio

Summary of "Acute pancreatitis in juvenile systemic lupus erythematosus: a manifestation of macrophage activation syndrome?"

Acute pancreatitis (AP) is a rare and life-threatening manifestation of juvenile systemic lupus erythematosus (JSLE). The objective of this study was to evaluate the prevalence and clinical features of AP in our JSLE population. AP was defined according to the presence of abdominal pain or vomiting associated to an increase of pancreatic enzymes and/or pancreatic radiological abnormalities. Of note, in the last 26 years, 5367 patients were followed up at our Pediatric Rheumatology Unit and 263 (4.9%) of them had JSLE diagnosis (ACR criteria). AP was observed in 4.2% (11/263) of JSLE patients. The median of age of the JSLE patients at AP diagnosis was 12.4 years (8.8-17.9). All of them had lupus disease activity at AP onset. Three patients were receiving corticosteroids before AP diagnosis. Interestingly, 10/11 JSLE patients fulfilled preliminary guidelines for macrophage activation syndrome, three of them with macrophage hemophagocytosis in bone marrow aspirate and hyperferritinemia. The hallmark of this syndrome is excessive activation and proliferation of T lymphocytes and macrophages with massive hypersecretion of proinflammatory cytokines and clinically it is characterized by the occurrence of unexplained fever, cytopenia and hyperferritinemia. AP treatment was mainly based on intravenous methylprednisolone. Four JSLE patients with AP died and two developed diabetes mellitus. In conclusion, AP was a rare and severe manifestation in active pediatric lupus. The association between AP and macrophage activation syndrome suggests that the pancreas could be a target organ of this syndrome and that pancreatic enzyme evaluation should also be carried out in all patients.


Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.

Journal Details

This article was published in the following journal.

Name: Lupus
ISSN: 1477-0962


PubMed Articles [10625 Associated PubMed Articles listed on BioPortfolio]

Juvenile systemic lupus erythematosus with unusual manifestation of lupus-associated panniculitis.

Juvenile systemic lupus erythematosus (JSLE) is a rare multisystem autoimmune disease with broad heterogeneity of clinical manifestations. Diagnosing JSLE is often very challenging. This life-threaten...

Bone Mineral Density in Juvenile Onset Systemic Lupus Erythematosus.

To compare bone mineral density in patients with juvenile-onset Systemic lupus erythematosus and healthy controls.

Tumor necrosis factor-alpha single nucleotide polymorphisms in juvenile systemic lupus erythematosus.

Juvenile systemic lupus erythematosus (JSLE) is a multi-system autoimmune disorder of unknown origin. Given the importance of the contribution of pro-inflammatory cytokines, including tumor necrosis f...

Redefining cutaneous lupus erythematosus: a proposed international consensus approach and results of a preliminary questionnaire.

There is currently no uniform definition of cutaneous lupus erythematosus (CLE) upon which to base a study population for observational and interventional trials. A preliminary questionnaire was deriv...

Lupus nephritis: frequency, histological forms and predictors of renal disease.

Lupus nephritis is a common and serious damage in systemic lupus erythematosus. The aim of our study is to define the frequency and to identify the predictors of lupus nephritis in patients with syste...

Clinical Trials [3860 Associated Clinical Trials listed on BioPortfolio]

Five-Year Actively Controlled Clinical Trial in New Onset Juvenile Systemic Lupus Erythematosus Nephritis

This is a 5-year project, involving 185 partners from 46 countries (110 in 21 EU States and 75 in 25 extra-EU States), with a randomised clinical trials (RCT) in juvenile systemic lupus er...

Duke Lupus Registry

Lupus is a systemic autoimmune disease that can present with many varied symptoms, including joint pain, fevers, kidney disease, and rashes. Lupus can affect anyone, but it is most common...

Study of GL701 in Men With Systemic Lupus Erythematosus

Lupus flares and other symptoms associated with systemic lupus erythematosus (SLE) may be caused by a deficiency of dehydroepiandrosterone (DHEA). GL701 is an investigational new drug mea...

Phase II Pilot Study of Cytarabine for Refractory Systemic Lupus Erythematosus

OBJECTIVES: I. Evaluate the toxicity of cytarabine in patients with refractory systemic lupus erythematosus. II. Evaluate objective disease parameters, including serum complement levels...

A Study of AMG 557 in Adults With Systemic Lupus Erythematosus

This is a Phase 1, randomized, placebo-controlled, double-blind, dose-escalation study of repeat SC doses of AMG 557 in adults with Systemic Lupus Erythematosus.

Medical and Biotech [MESH] Definitions

A form of lupus erythematosus in which the skin may be the only organ involved or in which skin involvement precedes the spread into other body systems. It has been classified into three forms - acute (= LUPUS ERYTHEMATOSUS, SYSTEMIC with skin lesions), subacute, and chronic (= LUPUS ERYTHEMATOSUS, DISCOID).

A chronic form of cutaneous lupus erythematosus (LUPUS ERYTHEMATOSUS, CUTANEOUS) in which the skin lesions mimic those of the systemic form but in which systemic signs are rare. It is characterized by the presence of discoid skin plaques showing varying degrees of edema, erythema, scaliness, follicular plugging, and skin atrophy. Lesions are surrounded by an elevated erythematous border. The condition typically involves the face and scalp, but widespread dissemination may occur.

Antiphospholipid antibodies found in association with systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC;), ANTIPHOSPHOLIPID SYNDROME; and in a variety of other diseases as well as in healthy individuals. The antibodies are detected by solid-phase IMMUNOASSAY employing the purified phospholipid antigen CARDIOLIPIN.

INFLAMMATION of the PANCREAS. Pancreatitis is classified as acute unless there are computed tomographic or endoscopic retrograde cholangiopancreatographic findings of CHRONIC PANCREATITIS (International Symposium on Acute Pancreatitis, Atlanta, 1992). The two most common forms of acute pancreatitis are ALCOHOLIC PANCREATITIS and gallstone pancreatitis.

An antiphospholipid antibody found in association with systemic lupus erythematosus (LUPUS ERYTHEMATOSUS, SYSTEMIC;), ANTIPHOSPHOLIPID SYNDROME; and in a variety of other diseases as well as in healthy individuals. In vitro, the antibody interferes with the conversion of prothrombin to thrombin and prolongs the partial thromboplastin time. In vivo, it exerts a procoagulant effect resulting in thrombosis mainly in the larger veins and arteries. It further causes obstetrical complications, including fetal death and spontaneous abortion, as well as a variety of hematologic and neurologic complications.


Relevant Topics

Latest News Clinical Trials Research Drugs Reports Corporate
Pancreatitis Acute pancreatitis is inflammation of the pancreas caused by the release of activated pancreatic enzymes. Common triggers are biliary tract disease and chronic heavy alcohol intake.  Diagnosis is based on clinical presentation...

Latest News Clinical Trials Research Drugs Reports Corporate
Lupus is a complex and poorly understood condition that affects many parts of the body and causes symptoms ranging from mild to life-threatening. There are several types of lupus. The main types are: discoid lupus erythematosus drug-induced lu...

Latest News Clinical Trials Research Drugs Reports Corporate
Astroesophageal Reflux Disease (GERD) Barrett's Esophagus Celiac Disease Cholesterol Crohn's Disease Gastroenterology Hepatitis Hepatology Irritable Bowel Syndrome (IBS) Pancreatitis Peptic Ulcer Disease...