Case presentation
We present a case report of a 17-year-old girl with problems that
started a year ago in the form of multiple bruises on the whole body and
bleeding from the gums, who was diagnosed with ITP (Immune
thrombocytopenic purpura) and treated with corticosteroids and IVIG
(Intravenous immune globulin). The patient had no problems until two
months ago. Until she was hospitalized due to leukopenia and seizures.
After investigations, proteinuria of 1100 mg/dL, lymphopenia and
leukopenia, positive ANA and anti-ds DNA and reduced complement was
detected (Table1). During hospitalization, due to abdominal pain and
swelling and Shifting Dullness in the examination, CT scan of the
abdomen and pelvis with oral and intravenous contrast is also performed
for the patient (Figure1). Necrotizing pancreatitis is reported with
involvement of less than 30% of the pancreas in addition to the
presence of abundant free fluid in the abdomen and pelvis and bilateral
pleural effusion. According to the seizure, MRI/MRV was performed for
patient, MRI reported was “Diffuse hyperintensity is seen at
subcortical white matter of high frontal lobes and parieto-occipital
lobes bilaterally, is reported and also MRV is reported as normal. In
order to investigate other causes of pancreatitis, MRCP was performed
for the patient, which was reported as a normal. Finally, according to
the history of thrombocytopenia, leukopenia and lymphopenia,
proteinuria, seizures, positive ANA and Anti dsDNA and reduced
complement, SLE was diagnosed and the patient was treated with
methylprednisolone pulse for 3 days. And then she was treated with
prednisolone at a dosage of 1 mg/kg and cyclophosphamide puls, with a
dose of 500 mg. Other potential causes of pancreatitis were
investigated, but no pathological findings were found. The patient’s
pancreatitis was justified in the context of SLE. During her
hospitalization, she received routine treatment for pancreatitis.
Currently, during the follow-up, cytopenia and ascites have improved,
seizures have not recurred, and 24-hour proteinuria is at the level of
566 mg/dl. The patient is received the third dose of cyclophosphamide
and 20 mg of prednisolone and 200 mg of hydroxychloroquine daily.
Discussion
In this case, we reported a 17-year-old person who constellation of her
symptoms raises the suspicion of both SLE and necrotizing pancreatitis.
Pancreatitis is a medical condition characterized by the inflammation of
the pancreas; Symptoms of pancreatitis typically involve severe
abdominal pain.
Based on the nature, duration, and characteristics of the inflammation
affecting the pancreas, pancreatitis classified into 3 groups: Acute
Pancreatitis (AP), Chronic Pancreatitis, Necrotizing Pancreatitis.
Necrotizing pancreatitis is a severe form of AP characterized by the
death of pancreatic tissue account for relatively high morbidity and
mortality rate (3).
AP, as a result of SLE is more common rather than necrotizing
pancreatitis (4). While AP manifests more frequently than necrotizing
pancreatitis in patients with SLE, timely medical intervention in the
acute inflammatory stage may attenuate progression to pancreatic
necrosis in this population (5). SLE can affect small blood vessels,
resulting in inflammation known as vasculitis (6). When this
inflammation occurs in the blood vessels supplying the pancreas, it can
restrict blood flow to the organ. This impaired perfusion deprives the
pancreatic tissue of oxygen and nutrients. The ischemia and resulting
dysfunction may then trigger the onset of AP in susceptible individuals
(7). Beyond the pancreas, vasculitis damage from SLE can impact organs
throughout the body.
The pathophysiological underpinnings of SLE and pancreatitis encompass
intricate molecular, immunological, and genetic interactions (8). SLE
has a substantial genetic component, which engenders immune perturbation
aberrant B and T lymphocyte reactivity, cytokine disproportion, and
epigenomic alterations critically regulate disease propagation. The
hallmark interferon-alpha signaling cascade overload characterizes SLE
immunopathology.
In comparison, pancreatitis molecular factors like genetic controllers
of pancreatic enzymes, inflammatory mediators, and autoantibodies
(notably in autoimmune pancreatitis) are operant. Premature pancreatic
digestive enzyme activation, oxidative injury, and genetic liability
collectively fuel pancreatitis’ development.
Despite differing specifics, SLE and pancreatitis share an
immunoregulatory breakdown and inflammation as a cardinal unifier. Both
conditions exhibit the loss of self-tolerance, enabling autoimmune
injury and tissue damage(9). Comprehending the molecular intricacies is
vital to further enable targeted therapeutics and superior handling of
these compound autoimmune and inflammatory illnesses. Recent studies
have been shown that patients with SLE often have higher rates of
metabolic conditions like diabetes and hypertriglyceridemia (10). These
are also independent risk factors for developing pancreatitis. Also,
Cytokine imbalances due to overactive immune systems in SLE may promote
inflammation in various organs, including theoretically the pancreas.
While numerous studies and reports extensively cover AP, there is a
scarcity of literature and research focusing on necrotizing pancreatitis
due to its rarity.
To the best of our knowledge, although there are several case reports of
lupus-associated AP, only one case of lupus-associated necrotizing
pancreatitis has been reported. In July 2014, a 37-year-old woman of
African descent, previously diagnosed with SLE, presented with sudden
abdominal pain, accompanied by nausea and vomiting. Despite being under
ongoing treatment with mycophenolate mofetil (MMF), prednisolone,
hydroxychloroquine (HCQ), and belimumab, she encountered recurrent
episodes of AP. Diagnostic assessments, including laboratory tests and
imaging, verified the presence of pancreatitis with 20% necrosis. The
medical team suspected SLE-induced pancreatitis, prompting the
administration of high-dose steroids. This intervention yielded
favorable responses in both clinical symptoms and biochemical markers.
Following this, the patient’s ongoing care plan incorporated rituximab
for enhanced disease management. A subsequent follow-up examination
indicated notable improvement in the pancreatitis condition. This case
highlights the complexities of addressing complications associated with
SLE and underscores the effectiveness of tailored approaches, including
high-dose steroids and rituximab, in managing AP and achieving positive
patient outcomes (11).
Also, retrospective studies indicate that 0.9% to over 5% of SLE
patients suffer from AP. Among 264 SLE patients studied, predominantly
female with an average age of 31.4 years, abdominal pain was a
consistent symptom. AP was more common in those with shorter disease
duration, high activity scores, and multiorgan involvement. Diagnosis as
”idiopathic” SLE-related AP was based on guidelines, excluding other
causes. Managing SLE-related AP is challenging due to the lack of a
standardized approach, and the use of glucocorticoids is debated due to
potential complications. Monitoring serum lipase levels after high-dose
steroids is suggested. Some studies suggest a positive outcome with
plasma exchange, and the occurrence of AP in SLE may signal an
association with macrophage activation syndrome, with reported mortality
rates of up to one third of cases (12).