Purpose It is known that morbidity and mortality risks are higher in systemic lupus erythematosus-related (SLE) intestinal pseudo-obstruction (IPO) cases. Therefore, it is crucial to recognize and treat these cases promptly. Here, we present a case of SLE-related IPO in a 41-year-old female patient.
Methods Laboratory tests and radiodiagnostic methods including magnetic resonance imaging (MRI), computed tomography (CT), and plain radiogaphy were utilized for diagnosis.
Results The patient who had polyarthritis, malar rash, and photosensitivity first presented to our clinic six years ago. She had a normal capillary pattern in nail fold capillaroscopy and no sclerodactyly. She also had ANA positivity and was diagnosed with SLE. The patient discontinued drug therapy and clinical follow-ups three years after the diagnosis. Then, she presented to our outpatient rheumatology clinic one month ago with nausea, abdominal pain, bloating, and bilateral lower extremity paresthesia. The patient was not able to walk without support, but muscle strength was normal in all extremities. There were no clinical signs of SLE activation except neurological complaints. ANA was positive at 1/640 dilution with nucleolar staining pattern. Complement levels were low. Anti-ds-DNA and anti-topoisomerase I were negative. CRP level was 25 mg/L. The rest of the lab parameters were normal. No pathological signs were detected in cranial MRI, cranial MR angiography, and spinal MRI. Cerebrospinal fluid had protein level 200 mg/dL, <5 white blood cells. No viral pathogen was found. The patient was given pulse steroid. After three doses of 1g prednisolone, the patient stated she had constipation and was flatulent for ten days. Subsequently, air-fluid levels were seen in the erect abdominal radiography (Figure 1). Her complaints were resolved by nasogastric tube insertion. Ileus was confirmed by CT. IPO was the possible diagnosis for an SLE patient with this clinical presentation. 1g cyclophosphamide was administered after the seventh dose of corticosteroids. Extra three doses of corticosteroids were given because the patient was not improving clinically. Eventually, rituximab was given two weeks after cyclophosphamide and the patient recovered. Major complaints in SLE-related IPO cases are abdominal pain, bloating, nausea, constipation, and diarrhea. The small intestine is usually affected. Intestinal perforation is a rare but life-threatening complication.1 Gastrointestinal symptoms are not specific to IPO, thus diagnosis may not be straightforward. The presence of abdominal symptoms at first evaluation, air-fluid levels in abdominal plain radiography, and absence of any prominent reason explaining the condition led us to diagnose IPO.
Conclusion Early diagnosis and appropriate timing to begin corticosteroid treatment play a crucial role to have better outcomes. We urge the clinicians to consider IPO in SLE patients who are admitted to the hospital with gastrointestinal symptoms.
Wang JL, Liu G, Liu T, Wei JP. Intestinal pseudo-obstruction in systemic lupus erythematosus: a case report and review of the literature. Medicine (Baltimore). 2014 Dec;93(29):e248.
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