Case presentation:
A 37-year-old Caucasian female, presented to the emergency room with abdominal pain, distension and vomiting. Symptoms were developed two months ago with reported diarrhea and weigh loss. She already was treated symptomatically but no diagnosis was done and her condition went worse as she developed sub-acute bowl obstruction signs. She had no other previous significant personal history except polyarthralgia. No concomitant medication was taken. At presentation, physical examination revealed marked abdominal distension, diffuse tympanism with tenderness without rebound tenderness. There was no fever and vital signs were stable. Neurological and cutaneous examinations were normal. Abdominal X-ray imaging showed several air-fluid levels in small and large bowel (figure 1). Investigations blood tests indicated normochromic anemia of 9 g/dl with reticulocytes count of 27058/mm3, leucopenia of 3500/mm3, lymphopenia of 500/mm3 and normal platelets count. The blood chemistry showed: hypokaliemia of 2.6 mmol/L, sodium of 142 mmol/L, albumin of 2.8 g/dL and normal lipase level. Thyroid function and hepatic tests were normal and there was no inflammatory syndrome. Haemolysis markers were negative. Dipstick urinalysis showed normal results. Abdominal CT scan revealed dilated loops in the small bowel, segmental thickened small intestinal walls without any mechanical obstacle (figure 2), bilateral pleural, pelvic, abdominal effusion and not marked bilateral hydronephrosis. The patient was diagnosed with intestinal pseudo-obstruction and nasogastric tube was placed. Antibiotics against bacterial growth, parenteral nutrition and potassium supplementation were prescribed, as well as intravenous perfusion of erythromycin with moderate improvement of symptoms. Colonoscopy was performed showing segmental thickened and edematous appearance of colonic mucosa with no specific findings at histopathology. Upper endoscopy was normal as well as histological examination of duodenal biopsy. Considering young age, female patient and history of polyarthralgia, we investigated systemic diseases.
Immunological tests showed positive anti-nuclear antibody (ANA) at a titer of 1:400, anti-dsDNA antibodies, anti-SSB and anti-SSA. Direct Coombs’ test was positive in the absence of hemolytic anemia.
Thus, she was diagnosed with SLE-related IPO as she met 5 criteria of Systemic Lupus International Collaborating Clinics (SLICC) classification criteria: leucopenia <4000/mm3 / lymphopenia <500/mm3, pleural effusion, positive Coombs’ test without hemolytic anemia, ANA, anti-dsDNA antibody. Subsequently, we started corticosteroid therapy. The patient received 3 days pulses of methylprednisolone (1000 mg per day) followed by oral prednisolone 1mg/kg/day associated with Hydroxychloroquine (400mg/day). Her condition had rapidly improved significantly with disappearance of abdominal symptoms, vomiting and air-fluid levels at X ray. The patient was discharged four days after starting corticosteroids and was symptom-free by the last time she was reviewed one month later.