Neck Pain With Abdominal Distention in a Patient With Systemic Lupus Erythematosus

Abstract

Question: A 13-year-old boy admitted for systemic lupus erythematosus (SLE) presented mild neck pain, throat pain, and abdominal distention. The SLE was diagnosed 6 months previously, and he received steroid pulse therapy, tacrolimus, and hydroxychloroquine for its treatment. Prolonged steroid therapy was required for his refractory status. Three months previously, he had an episode of right-sided cervical lymphadenitis caused by Staphylococcus aureus. He had no history of trauma. The patient had a good appetite and denied having symptoms such as dyspnea, cough, abdominal pain, nausea, vomiting, diarrhea, and bloody stool. A physical examination revealed right-sided neck pain with mild subcutaneous emphysema. No abnormalities were observed in his throat. Abdominal distention without tenderness was found. Laboratory tests were negative for elevated inflammatory markers, hemoconcentration, and lactic acidosis. Radiography demonstrated linear air leaks in the neck, multiple air leaks along the trachea, free air, and a curvilinear collection of gas within the bowel wall (Figures A and B). Given these physical and radiological findings, what is the most likely diagnosis? See the Gastroenterology website (www.gastrojournal.org) for more information on submitting to Gastro Curbside Consult. Additional contrast computed tomography performed for a possible intestinal perforation found pneumomediastinum (Figures C and D), massive free air in the abdomen, and extensive air accumulation within a thickened bowel wall (Figures E, F, and G). Based on the mild clinical appearance and laboratory findings, pneumatosis cystoides intestinalis and pneumomediastinum without intestinal perforation were diagnosed. Careful observation with oxygen therapy, antibiotic administration, bowel rest, and total parenteral nutrition were begun, and the steroid dosage was gradually reduced. As a result, the patient recovered in 2 weeks without surgical intervention. Based on the clinical course and follow-up radiography and computed tomography findings, we concluded that his condition was idiopathic pneumatosis cystoides intestinalis with pneumomediastinum. Idiopathic pneumatosis cystoides intestinalis is characterized by the presence of gas within the intestinal submucosa or subserosa. The condition may be asymptomatic or present mild, nonspecific clinical manifestations, such as abdominal distention, discomfort, nausea, diarrhea, and anorexia, which may lead to an underdiagnosis of this condition.1Heng Y. Schuffler M.D. Haggitt R.C. et al.Pneumatosis intestinalis: a review.Am J Gastroenterol. 1995; 90: 1747-1758PubMed Google Scholar The risk factors of pneumatosis cystoides intestinalis have not been definitively identified, but prolonged administration of steroids or immunosuppressive agents is thought to be involved in its onset.2Mizoguchi F. Nanki T. Miyasaka N. Pneumatosis cystoides intestinalis following lupus enteritis and peritonitis.Intern Med. 2008; 47: 1267-1271Crossref PubMed Scopus (23) Google Scholar Atrophy of submucosal tissue caused by prolonged steroid therapy may also occur. Gastrointestinal involvement in SLE takes various forms,3Frittoli R.B. Vivaldo J.F. Costallat L.T.L. et al.Gastrointestinal involvement in systemic lupus erythematosus: a systematic review.J Transl Autoimmun. 2021; 4100106Crossref PubMed Scopus (12) Google Scholar making the correct diagnosis particularly important. The clinical presentations of our patient were not caused by complications of SLE because his clinical SLE activity was under control with prednisolone, tacrolimus, and hydroxychloroquine. The fact that reducing the prednisolone dosage did not exacerbate his condition corroborated our diagnosis. We herein described a case of massive pneumatosis cystoides intestinalis with pneumomediastinum accompanied by mild symptoms. Appropriately evaluating the symptoms, even when they are mild, is necessary in patients with prolonged steroid therapy.