Summary
Pneumatosis cystoides intestinalis (PCI) is an uncommon disorder usually associated with intestinal and pulmonary obstructive diseases, recent abdominal procedures and systemic illnesses. PCI has been reported in patients with systemic lupus erythematosus associated with intestinal vasculitis. We describe herein a patient with a month history of intermittent abdominal pain, diarrhoea, hyporexia, and weight loss who underwent intestinal resection for acute abdomen. Post-operatively she gave a three-month history of arthritis of the right knee, ankles and feet, arthralgia of the wrists, MCPs and shoulders. She also described weakness, weight loss, Raynaud's phenomenon, and a skin rash. Laboratory examination revealed an increased ESR, low haemoglobin and haematocrit, positive rheumatoid factor, a positive ANA with a speckled pattern, as well antibodies to DNA, SS-A and cardiolipin. The abdominal symptomatology especially pain, cramps and bouts of diarrhoea persisted after the surgery and became worse two months later. Abdominal X-ray showed distention of bowel with cyst formation in the wall of the entire colon. A diagnosis of PCI was made radiologically. The intestinal pathology was reviewed and vasculitis was identified. The patient received treatment with high dose prednisone with an excellent response; prednisone was progressively tapered and she has been asymptomatic without abdominal complaints or other symptoms for over a year.
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Alford, J.E., Galletti, G., Culver, G.J. Pneumatosis cystoides intestinalis. Am J Surg 1956, 92, 648–656.
Hoffman, B.I., Katz, W.A. The gastrointestinal manifestations of systemic lupus erythematosus: A review of the literature. Sem Arthr Rheum 1990, 4, 237–247.
Priest, R.J., Goldstein, F. Pneumatosis cystoides intestinalis: In: Gastroenterology. Ed.: Berk, J.E. Philadelphia Saunders 1985, 2674–2683.
Hanever, S.B., Kraft, S.C. Gastrointestinal manifestations of the vasculitis syndromes. In: Gastrointestinal Diseases Pathophysiology, Diagnosis, Management. Eds.: Sleisenger, M.H., Fondtran, J.S. Philadelphia Saunders 1989, 4525–4544.
Priest, R.J., Goldstein, F. Pneumatosis cystoides intestinalis. In: Gastrointestinal Diseases Pathophysiology, Diagnosis, Management. Eds.: Sleisenger, M.H., Fondtran, J.S. Philadelphia Saunders 1989, 2474–2483.
Christl, S.U., Gibson, G.R., Murgatroyd, P.R., Scheppach, W., Cummings. Impaired hydrogen metabolism in pneumatosis cystoides intestinalis. Gastroenterology 1993, 104 (2), 392–397.
Morrison, W.J., Siegelman, S.S. Pneumatosis intestinalis in association with connective tissue disease. South Med J 1976, 69, 536–1539.
Freiman, D., Chon, H., Bilaniuk, L. Pneumatosis intestinalis in systemic lupus erythematosus. Radiology 1975, 116, 563–564.
Zizic, T.M., Classed, J.N., Stevens, M.B. Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodose. Am J Med 1982, 73, 525–528.
Pollack, V., Grove, W.J., Kart, R.M., Muenhrcke, R.C., Pirani, C.L., Steck, I.E. Systemic lupus erythematosus simulating acute surgical condition of the abdomen. N Engl J Med 1958, 259, 258–266.
Tak, P.P., Van Duinen, C.M., Bun, P., Eulderink, F., Kreuning, J., Gooszen, H.G., Lamers, C.B.H.W. Pneumatosis cystoides intestinalis in intestinal pseudoobstruction. Resolution after therapy of metronidazole. Dig Dis Sciences 1992, 37(6), 949–954.
Atsumi, T., Sagawa, A., Watanabe, I., Amasaki, Y., Katsumata, K., Nakabayashi, T., Mukai, M., Fujisaku, A., Nakagawa, S. Pneumoperitoneum without perforation of the gastrointestinal tract in a patient with systemic lupus erythematosus. Ryamuchi 1991, 31(4), 398–404.
Carbajal-Rodriguez, L., Perea-Martínez, A., Oldak-Skvirsky, D., Loredo-Abdalá, A., Reynes-Manzur, J.N. Complicaciones abdominales en el lupus eritematoso sistémico. Bol Med Hosp Infant Mex. 1991, 8(3), 178–184.
Decrop, E., Ponette, E., Baert, A.L., Verberckmoes, R., Kerremans, R., Geboes, K. Pre-operative radiological diagnosis of acute necrotizing enteritis in systemic lupus erythematodes. JBR-BTR 1990, 73, 31–35.
Pruitt, R.E., Tumminello, V.V., Reveille, J.D. Pneumatosis cystoides intestinalis and benign pneumoperitoneum in patient with antinuclear antibody negative systemic lupus erythematosus. J Rheumatol 1988, 15, 575–1577.
Laing, T.J. Gastrointestinal vasculitis and pneumatosis intestinalis due to systemic lupus erythematosus: successful treatment with pulse intravenous cyclophosphamide. Am J Med 1988, 85, 55–558.
Derksen, O.S. Pneumatosis intestinalis in a female patient with systemic lupus erythematosus. Radiologia Clin 1979, 47, 334–339.
Binstandt, D.H., L'Heureux, P.R. Pneumatosis cystoides intestinalis in childhood systemic lupus erythematosus. Minn Med 1977, 60, 408–409.
Kleinman, P., Meyers, M.A., Abbott, G., Kazam, E. Necrotizing enterocolitis with pneumatosis intestinalis in systemic lupus and polyarteritis. Radiology 1976, 121, 595–598.
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Cabrera, G.E., Scopelitis, E., Cuellar, M.L. et al. Pneumatosis cystoides intestinalis in systemic lupus erythematosus with intestinal vasculitis : Treatment with high dose prednisone. Clin Rheumatol 13, 312–316 (1994). https://doi.org/10.1007/BF02249034
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DOI: https://doi.org/10.1007/BF02249034