Intestinal pseudo-obstruction with life-threatening hypokalaemia in a patient with adult-onset Still’s disease
  • Pierre Rossignon
    Department of Internal Medicine, Moliere-Longchamp Hospital, Brussels, Belgium
  • Alain Soupart
    Research Unit for the Study of Hydromineral Metabolism, Department of Internal Medicine, Erasmus University Hospital, ULB, Brussels, Belgium

Keywords

Adult-onset Still’s disease, hypokalemia, intestinal pseudo-obstruction

Abstract

Adult-onset Still’s disease (AOSD) is a rare autoinflammatory disorder that can lead to a cytokine storm, causing a range of symptoms. Acute intestinal pseudo-obstruction is another rare condition that results in intestinal obstruction without anatomical cause. Although the two conditions are rarely reported together, we present the case of a 62-year-old male who developed acute intestinal pseudo-obstruction in the context of an AOSD flare. This led to severe hypokalaemia and a critical condition. Other symptoms included a high-spiking fever lasting for weeks, polyarthralgias and a typical salmon-coloured rash. After ruling out other potential causes, the patient was diagnosed with AOSD. Our findings suggest that the cytokine storm associated with this disease triggered the acute intestinal pseudo-obstruction and life-threatening hypokalaemia, establishing a causal relationship.
Only four other cases of AOSD complicated by intestinal pseudo-obstruction have been reported, and this is the first to present with life-threatening hypokalaemia. This case serves as a crucial reminder that, despite being a diagnosis of exclusion, Still’s disease should be considered as a potential cause of intestinal pseudo-obstruction, as prompt recognition and treatment of the underlying cause is crucial in managing this potentially life-threatening condition.

VIEW THE ENTIRE ARTICLE

References

  • Tomaras S, Goetzke CC, Kallinich T, Feist E. Adult-onset Still’s Disease: clinical aspects and therapeutic approach. JCM 2021;10:733.
  • Batke M, Cappell MS. Adynamic ileus and acute colonic pseudo-obstruction. Med Clin North Am 2008;92:649–70.
  • Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19:424–30.
  • Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Sève P. Adult-onset Still’s disease. Autoimmun Rev 2014;13:708–22.
  • Reginato AJ, Schumacher HR, Baker DG, O’Connor CR, Ferreiros J. Adult onset Still’s disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum 1987;17:39–57.
  • Shinohara T, Hidaka T, Matsuki Y, Suzuki K, Ohsuzu F. Calcinosis cutis and intestinal pseudoobstruction in a patient with adult onset Still’s disease associated with recurrent relapses of disordered coagulopathy. Intern Med 1999;38:516–20.
  • El Younsi S, Perlemuter G, Clerc D, Buffet C, Pelletier G. Maladie de Still et pseudo-obstruction intestinale [Still’s disease and intestinal pseudo-obstruction]. Gastroenterol Clin Biol 2004;28:309–10.
  • Hori H, Yabe H, Fukuchi T, Sugawara H. A woman with adult-onset Still’s disease and acute intestinal pseudo-obstruction. Clin Case Rep 2021;9:153–7.
  • Manzotti C, Castellani M, Murgo A, Basilisco G. Duodenojejunal inflammation causing chronic vomiting in adult-onset Still’s disease. BMJ Case Rep 2023;16:e252345.
  • Kaplanski G. Interleukin-18: biological properties and role in disease pathogenesis. Immunol Rev 2018;281:138–53.
  • Fautrel B. Adult-onset Still disease. Best Pract Res Clin Rheumatol 2008;22:773–92.

    • Views: 288
    HTML downloads: 45
    PDF downloads: 293

    Published: 2023-05-30
    Issue: 2023: Vol 10 No 6 (view)

    How to cite:
    1.
    Rossignon P, Soupart A. Intestinal pseudo-obstruction with life-threatening hypokalaemia in a patient with adult-onset Still’s disease. EJCRIM 2023;10 doi:10.12890/2023_003887.