Adult-onset Still's disease evolving with multiple organ failure and death: A case report and review of the literature

Abstract

BACKGROUND

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease, which is characterized by daily fever and arthritis, with an evanescent rash and neutrophilic leukocytosis. To date, there has been no definite laboratory or imaging test available for diagnosing AOSD; the diagnosis is one of exclusion, which can be very challenging. In particular, AOSD patients may experience different complications affecting their clinical picture, management, and prognosis. The treatment of AOSD remains largely empirical and involves therapeutic agents.

CASE SUMMARY

We report the case of a 36-year-old woman who presented with fever, red rash, arthralgia, and sore throat. Her serum ferritin level and white blood cell count were markedly elevated, and the first diagnosis 22 years prior was "juvenile rheumatoid arthritis of systemic type". The patient was treated with prednisone, sulfasalazine, methotrexate, and leflunomide. After remission of her symptoms, the patient stopped taking the medications, and the disease recurred. Ultimately, the patient was diagnosed with adult-onset Still's disease. Relapse occurred several times due to self-medication withdrawal, and an interleukin-6 antagonist (tocilizumab/Actemra) was administered to control the disease. Recently, she was hospitalized because an incision did not heal, and the patient suddenly developed high fever and diarrhea during hospitalization. The patient's disease progressed violently and quickly developed into macrophage activation syndrome, disseminated intravascular coagulation, shock, and multiple organ failure. The patient had sudden cardiac arrest, and she died despite emergency rescue efforts.

CONCLUSION

AOSD patients need regular follow-up in the long-term treatment process, and must press formulary standard medication, and do not voluntarily withdraw or reduce the dose. Otherwise it may cause disease back-and-forth or serious life-threatening complications. Meanwhile, strict management of trauma, infections, tumors, and other diseases may contribute to improved outcomes in patients with complications.

Keywords: Adult-onset Still's disease; Macrophage activation syndrome; Disseminated intravascular coagulopathy; Multiple organ failure; Death; Case report

Core Tip: Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disease, and the lack of disease-specific symptoms and laboratory markers hinders the diagnosis and assessment of its progression. More importantly, the treatment of AOSD remains largely empirical, with a lack of controlled clinical trials. Few cases of the disease with multiple complications have been reported. The present case highlights the characteristics of AOSD and severe complications and shares our experience in its diagnosis and treatment to provide experience for the effective recognition and treatment of this disease. The authors also note the link between the biopsychosocial model and autoimmune diseases.