DOMINIK BURSA1, 2, MARCIN PACIOREK1, 2, DAWID POROWSKI1, 2, AGNIESZKA BEDNARSKA1, 2
1 Department for Adults' Infectious Diseases, Medical University of Warsaw, Warsaw, Poland - 2Hospital of Infectious Diseases, Warsaw, Poland
Introduction: Severe systemic inflammatory response syndrome (SIRS) is commonly related to sepsis but rare autoimmunolo- gical or hematological diseases can provide similar conditions: adult-onset Still's disease (AOSD), systemic lupus erythematous (SLE), hemophagocytic limphohistiocytosis (HLH), macrophage activation syndrome (MAS). These diseases cause cytokine storm and trigger extreme hyperferritinemia, thus they are called hyperferritinemic syndromes.
Case presentation: We present a case of a 55-year-old female who had fever, rash and arthralgia. She was admitted to the Hospital of Infectious Diseases in Warsaw with SIRS initially attributed to bacterial infection and suspicion of sepsis. During diagno- stic management the patient developed overwhelming SIRS with hyperferritinemia. Broad-spectral antibiotics with short course of steroids were administered for the suspected autoimmunological syndrome. The response to treatment was quick. None syndromes met diagnostic criteria. After vancomycin infusion the second exacerbation appeared with acute kidney injury. Methylprednisolone pulses provided improvement. The patient met HLH criteria a few days after the second exacerbation. Slow, complete remission was achieved with immunosuppressive therapy (steroids and methotrexate). MAS was recognized due to AOSD.
Conclusion: The clinical presentation of patients with SIRS is nonspecific. They require thorough assessment because similar symptoms may occur in other several non-infectious conditions, such as autoimmune or hematological disorders.
sepsis, systemic inflammatory response syndrome, hemophagocytic limphohistiocytosis, macrophage activation syn- drome, adult-onset Still's disease, red man syndrome