Patient with Undetermined Fulminant Systemic Inflammatory Response Syndrome: Clinical Implications from Case Report

Systemic inflammatory response syndrome (SIRS) is a non-specific immune system stimulation that has different sources. An infection is a common etiology of SIRS forming sepsis. Sepsis and septic shock are major healthcare problems affecting millions of people around the world each year, killing one in four (and often more) and increasing in incidence. However, severe SIRS can occur in other rare conditions as well; particularly in connective tissue diseases: adult-onset Still’s disease (AOSD), systemic lupus erythematous (SLE) or may arise from pathological immune system activation in hematological diseases: hemophagocytic limphohistiocytosis (HLH) and macrophage activation syndrome (MAS) . Both sepsis and above mentioned diseases, collectively called hyperferritinemic syndromes, are marked with similar symptoms, pathogenesis and present simultaneous hyperferritinemia. Of the great importance is the fact that these autoimmunological and hematological diseases, although very rare in incidence, imitate sepActa Medica Mediterranea, 2017, 33: 569