Multiorgan failure in an adolescent with null complement hemolytic activity, systemic inflammatory syndrome and autoimmune manifestations - case description and approaches to differential diagnosi

The article describes a case of acute renal failure (ARF) in a 16 years old adolescent. Renal failure arose on the background of severe systemic inflammatory syndrome with pronounced hemorrhagic rash, multiorgan failure in combination with signs of thrombotic microangiopathia, disseminated intravascular coagulation (DIC), cryoglobulinemia. Persistent null complement hemolytic activity was further revealed in the patient. The differential diagnosis concerned meningococcemia, sepsis, systemic vasculitis, including cryoglobulinemic vasculitis, thrombotic microangiopathies (TMAs). Along with diagnostic search intensive complex therapy was carried out, including application of wide spectrum antibiotics, plasma exchanges, methylprednisolone pulses, infusions of human immunoglobulin. The therapy resulted in the improvement of the patient’s general condition, subsiding of inflammation, stable disappearance of TMA/DIC symptoms. Nephrosclerosis and chronic kidney disease (CKD) stage 4 has developed in the outcome of the acute disease. After careful analysis of the disease manifestation and course during acute period, laboratory examination, follow-up observation we came to a conclusion that the teenager has hereditary terminal complement deficiency predisposing him to recurrent meningococcemia. The last acute episode was complicated by multiorgan failure, ARF, DIC and resulted in nephrosclerosis. Further therapy in our patient must be targeted to the slowing of CKD progression and to the prevention of invasive meningococcal infection by vaccination with a tetravalent meningococcal vaccine.

острая почечная недостаточность, криоглобулинемия, комплемент, менингококцемия, тромботическая микроангиопатия, acute renal failure, cryoglobulinemia, thrombotic microangiopathia, complement, meningococcemia, thrombotic microangiopathia

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