High procalcitonin is a marker of activity in Adult onset of still`s disease

Author(s): Fayed F, Eldeeb R, Tayel M, Abdelrahim S

Adult Onset Still's Disease (AOSD) is a rare inflammatory disorder characterized by the classical clinical trial of a daily high spiking fever, arthritis, and an evanescent rash. Diagnosis is clinical and requires exclusion of infectious, neoplastic, and other autoimmune diseases. High procalcitonin level in patient with Pyrexia of unknown origin is misleading of systemic infection and it is normalized after corticosteroid therapy.

A 22 years old Egyptian man presented by acute appendicitis associated with high spiking rash, an evanescent rash, and leukocytosis and underwent an appendectomy. One-week postoperative, He developed acute hepatitis and high procalctonin level complicated by severe hyperferritinemia as well as haemophagocytosis. After exclusion of sepsis and other causes of liver disease (infection, metabolic, autoimmune hepatitis, malignancy, lymphoma), the diagnosis of AOSD complicated by severe hepatitis and haemophagocytosis is more likely. Corticosteroid-induced clinical remission with a resolution of fever, rash and arthritis and induced rapid normalization of liver function and reduction of ferritin level. Cyclosporine was added as maintenance therapy but the patient developed polyuria, hypocalcemia, and hypercalciuria, after stopping cyclosporine, urine output and calcium level became normal by one week after cessation of cyclosporine. Severe hepatitis and very high ferritin could be the only manifestation of disease activity of AOSD. Therefore, monitoring of liver function, blood picture, and ferritin level even after resolution of clinical symptoms of AOSD. Prompt initiation of corticosteroid can improve liver function and blood picture and prevents liver failure, bone marrow suppression, and death. High procalctonin level was misleading of sepsis in AOSD. It is proven that the procalctonin level is elevated in autoimmune diseases especially ASOD and related to hyperferrtinemia.