Case Report: Intracranial hypertension in an adult-onset Still’s disease patient initially presented with prolonged fever

This article describes the case of a 19-year-old woman who presented with prolonged fever, positive antinuclear antibodies (ANA) and splenomegaly. Pulmonary infiltrates were discovered and the patient was treated for community-acquired pneumonia, with no clinical amelioration. A more thorough evaluation was subsequently made, revealing elevated serum IgE and IgG4 levels and negative ANA tested by the hospital’s laboratory with two methods. During hospitalization thrombocytopenia, liver function test impairment, and evanescent rash during some febrile episodes developed. Vomiting also presented without any concomitant symptoms or signs; a funduscopic examination was consequently ordered, showing bilateral papilledema. Brain imaging was totally normal but a lumbar puncture revealed elevated opening pressure and lymphocytic pleocytosis along with low cerebrospinal fluid lactate dehydrogenase (CSF LDH). The patient was empirically treated with antimicrobials, dexamethasone, and acetazolamide and had immediate clinical and laboratory improvement. Diagnostic workup, however, was negative for an infectious agent; antimicrobials were ceased but the patient continued to improve. Adult-onset Still’s disease (AOSD) was considered as the working diagnosis because the patient fulfilled Yamaguchi criteria, responded to corticosteroids, and an alternative diagnosis was lacking. Nevertheless, because of the patient’s atypical features a trial to discontinue dexamethasone was undertaken, leading to immediate recurrence; the possibility of a self-limiting viral illness was excluded. Thrombocytopenia was attributed to hemophagocytic lymphohistiocytosis (HLH) that complicated AOSD. Corticosteroid reinitiation combined with methotrexate fully controlled all clinical and laboratory parameters. One month later papilledema had disappeared and the patient remained symptom-free even without acetazolamide. To our knowledge, this is the first report in the literature of an AOSD case presenting intracranial hypertension without cerebral imaging abnormalities and neurological or meningeal symptoms and signs, as well as with the initial observation of serum IgG4 elevation. A classic regimen combined with acetazolamide led to a positive outcome.