Clinical case of a patient with adult-onset still⇔s disease ⇓ A diagnostic challenge or a late diagnosis?

Abstract

Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disease with poorly understood etiology, that features quotidian spiking fevers, polyarthritis and distinctive salmon-colored maculopapular rash. Extremely high levels of ferritin are present in most patients, making it an important laboratory test for diagnosis. AOSD is a crucial diagnostic and therapeutic problem, which in many cases remains unrecognized due to the rarity in the population and the diverse clinical features. We present the clinical case of a 34-year-old woman with a first manifestation – maculopapular rash on the dorsal surface of the wrist joints and on the knee joints. At this time the patient has first-time pregnancy in the 6th gestational week. During a gynecological examination, intrauterine fetal death was found, which necessitated premature termination of pregnancy in the 8th gestational week. At the same time the patient was subfebrile and a couple days later she reported fever, reaching up to 39°C. The patient was hospitalized in the Clinic of Rheumatology with a fever of unknown origin up to 39°C, polyarthralgia, polymyalgia, maculopapular rash on the chest, leukocytosis, thrombocytosis, increased LDH-, ferritin-, GGT-acute phase reactant levels, microcytic hypochromic anemia, negative immunological findings (anti-MCV, anti- CCP, ANA-screen, ANA-blot, anti-dsDNA, ANCA, anti-MPO, anti-PR3, aCL, anti-B2GPI, anti-prothrombin), negative Quantiferon test. The patient was diagnosed with AOSD and started therapy with Methylprednisolone 60mg/daily, when she got a persistent afebrile condition and significant improvement in the clinical status and laboratory parameters.