BACKGROUNDAdult-onset Still's disease (AOSD) remains a rare multisystemic autoinflammatory disorder of unknown etiology, including the archetype of nonfamilial, or sporadic (Fautrel 2008, McGonagle and McDermott 2006, Peckham et al. 2017) and difficult diagnosis because of the wide range of differential diagnoses (Gerfaud-Valentin et al. 2014a). The disease may be life-threatening, leading to death because of severe complications, including macrophage activation syndrome (MAS), cardiopulmonary system involvement, and fulminant hepatitis (Gerfaud-Valentin et al. 2014a). MAS occurs in about 12 to 15% of AOSD cases, being an important manifestation which can limit life expectancy (Gerfaud-Valentin et al. 2014b, Hot et al. 2010. CASE REPORTA 48-year-old woman was hospitalized on February 17, 2022 with fever that started one month earlier associated with pruritic confluent macropapular rash on upper limbs, trunk and back and oligoarthritis in hands, knees and ankles. Serologies tests were made for viral hepatitis, HIV, syphilis, dengue, chikungunya, zika, parvovirus, aspergillosis, histoplasmosis and paracoccidioidomycosis; results were negative, endocarditis was excluded. Thus, the diagnosis of Still's disease was confirmed by the clinical presentation of the patient and the laboratory results: leukocytosis (26,740 μL), hyperferritinemia (17,150 μL), nonreactive autoantibody and increased liver enzymes. The patient was treated with oral prednisone 1 mg/kg to attenuate the systemic condition. After changed the laboratory to pancytopenia and fever daily, it was suggestive for MAS and a red bone marrow biopsy confirming which showed granulocytic hyperplasia and hemophagocytic syndrome. Furthermore, the immunophenotypic study identified T lymphocytosis at the expense of CD8 T lymphocytes, without phenotypic and polyclonal aberrations to TRBC1, in addition to the absence of CD25, which may correspond to a condition secondary to an inflammatory/infectious process. After 10 days, she returned with daily fever and worsening laboratory tests, which improved with pulse therapy of methylprednisolone 1 g/day for 3 days. CONCLUSIONAOSD has an unknown etiology and the diagnosis is clinical, being realized by exclusion. In this report we present a patient who envolved with pancytopenia and fever had the disease with MAS and was refractory to treatment with oral corticosteroids, just pulse therapy was effective. This case is relevant once it shows a disease that is an important cause of fever with obscure origin, which can be fatal if not properly diagnosed and treated.
Behçet's disease is a systemic vasculitis that manifests in outbreaks and can affect the skin, mucosa, joints, vessels, eyes, and nervous and gastrointestinal systems. Neurological involvement is present in 5% of patients with this disease. It is classified into parenchymatous, which comprises the cerebral form, and nonparenchymal, related to cerebral venous thrombosis and intracranial hypertension. CASE REPORTA 40-year-old woman, former drug user, hospitalized with convulsive crisis, fever and severe holocranial headache, was diagnosed with bacterial meningitis, liquor with gram-positive coconuts. She was treated with ceftriaxone and vancomycin. During hospital evolution, she presented hemiparesis of the left hemibody without etiological definition. She was discharged from hospital with outpatient follow-up with a neurologist, who requested a brain nuclear magnetic resonance, which showed poorly delimited hypodense areas, involving the bridge, midbrain and nucleocapsular regions, expanding them. In view of the suspected examination of central nervous system vasculitis, she was referred for emergency hospitalization and pulse therapy with methylprednisolone 1 g for 5 days was started. It evolved with significant recovery of the strength of the left hemibody, being redirected to follow-up with outpatient neurologist. Due to delay in outpatient consultations, after 8 months, the patient presented hemiplegia of the right hemibody, paralysis of the VI cranial nerve to the right, central facial paralysis, dysarthria, dysphagia and ataxia. During the systems review, recurrent oral and genital ulcers were observed. Pulse therapy was initiated with methylprednisolone 1 g for 5 days and cyclophosphamide 1 g, due to the diagnosis of Behçet's disease with central nervous system involvement. After pulse therapy, she presented significant improvement of the neurological picture, with partial recovery of strength and partial improvement of dysphagia. She maintained cyclophosphamide with monthly infusions. After the sixth dose, a new cranial magnetic resonance imaging revealed great improvement in brain stem injury. CONCLUSIONWe conclude that effective patient care, based on physical examination and clinical history, is extremely important. Behçet's disease presents a varied form of manifestations, including the neurological form, which is related to high morbidity and mortality rates.
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