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.
BACKGROUNDCyclophosphamide (CYC) is a nitrogen mustard alkylating agent with potent immunomodulatory and immunosuppressive properties. Its cardiotoxicity effects, however, are reported to be dose-dependent, especially at doses above 100 mg/kg. Furthermore, cases of acute myocardial infarction at the time of infusion are rare. Hypotension, arrhythmias, heart failure and myocarditis are more common between two to ten days after infusion. CASE REPORTWe report the case of a 43-year-old black female, with a diagnosis of systemic lupus erythematosus (SLE) in 2022. Treatment with CYC and methylprednisolone followed due to glomerulonephritis. During the CYC infusion, she started having typical chest pain. Asymptomatic in the pre-infusion, she received only 100 mg of CYC until the onset of symptoms. The infusion was interrupted, and the patient received isosorbide dinitrate, with improvement after. Troponin test and electrocardiogram (ECG) were ordered in the emergency room. The patient's ECG was compatible with a subendocardial lesion current in the lateral, high lateral wall, and inferior wall. In addition, her troponin levels went from 40.9 to 1,026 pg/mL. An immediate cardiac catheterization was compatible with the diagnosis of myocardial infarction with nonobstructive coronary arteries (MINOCA). After catheterization, she remained in the intensive care unit until full symptom recovery, being transferred to the infirmary and subsequently discharged from the hospital, with a prescription of clopidogrel 75 mg daily, prednisone 40 mg and hydroxychloroquine 400 mg daily. Due to CYC cardiotoxicity and the risk of new cardiac events, it was decided not to perform new infusions, and mycophenolate mofetil 500 mg 4 tablets/day was started to control renal damage. CONCLUSIONThe knowledge of the cardiotoxicity of CYC is widespread in the medical literature, but the pathophysiology of the myocardial toxicity is not completely understood. Due to the use of different dosages, the side-effects profile and intensity may vary greatly. In this case, we observed endothelial and molecular damage even with low dose CYC, highlighting the importance of its side effects for the Rheumatologist.
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