Postmortem Diagnosis of Takayasu Arteritis in a Two-Month-Old Infant

Abstract

Introduction: Takayasu arteritis is a chronic vasculitis, rare in children, characterized by an inflammatory infiltrate with giant cells in the wall of large vessels. Objective: To present the histological findings that led to the diagnosis of Takayasu arteritis in a two-month-old infant. Case Report: A two-month-old infant began, two days before admission, with 38˚C fever, which resolved with antipyretics and reappeared 48 hours later associated with cough and nasal discharge. Initial physical and laboratory examinations were negative; only right parahilar opacity was detected on chest X-ray. The patient began to present generalized skin rash, fever spikes, cough, greenish expectoration, anemia that required blood transfusion, progressive increase in the erythrocyte sedimentation rate, leukocytosis, high platelet count, deterioration of the general condition, respiratory distress (this improved, but reappeared days later accompanied by tachycardia); ultrasound study revealed decreased myocardial contractility, hepatomegaly and ascites. Slowed capillary filling was detected in the lower limbs. He died 28 days later, after several episodes of cardiorespiratory arrest. The anatomopathological study concluded Takayasu arteritis, which affected the aorta, coronary, renal, pulmonary and splenic arteries; complicated by thrombosis and old infarcts in development in the spleen and myocardium, enterocolitis, two acute ulcers in the jejunum, one of them perforated, and generalized peritonitis. Conclusions: Takayasu arteritis is an unusual disease in children. Because it presents with very varied and nonspecific signs and symptoms, the clinical diagnosis is very difficult and it is usually discovered by its fatal course at autopsy.