Wednesday, 3 August 2016

A Multidisciplinary Collaboration


Takayasu arteritis is defined as an inflammatory vasculitis that primarily affects the large vessels. Based on the criteria copublished by the Paediatric Rheumatology European Society (PRES), European League against Rheumatism (EULAR) and the Paediatric Rheumatology International Trials Organization (PRINTO), Takayasu Arteritis (TA) requires evidence of abnormalities within the aorta and/or its main branches. Major criteria can be visualized via angiography, Computerized Tomography (CT), or Magnetic Resonance Imaging (MRI) and consist of aneurysms, narrowing/occlusions, dilatations, or wall thickening. These have 100% sensitivity and 99.9% specificity when present. In addition to the major criteria, 1 of 5 minor criteria must be present including: 

1) pulse deficits or claudication, 
2) four limb blood pressure discrepancy > 10mmHg, 
3) bruits or a palpable thrill, 
4) hypertension > 95 percentile for age, gender, and height percentile 
5) elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) above normal. 

Takayasu Arteritis rarely occurs in the pediatric population, and often the diagnosis is delayed due to non-specific initial symptoms caused by systemic inflammation. Although the etiology of the disease is unknown, the pathophysiology includes chronic, autoimmune inflammation causing granulomatous changes to the great arteries. This ultimately results in the pathognomonic findings of aneurysms, vascular stenosis, and/or dilatation. The condition is predominantly described in Asian populations, however no ethnicity is spared, and overall females are affected at a rate of 2:1 over males (though it has been reported as high as 8.5:1). Patients classically present between the 2nd and 4th decade of life. The incidence of Takayasu arteritis in the pediatric population is not known, however it is estimated that annually there may be as many as 2.6 cases per million in North America and 1 case per million in Europe.

The patient is a three month old Hispanic female who was referred to a community hospital emergency room by her primary care physician with three days of cough, congestion, rhinorrhea, decreased oral intake and urine output, non-bloody/ bilious emesis, fatigue, tachypnea and persistent fever (TMax 39.7° C). Parents described development of a new rash that waxed and waned with each fever. A full sepsis screen was performed including blood, urine and cerebrospinal fluid (CSF) studies; and empiric ceftriaxone and gentamicin were initiated. She was briefly admitted to this hospital for concern of a systemic infectious process and dehydration; however required transfer to a tertiary care center pediatric intensive care unit (PICU) for progressive respiratory distress.

Medical history includes premature birth at 34 weeks via caesarean-section, complicated by maternal pre-eclampsia and gestational diabetes. She had a nine day neonatal intensive care unit (NICU) admission for feeding intolerance and hyperbilirubinemia requiring brief phototherapy. Upon discharge she was thriving, growing and developing well. The patient lives with her parents and two healthy siblings. She did not have sick contacts, had not travelled, had no pets, no unusual diet, and no exposure to farm animals or environmental water sources. Her family is Spanish-speaking only, and their history was only significant for a maternal 3rd trimester still-birth of unknown cause.

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