A Case Report of Takayasu’s Arteritis and Ulcerative Colitis in a Pediatric Patient with Chronic Recurrent Multifocal Osteomyelitis Successfully Treated with Infliximab: Diagnostic Clues in Disease Associations and Immune Dysregulation

Abstract

Background. Takayasu’s arteritis with comorbid chronic recurrent multifocal osteomyelitis and ulcerative colitis is rare in the pediatric population. Treatment with anti-TNF alpha agents such as infliximab has been a successful treatment strategy in adults and can be used effectively in the pediatric population. Case Presentation. We present the case of a 15-year-old Caucasian girl with a history of chronic recurrent multifocal osteomyelitis and ulcerative colitis presenting with hypertensive emergency secondary to Takayasu’s arteritis with middle aortic syndrome. She was treated with corticosteroids and methotrexate and ultimately required infliximab infusions of 15 mg/kg every 4 weeks to successfully control her symptoms and normalize her inflammatory markers. Conclusions. In this case, we discuss the use of infliximab in an adolescent patient with chronic recurrent multifocal osteomyelitis, ulcerative colitis, and Takayasu’s arteritis. The significance of this case is determined by the unique occurrence of all three conditions in a pediatric patient, the important consideration of vasculitis in the differential of a pediatric patient presenting with hypertensive emergency, the need for vigilance for detecting diagnostic clues, signs, and symptoms, knowledge of disease associations when evaluating a patient with a predisposition for autoinflammatory conditions, and the use of increasing doses of infliximab to control symptoms.

Clinical Observations on Infliximab Treatment of Infantile Onset Takayasu Arteritis

“…Clinical manifestations of TA are nonspeci c in infants and children. Fever and in ammation are the most common manifestations; the abdominal aorta is the most commonly involved vessel [10][11][12][13][14][15][16]. In our series, eight (80%) cases had fever as the rst symptom and most prominent clinical manifestation.…”

Clinical observations on infliximab treatment of infantile onset Takayasu arteritis

“…Clinical manifestations of TA are nonspecific in infants and children. Fever and inflammation are the most common manifestations, and the abdominal aorta is the most commonly involved vessel [ 5 , 10 – 15 ]. In our series, eight (80%) cases had fever as the first symptom and most prominent clinical manifestation.…”

Mandibular sterile osteitis as a manifestation of synovitis, acne, pustulosis, hyperostosis, osteitis syndrome: a literature review

“…2,3 Chronic pruritus, pruritus lasting more than 6 weeks, has been reported by 91% of AD patients. 4,5 The pathophysiology of AD is driven by a combination of skin barrier dysfunction, neuroinflammation and immune system dysregulation. 6,7 Elevated substance P (SP) is found in both serum and lesional skin of patients with AD.…”

“…6,7 Elevated substance P (SP) is found in both serum and lesional skin of patients with AD. 5,8,9 SP, a neuropeptide released from the activation of sensory neurons, preferentially binds to the neurokinin-1 (NK-1) receptor and is a known itch mediator. 5 Tradipitant (VLY-686), a novel NK-1 receptor antagonist, has the potential to reduce itch related to AD through inhibition of SP-mediated itch signalling.…”

“…5,8,9 SP, a neuropeptide released from the activation of sensory neurons, preferentially binds to the neurokinin-1 (NK-1) receptor and is a known itch mediator. 5 Tradipitant (VLY-686), a novel NK-1 receptor antagonist, has the potential to reduce itch related to AD through inhibition of SP-mediated itch signalling. We examined the efficacy and safety of tradipitant, in reduction of chronic pruritus in adults with mild to severe AD.…”