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
Table 1
Timeline of patient’s symptoms and course of disease.
Date
Symptoms and exam findings
Laboratory findings and histopathology and procedures
Imaging findings
Therapy administered
Diagnosis
June 2007 (age 5)
Right thigh pain
Bone biopsy of the right femur was not consistent with malignancy and showed red blood cells and scattered neutrophils and lymphocytes
MRI lower extremities: multifocal abnormal bone marrow signal in the right femur, left femoral neck, proximal epiphysis, and metaphysis of the right tibia associated with osteolysis and callus formation
Intermittent ibuprofen
CRMO
Bone scan: increased activity involving the left sacrum, left proximal femur and femoral neck, midshaft of the right femur and proximal right tibia
2007–2015
Leg length discrepancy noted at the age of 7 years
Surgery by orthopedics to fuse the growth plate to stop right leg growth at age of 12 years
Lower extremity x-ray: leg length discrepancy, right leg 4.5 cm longer than left leg
Intermittent ibuprofen
March 2015 (age 13)
Muscle atrophy of the right leg, FTT1, weight and height <3rd%
Bone scan: extensive increased activity in the right femur and asymmetry of activity in the growth plates of the knees and ankles with decreased activity in the right side compared to left
Naproxen 250 mg twice daily (8.7 mg/kg·BID) Prednisone 20 mg per day (0.7 mg/kg) and decreased by 5 mg per week
May 2015
Good control of her leg pain
Continued naproxen and discontinued prednisone
February–April 2016 (age 14)
Hypertension BP2: 154/84, symmetric pulses, diarrhea, vomiting, abdominal pain, weight loss, and perirectal skin tag
Sulfasalazine Oral prednisone 20 mg daily (0.6 mg/kg per day) with tapering Infliximab 3 mg/kg every 8 weeks
Ulcerative colitis
Colonoscopy with pancolitis and crypt inflammation and crypt abscesses, with no granuloma
Electrocardiogram: unremarkable
May 2016
Hypertensive emergency with a BP of 230/190 prior to second dose of infliximab, admitted to the PICU, right Horner’s syndrome, headache, fatigue, asymmetric pulses, and abdominal bruit
Echocardiogram: LVEF 47% and mild LVH
CTA abdomen/pelvis: narrowing of the mid-aorta, proximal renal artery, celiac artery and SMA4
Takayasu’s arteritis complicated by middle aortic syndrome
Negative ANA and ANCA screens and normal C3, C4, and vWbAg3
CTA chest: marked descending thoracic and abdominal aortic wall thickening with progressive luminal narrowing and wall thickening of the right common carotid artery and celiac trunk. Enlarged left atrium, and left ventricular hypertrophy. Small pericardial effusion
MRA chest, abdomen, and pelvis with cardiac MR: lumen narrowing of the distal thoracic and upper abdominal aorta likely similar to prior CTA. Stenosis of origin of the celiac axis, proximal SMA, and moderate stenosis of bilateral proximal renal artery. Concentric LVH5 with mildly reduced function, measuring 45% with the normal coronary artery
Methylprednisolone 1 gram weekly for 8 weeks followed by prednisone taper Infliximab was increased from 5 mg/kg to 10 mg/kg every 4 weeks
MRI/MRA chest, abdomen, and pelvis: stable changes without the need for stenting
1FTT: failure to thrive; 2BP: blood pressure; 3vWb Ag: von Willebrand antigen; 4SMA: superior mesenteric artery; 5LVH: left ventricle hypertrophy; metoprolol was later changed to carvedilol; patient continued infliximab every 8 weeks rather than every 4 weeks as recommended; these were started 2 months later due to social circumstances.