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, FTT^1, weight and height <3^rd%		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 BP^2: 154/84, symmetric pulses, diarrhea, vomiting, abdominal pain, weight loss, and perirectal skin tag	ESR: 69 mm/hr (nl 0–10 mm/hr), CRP 129 mg/L (nl 0–3 mg/L), Hb 7.1 g/dL (nl 12–16 g/dL), platelets 744 k (nl 150–450 k), calprotectin > 2000 μg/g (nl <50 μg/g), positive occult blood	Renal ultrasound with Doppler: normal	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 SMA^4	Metoprolol 50 mg daily (1.5 mg/kg); Amlodipine 5 mg daily (0.15 mg/kg); Famotidine 20 mg BID (0.6 mg/kg); Aspirin 81 mg; Increase infliximab to 5 mg/kg IV every 4 weeks; Increased prednisone to 20 mg daily (0.6 mg/kg); Mesalamine 1000 mg BID (31 mg/kg)	Takayasu’s arteritis complicated by middle aortic syndrome
		Negative ANA and ANCA screens and normal C3, C4, and vWbAg^3	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
		ESR 31 mm/hr (nl 0–10 mm/hr) and CRP 23 mg/L (nl 0–3 mg/L)	MRI/MRA brain: normal
August–September 2016 (age 15)	Right leg pain and inflammatory arthritis of the left ankle	ESR 55 mm/hr (nl 0–10 mm/hr) and CRP 73 mg/L (nl 0–3 mg/L)	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 LVH^5 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
December 2016	Asymptomatic	ESR 49 mm/hr (nl 0–10 mm/hr) and CRP 78 mg/L (nl 0–3 mg/L)	Carotid duplex: minimal stenosis in internal carotid artery bilaterally and wall thickening around the right common carotid artery	Infliximab 10 mg/kg every 4 weeks,; Prednisone was increased to 10 mg daily,; Methotrexate 20 mg once a week orally (15 mg/m^2)
			Echocardiogram: moderate left ventricle dilation. Borderline (low normal to mildly depressed left ventricular systolic function)
January 2017 (age 15)	Asymptomatic	ESR 6 mm/hr (nl 0–10 mm/hr) and CRP 7.8 mg/L (nl 0–3 mg/L)	Head and neck CTA: progression of right carotid artery stenosis with about 80% stenosis	Infliximab was increased to 15 mg/kg every 4 weeks; Prednisone 10 mg daily; Methotrexate 20 mg weekly (15 mg/m^2)
June 2017	Denies any complaints	ESR 2 mm/hr (nl 0–10 mm/hr) and CRP 0.2 mg/L (nl 0–3 mg/L)	MRI/MRA brain: normal	Infliximab 15 mg/kg every 4 weeks; Prednisone 5 mg daily; Methotrexate 20 mg weekly (15 mg/m^2)
			MRA chest and abdomen: stable study as before without worsening or improvement
June 2018	Denies any complaints	ESR and CRP normal	MRI/MRA brain: normal	Infliximab 15 mg/kg every 4 weeks; Methotrexate 20 mg weekly (15 mg/m^2)
			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.