A case of Takayasu arteritis complicated by refractory ulcerative colitis successfully treated with tofacitinib

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Sir, Takayasu arteritis (TA), a systemic vasculitis affecting large vessels (aorta and its branches), mainly occurs in young females [1, 2]. Gastrointestinal (GI) complications are rare in TA [3]; however, a recent study has elucidated that the complication of ulcerative colitis (UC) is quite common because these two diseases share a genetic background (HLA-B*52:01) [2]. UC is a major inflammatory bowel disease characterized by the increased frequency of bowel movements and recurrent bloody diarrhoea. Recently, the Janus kinase (JAK) inhibitor tofacitinib was approved for UC treatment and showed favourable therapeutic effects [4]. However, at present the effects of tofacitinib on TA remain unknown. We present the first case of a patient with TA complicated by UC who was successfully treated with tofacitinib.

A 17-year-old female with recurrent haematochezia and intermittent right neck pain was admitted to our hospital in June 2018. She had suffered continuous diarrhoea and haematochezia in October 2016 and was diagnosed with UC at the previous hospital and received oral prednisolone (10–20 mg) in combination with mesalazine and azathioprine. However, her GI symptoms persisted, and simultaneously, she felt right intermittent neck pain; therefore, she was referred to the department of gastroenterology at our hospital for further treatment in May 2018. Cervical ultrasonography revealed diffuse wall thickness of the right common carotid artery with 4.4 mm of maximum intima–media thickness, showing the macaroni sign (Fig. 1A). Enhanced CT showed diffuse wall thickness of the right common carotid artery (Fig. 1B) and stenosis of the left subclavian artery, with diffuse wall thickness of the whole colon due to UC inflammation. Colonoscopy showed diffuse mucosal erythema and erosions with diminished vascular pattern (endoscopic Mayo score 2) (Fig. 1C). Further, she was positive for HLA-B52 and owing to refractory UC, TNF inhibitor golimumab was added for remission induction. However, GI symptoms were not completely diminished. In December 2018, golimumab was switched to vedolizumab, a humanized monoclonal antibody designed to specifically antagonize the α4β7 integrin. However, GI symptoms worsened, and 2 months later, enhanced CT revealed continuous wall thickness and stenosis of the affected arteries. Therefore, she was referred to our department. Her laboratory data showed elevated CRP (2.78 mg/dl, normal range: 0–0.3 mg/dl), serum IL-6 (9.1 pg/ml, normal range: <4.0 pg/ml) and serum amyloid A (143 µg/ml, normal range: 0–8 µg/ml) levels. Second colonoscopy showed similar rectal inflammatory mucosa (Mayo score 2). Therefore, the JAK inhibitor tofacitinib was administered for both UC and TA treatment. After steroid pulse therapy (1 g of methylprednisolone for 3 days), oral tofacitinib (20 mg) was administered in combination with oral prednisolone (20 mg/day) and mesalazine. After initiation, CRP levels rapidly normalized and her GI symptoms improved (diminished bloody diarrhoea). After 4 months of tofacitinib administration, the right common carotid artery wall thickness reduced to 1.9 mm of intima–media thickness (Fig. 1D), in accordance with the wall thickness improvement observed in enhanced CT (Fig. 1E). Further, colonoscopy after tofacitinib administration showed mild disease (Mayo score 1) (Fig. 1F). Serum IL-6 levels and serum amyloid A protein levels were almost normalized (3.7 pg/ml and 10.2 µg/ml, respectively). After 6 months of tofacitinib administration, no major relapse is observed with oral prednisolone (13 mg), and she is now preparing for tofacitinib tapering.