Bhowmick Kaustav, Roongta Rashmi, Dey Soumya, Dey Sonali, Mondal Sumantro, Sinhamahapatra Pradyot, Ghosh Parasar, Ghosh Biswadip
Department of Clinical Immunology and Rheumatology, Institute of Post Graduate Medical Education and Research, Kolkata, India.
Clin Rheumatol. 2023 May;42(5):1469-1477. doi: 10.1007/s10067-023-06506-x. Epub 2023 Jan 13.
Takayasu arteritis (TA) is an uncommon chronic granulomatous large-vessel vasculitis affecting the aorta and its branches. Pyoderma gangrenosum (PG) is a chronic neutrophilic dermatosis characterized by rapidly developing painful ulcers. The association of PG with TA is relatively uncommon. We report a case of a 22-year-old lady with a history of recurrent pyoderma lesions for 4 months following which she developed right upper limb claudication. She underwent contrast-enhanced magnetic resonance angiography of the aorta and its branches and was initially diagnosed with type IIb TA. She was put on prednisolone and methotrexate but had a major relapse with new-onset lower limb claudication despite an appropriate course of immunosuppression. She was planned for tocilizumab infusion 8 mg/kg intravenous every 4 weeks. Following the first dose of tocilizumab, her vascular symptoms improved but she had a flare of PG. This was followed by another flare after the second dose. She was switched to tofacitinib which led to sustained remission of her TA activity and healing of her skin lesions, and the prednisolone dose could be reduced to 5 mg daily over the next 1 year. Various immunosuppressives were used to date for treating PG in TA. However, tofacitinib is being reported for the first time in literature for treating PG and controlling TA activity. The paradoxical flare of PG with tocilizumab is quite uncommon and is also reported in our case with literature review.
高安动脉炎（TA）是一种罕见的慢性肉芽肿性大血管血管炎，累及主动脉及其分支。坏疽性脓皮病（PG）是一种慢性嗜中性皮肤病，其特征为迅速发展的疼痛性溃疡。PG与TA的关联相对少见。我们报告一例22岁女性病例，该患者有4个月复发性坏疽性脓皮病皮损病史，之后出现右上肢间歇性跛行。她接受了主动脉及其分支的对比增强磁共振血管造影，最初被诊断为IIb型TA。她开始使用泼尼松龙和甲氨蝶呤治疗，但尽管进行了适当的免疫抑制疗程，仍出现了新发下肢间歇性跛行的严重复发。计划每4周静脉输注8mg/kg托珠单抗。首次输注托珠单抗后，她的血管症状有所改善，但出现了PG病情加重。第二次输注后又出现了一次病情加重。她改用托法替布治疗，这使得她的TA活动持续缓解，皮肤病变愈合，在接下来的1年里泼尼松龙剂量可减至每日5mg。迄今为止，多种免疫抑制剂被用于治疗TA合并的PG。然而，托法替布用于治疗PG并控制TA活动在文献中尚属首次报道。托珠单抗导致PG病情加重的矛盾现象相当少见，我们的病例经文献复习后也有报道。
