■结节性皮肤多动脉炎(CPAN)是一种独特的临床实体,由慢性,复发,良性病程,罕见的系统性参与。治疗是用CSs,CYC或其他常规合成DMARD(csDMARD)。在这个系列中,我们旨在分享我们成功治疗CPAN患者的各种临床经验,托法替尼为难治性/复发性疗程或作为不含CSs/csDMARDs的前期单一疗法。
我们报告了2019年至2022年在班加罗尔风湿病中心管理的回顾性病例系列。在活检中确定为CPAN的四名患者能够使用托法替尼作为其治疗的一部分实现无病缓解,在进一步随访中没有复发。我们的患者表现为皮下结节和皮肤溃疡。经过系统评估,所有的病人都做了皮肤活检,显示真皮血管壁中的纤维蛋白样坏死,具有CPAN的组织病理学印象。他们最初用常规方法治疗有/没有csDMARD的CSs。在经历难治性/复发性病程时,托法替尼在所有患者中作为CS保留或不伴随csDMARDs的前期单一疗法进行试验.
■使用托法替尼可改善溃疡和感觉异常,并使皮肤病变逐渐愈合,尽管有疤痕,所有患者在6个月的随访期内没有进一步复发或复发。托法替尼的治疗效果是一致的,无论是作为CS保留或作为前期单一疗法,从而证明该药物是一个有希望的选择,值得在未来进行更大的试验来治疗已建立CPAN的患者子集。
■Tofacitinib可用于无病缓解,作为CPAN的单一疗法,无论是前期还是CS保留,即使没有伴随的CSDMARDs,在那些依赖CSs或多种DMARDs的患者中。
UNASSIGNED: Cutaneous polyarteritis nodosa (CPAN) is a distinct clinical entity represented by a chronic, relapsing, benign course, with rare systemic involvement. Treatment is with CSs, CYC or other conventional synthetic DMARDs (csDMARDs). In this case series, we aimed to share our varied clinical experience of successfully treating patients with CPAN, with tofacitinib in a refractory/relapsing course or as upfront monotherapy without CSs/csDMARDs.
UNASSIGNED: We report this retrospective case series managed at our rheumatology centre in Bangalore from 2019 to 2022. Four patients identified as CPAN on biopsy were able to achieve disease-free remission with tofacitinib as part of their treatment, with no relapse on further follow-up. Our patients presented with subcutaneous nodules and cutaneous ulcers. After systemic evaluation, all the patients underwent skin biopsy, which showed fibrinoid necrosis in the vessel walls of the dermis, with a histopathological impression of CPAN. They were initially treated with a conventional approach of CSs with/without csDMARDs. On experiencing a refractory/relapsing course, tofacitinib was tried in all the patients as either CS sparing or upfront monotherapy without concomitant csDMARDs.
UNASSIGNED: Use of tofacitinib resulted in improvement of ulcers and paraesthesia and in gradual healing of skin lesions, albeit with scarring, with no further recurrence or relapse over a follow-up period of 6 months for all the patients. The therapeutic effect of tofacitinib was consistent when used either as CS sparing or as upfront monotherapy, thereby proving the drug to be a promising option that warrants larger trials in future to treat the subset of patients with established CPAN.
UNASSIGNED: Tofacitinib could be used for disease-free remission as monotherapy for CPAN either upfront or as CS sparing, even without concomitant csDMARDs, in those patients who are dependent on CSs or multiple DMARDs.