Dr Mohammad Mamun Khan, Resident, Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh; E-mail:
Mymensingh Med J. 2023 Oct;32(4):1208-1213.
Takayasu's arteritis is a chronic large vessel vasculitis that mainly affects the aorta and its major branches such as brachiocephalic, carotid, subclavian, vertebral, renal, coronary and pulmonary arteries. It most commonly occurs in female at child bearing age and female to male ratio is 8:2. Initial presentations of Takayasu's arteritis may be nonspecific like fever, malaise, weakness, fatigue, arthralgia, myalgia and weight loss but in advanced stage there may be features of vascular inflammation like segmental stenosis, occlusion, dilatation and/or aneurysm. It is a sub acute process over months to years and usually with good collateral formation. So, critical limb ischemia due to acute onset of vascular stenosis is very rare. Here we are reporting a case of a 50-year- old male patient who presented with digital gangrene due to critical limb ischemia. Though the age of onset was at 50 years and the patient lacks clinical features like constitutional symptoms, bruit, claudication, asymmetrical blood pressure, the patient was diagnosed as a case of Takayasus's arteritis. The patient dramatically improved with oral glucocorticoid treatment. Although rare, takayasu's arteritis can presents only with digital gangrene without having other clinical features. The physicians should be aware of this unusual but limb threatening presentation and more studies are needed to find out the exact mechanism of this presentation.
Takayasu 动脉炎是一种慢性大动脉血管炎,主要影响主动脉及其主要分支,如头臂干、颈动脉、锁骨下动脉、椎动脉、肾动脉、冠状动脉和肺动脉。它最常发生在生育期的女性,女性与男性的比例为 8:2。Takayasu 动脉炎的初始表现可能是非特异性的,如发热、不适、乏力、疲劳、关节痛、肌痛和体重减轻,但在晚期可能有血管炎症的特征,如节段性狭窄、闭塞、扩张和/或动脉瘤。它是一个亚急性过程,持续数月至数年,通常有良好的侧支形成。因此,由于血管狭窄急性发作导致的严重肢体缺血非常罕见。在这里,我们报告了一例 50 岁男性患者,因严重肢体缺血导致指端坏疽。尽管发病年龄为 50 岁,且患者缺乏如全身症状、杂音、跛行、血压不对称等临床特征,但该患者被诊断为 Takayasu 动脉炎。患者口服糖皮质激素治疗后显著改善。尽管罕见,但 Takayasu 动脉炎可能仅表现为指端坏疽,而无其他临床特征。医生应该意识到这种不常见但威胁肢体的表现,需要更多的研究来找出这种表现的确切机制。