Vulasala Sai Swarupa, Ahmed Shohana, Onteddu Nirmal K, Mannem Maneesh, Mukkera Srikanth
Radiology, University of Florida College of Medicine - Jacksonville, Jacksonville, USA.
Internal Medicine, Texas Tech University Health Sciences Center - Permian Basin, Odessa, USA.
Cureus. 2021 Aug 30;13(8):e17551. doi: 10.7759/cureus.17551. eCollection 2021 Aug.
Raynaud's phenomenon (RP) is an episodic digital vasospastic condition that is prevalent among 5% of the population. The symptoms range from reversible pallor to ischemia and gangrene. RP can be primary or secondary. We discuss a case of severe RP secondary to granulomatosis with polyangiitis (GPA) that presented with ischemia and gangrene. Studies show that approximately <1% of GPA cases have similar presentations. Early diagnosis and management are essential to halt the progression of ischemia. Calcium channel blockers are the first-line medications used in RP. Phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, and prostacyclin analogs are proven to be effective in cases of severe RP. Sympathectomy and amputation are considered as the extreme options in patients with refractory symptoms.
雷诺现象(RP)是一种发作性的手指血管痉挛性疾病,在5%的人群中较为常见。症状从可逆性苍白到缺血和坏疽不等。RP可分为原发性或继发性。我们讨论一例继发于肉芽肿性多血管炎(GPA)的严重RP病例,该病例表现为缺血和坏疽。研究表明,约<1%的GPA病例有类似表现。早期诊断和治疗对于阻止缺血进展至关重要。钙通道阻滞剂是RP的一线用药。5型磷酸二酯酶抑制剂、内皮素受体拮抗剂和前列环素类似物已被证明对严重RP病例有效。交感神经切除术和截肢被认为是症状难治患者的极端选择。