Mahmoud Geilan A, Elsaid Nora Y, Zayed Hania S
Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University, Egypt.
Egypt Rheumatol. 2020 Sep 10. doi: 10.1016/j.ejr.2020.08.010.
Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by vasculopathy, inflammation, autoimmunity, and fibrosis in the skin, lungs and other organs. The occurrence of frank vasculitis is uncommon.
A 36-year old male patient with limited cutaneous SSc developed multiple necrotic ulcers on both legs and feet and gangrene of several toes, followed by an acute onset of axonal sensorimotor neuropathy affecting both radial and peroneal nerves, severe testicular pain with gangrenous patches over the scrotum. The hepatitis B virus (HBV) core antibody was positive while HB surface antigen and surface antibody, HAV and HCV antibodies were negative. The polymerase chain reaction for HBV and HCV showed no detectable viraemia. Antineutrophil cytoplasmic antibodies, cryoblobulins, anticardiolipin antibodies, lupus anticoagulant, antimitochondrial and anti- liver-kidney microsomal antibodies were negative. Pelvi-abdominal ultrasound and portal vein Doppler study showed a coarse and heterogeneous echo-texture of the liver, splenomegaly, moderate ascites and an enlarged, patent portal vein. Fibroscan revealed grade III liver fibrosis. He had an attack of haematemesis with elevation of the liver enzymes and low serum albumin and prothrombin concentrations. He was diagnosed as a case of polyarteritis nodosa. He was successfully treated by methylprednisolone intravenous pulses, followed by oral prednisone 40 mg/day. Plasmapheresis and six monthly doses of 1000 mg intravenous cyclophosphamide. Prednisone was gradually tapered to 5 mg/day with addition of azathioprine 100 mg/day.
The association between systemic sclerosis and polyarteritis nodosa is very rare. The co-existence of SSc and vasculitis necessitates modification of the treatment plan.
系统性硬化症（SSc）是一种多器官结缔组织疾病，其特征为血管病变、炎症、自身免疫以及皮肤、肺部和其他器官的纤维化。明显的血管炎并不常见。
一名36岁男性局限性皮肤型SSc患者，双下肢和足部出现多处坏死性溃疡，多个足趾坏疽，随后急性发作轴索性感觉运动性神经病变，累及桡神经和腓总神经，伴有严重的睾丸疼痛，阴囊出现坏疽斑。乙肝病毒（HBV）核心抗体阳性，而HB表面抗原和表面抗体、甲型肝炎病毒（HAV）和丙型肝炎病毒（HCV）抗体均为阴性。HBV和HCV的聚合酶链反应未检测到病毒血症。抗中性粒细胞胞浆抗体、冷球蛋白、抗心磷脂抗体、狼疮抗凝物、抗线粒体抗体和抗肝肾微粒体抗体均为阴性。盆腔腹部超声和门静脉多普勒检查显示肝脏回声粗糙且不均匀，脾肿大，中度腹水，门静脉增宽且通畅。Fibroscan检查显示肝脏纤维化程度为III级。他出现了一次呕血，同时伴有肝酶升高、血清白蛋白降低和凝血酶原浓度降低。他被诊断为结节性多动脉炎。通过静脉注射甲泼尼龙冲击治疗，随后口服泼尼松40mg/天，成功治愈。进行了血浆置换，并每6个月静脉注射1000mg环磷酰胺。泼尼松逐渐减量至5mg/天，并加用硫唑嘌呤100mg/天。
系统性硬化症与结节性多动脉炎之间的关联非常罕见。SSc和血管炎并存需要调整治疗方案。
