Kennedy Freda, Kapelow Rachel, Kalyon Bilge D, Roth Nitzan C, Rishi Arvind, Barilla-LaBarca Maria-Louise
Department of Medicine, Northwell Health, 300 Community Drive, Manhasset, NY, 11030, USA.
Division of Rheumatology, Department of Medicine, Northwell Health, 865 Northern Boulevard, Suite 302, Great Neck, NY, 11021, USA.
BMC Rheumatol. 2021 May 26;5(1):17. doi: 10.1186/s41927-021-00188-1.
Polyarteritis nodosa is a type of vasculitis affecting medium- and small-sized arteries that has been associated with hepatitis B but does not have an established relationship with autoimmune hepatitis. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa.
A 45-year-old woman was diagnosed with autoimmune hepatitis after initially presenting with a two-month history of fatigue, nausea, and anorexia and a three-week history of scleral icterus. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. Azathioprine was discontinued and she was treated with pulse steroids followed by a prednisone taper, cyclophosphamide, and intravenous immune globulin with overall improvement in her symptomatology. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil.
In patients with recent diagnosis of autoimmune hepatitis, there should be a modest suspicion for concomitant polyarteritis nodosa if symptoms and signs of multisystem vasculitis develop.
结节性多动脉炎是一种影响中小动脉的血管炎，与乙型肝炎有关，但与自身免疫性肝炎尚无明确关联。在此，我们报告一例成年自身免疫性肝炎患者，在诊断后不久即发生危及生命的结节性多动脉炎。
一名45岁女性，最初出现两个月的疲劳、恶心和厌食病史以及三周的巩膜黄疸病史后，被诊断为自身免疫性肝炎。她的肝脏活检显示轻度门静脉纤维化，使用泼尼松和硫唑嘌呤后肝功能有所改善。三个月后，她因发热、双侧踝关节疼痛、皮疹、口腔溃疡和视力下降就诊于急诊科。体格检查发现有结节性红斑、前葡萄膜炎、视网膜血管炎和霜样树枝状视网膜血管炎（霜样树枝状视网膜血管炎（一种广泛的、明显的半透明血管周围渗出物））。随后，她反复出现缺血性急性肠坏死，需要多次手术和广泛的小肠切除术。小肠切除的手术病理显示缺血性坏死、中小血管血管炎伴微血管血栓形成，符合结节性多动脉炎。停用硫唑嘌呤，她接受了冲击性类固醇治疗，随后逐渐减少泼尼松剂量，使用环磷酰胺和静脉注射免疫球蛋白，症状总体有所改善。自住院以来，她一直维持低剂量泼尼松和霉酚酸酯治疗。
对于近期诊断为自身免疫性肝炎的患者，如果出现多系统血管炎的症状和体征，应适度怀疑合并结节性多动脉炎。
