Alfraji Nasam, Upadhyaya Vandan D, Bekampis Christopher, Kuzyshyn Halyna
Department of Medicine, Jersey Shore University Medical Center, Neptune, NJ, 07753, USA.
Internal Medicine Residency Program, Department of Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, 07753, USA.
BMC Rheumatol. 2020 Nov 18;4(1):58. doi: 10.1186/s41927-020-00159-y.
The mixed cryoglobulinemia (MC) syndrome is a systemic inflammatory syndrome that causes small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes most commonly caused by chronic hepatitis C virus (HCV), and rarely by chronic hepatitis B virus (HBV). Its clinical presentation is significantly varied, with manifestations ranging from purpura, arthralgia, and myalgia to more severe neurologic and renal involvement. Pulmonary involvement as organizing pneumonia, alveolar hemorrhage, and pulmonary vasculitis have been reported, but appear to be quite rare.
We report an uncommon case of a patient who presented with primary pulmonary syndrome without renal involvement in the setting of MC, due to untreated chronic hepatitis B infection. Early diagnosis and consequent institution of glucocorticoids, B-cell-depleting monoclonal antibody and antiviral therapy led to a favorable outcome and prevented any fatal sequelae.
Pulmonary compromise in MC syndrome is very uncommon and carries a high rate of mortality. Therefore, in patients with HBV presenting with hemoptysis, physicians must carry a high clinical suspicion for alveolar hemorrhage secondary to cryoglobulinemic vasculitis.
混合性冷球蛋白血症（MC）综合征是一种全身性炎症综合征，由含冷球蛋白的免疫复合物引起中小血管血管炎，最常见的病因是慢性丙型肝炎病毒（HCV），很少由慢性乙型肝炎病毒（HBV）引起。其临床表现差异很大，从紫癜、关节痛和肌痛到更严重的神经和肾脏受累。已有报道称有组织性肺炎、肺泡出血和肺血管炎等肺部受累情况，但似乎相当罕见。
我们报告一例罕见病例，一名因未治疗的慢性乙型肝炎感染而在MC背景下出现无肾脏受累的原发性肺部综合征患者。早期诊断并随后使用糖皮质激素、B细胞耗竭单克隆抗体和抗病毒治疗取得了良好效果，并预防了任何致命后遗症。
MC综合征中的肺部损害非常罕见，死亡率很高。因此，对于出现咯血的HBV患者，医生必须高度怀疑冷球蛋白血症性血管炎继发的肺泡出血。
