原发性干燥综合征是一种以外分泌腺损伤为特征的慢性炎症性自身免疫性疾病.干燥综合征起病个体差异性较大,临床症状多样,损伤严重程度各不相同.原发性干燥综合征除了导致腺体损伤外,还可能涉及多个器官和系统,其常见的临床表现之一为神经系统症状,中枢神经系统、周围神经系统均可累及.其中干燥综合征首发表现为视神经脊髓炎谱系疾病的情况在临床上较少见,在实际的临床诊疗过程中,询问病史、筛查抗核抗体及抗水通道蛋白4(Aquaporin Protein 4,AQP4)抗体发挥重要的作用.文章报道1例以视神经脊髓炎谱系疾病为首发表现的干燥综合征青年女性患者,该患者以下肢麻木疼痛伴瘙痒为首发表现,多次就诊于皮肤科,发病2个月后才出现干燥症状,确诊后予中西医治疗,治疗后好转,随访1年未见复发.文章通过分析此病例的临床表现、化验检查、诊治预后等,以提高对此类疾病的认识,以便对病情进行早期诊断,以免出现误诊或漏诊,并及时进行中西医联合治疗,控制病情,避免出现不可逆转的损害.同时在此过程中还应注意动态随访,减少复发,提高患者的生活质量.
One case of Sjogren's syndrome with neuromyelitis optica spectrum disorders as the initial presentation
Primary Sjogren's syndrome is a chronic inflammatory autoimmune disease characterized by exocrine gland injury.Patients with Sjogren's syndrome have great individual differences,with various clinical symptoms and varying degrees of injury.In addition to causing glandular damage,primary Sjogren's syndrome may also involve multiple organs and systems.One of its common clinical manifestations is neurological symptoms,and there are also central nervous system and peripheral nervous system symptoms.Among them,neuromyelitis optica spectrum disorders as the first manifestation of Sjogren's syndrome is rare in clinical practice.In the actual process of clinical diagnosis and treatment,taking medical history,screening for antinuclear antibodies and anti-aquaporin protein 4 antibodies play an important role.This paper reports one young female patient with Sjogren's syndrome with neuromyelitis optica spectrum disease as the first manifestation.The patient presented to the dermatology department for several times with numbness and pain of lower limbs accompanied by pruritus as the first manifestation.The dry symptom did not occur until 2 months after the onset of NMOSD.After diagnosis,the patient was treated in traditional Chinese medicine and Western medicine for amelioration,and no recurrence was observed during 1 year of followed-up.This paper analyzes the clinical manifestations,laboratory examinations,and diagnosis,treatment and prognosis of this case,so as to improve the understanding of such diseases and make an early diagnosis of the patient's condition to avoid misdiagnosis or missed diagnosis.The combination of traditional Chinese medicine and Western medicine should be taken in time to control the condition in order to avoid irreversible damage.At the same time,dynamic follow-up should also be paid attention to in this process to reduce recurrence and improve the quality of life of patients.
NETL
NSTL
万方数据
维普
