脂膜炎患者的临床特征及治疗随访分析

目的 探讨脂膜炎的临床特征、治疗及预后.方法 随访在我院住院诊断为脂膜炎的患者,对原发性脂膜炎皮肤型和系统型患者的临床特征和治疗随访的结果进行分析.结果 61例患者平均随访49.2个月(2～216个月),17例达到临床缓解,26例反复发作,18例继发结缔组织病(CTD)、血液病、肿瘤和结核.43例原发性脂膜炎患者平均发病年龄(33±17)岁,男女比例1:1.15,皮肤型12例,系统型31例.系统型患者出现肝脏病变25例,脾脏的肿大23例,肺部受累11例,肾脏受累12例,心脏受累3例.系统型9例出现血白细胞减少,而皮肤型为0,两者比较P=-0.044.组织病理学上原发性脂膜炎16.3%患者可见核尘.皮肤型4例单用激素或对症治疗,8例激素联合免疫抑制剂治疗,平均随访63.2个月,7例缓解,5例反复发作;系统型11例单用激素或对症治疗,20例激素联合免疫抑制剂治疗,平均随访47.5个月,10例缓解,21例反复发作.结论 脂膜炎可继发CTD、肿瘤、血液病和结核感染.系统型患者最常受累的脏器为肝脾,可出现白细胞减少.激素和免疫抑制剂治疗部分患者缓解,大多数激素减量易复发.

Analysis of clinical characteristics and follow-up of patients with panniculitis

Objective To explore the clinical characteristics, treatment and outcomes of patients with panniculitis. Methods Clinical data of patients with panniculitis admitted to our hospital were analyzed and followed up. The disease was classified into two sub-types: cutaneous type and systemic type. The clinical profile and treatment as well as the outcomes were compared. Results The mean follow up time of 61 in-patients with panniculitis was 49.2 months (range: 2～216 months). Remission could be achieved in 17 patients, recurrent episodes happened in 26 patients. Eighteen patients turued out to be diffuse connective tissue disease (CTD), hematological disorders, malignant tumors and tuberculosis during follow-up. The age at sympton onset was 33±17 years old in 43 patients with primarily panniculitis which eutaneoua sub-tye was 12 patients and systemic sub-tye was 31 patients. The male: female ratio was 1:15. Patients with systemic sub-type were associated with liver (25 patients), spleen (23 patients), respiratory system (11 patients), kidney (12 patients), and circulatory (3 patients) system involvement. Nine patients with systemic sub-type presen-ted white blood cell decrease while none of in the cutaneous sub-type. The difference was significant (P=0.044). Biopsy of the lesions had shown nuclear dust in 16.3% of primarily panniculitis patients. Conclusion Pannieulitis can associate with systemic rheumatic diseases, malignant tumors, hematological disorders and tuberculosis. The main systemic involvements with systemic sub-type are liver and spleen, and may have whiteblood cell decreasing. Some patients could achieve remission when treated with corticosteroids and/or immunosuppressive drugs. However,the majority of patients will have recurrences when corticosteroids is tapered.