Recent advances in role of monocyte-macrophage in anti-neutrophil cytoplasmic antibody associated vasculitis / 上海交通大学学报（医学版）

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) constitues a group of autoimmune diseases with poor prognosis. Inflammation and fibrinoid necrosis of the small vessels are the pathological features of the disease, and many organs and systems can be involved. Monocyte-macrophages are important to innate immune system. Monocyte-macrophage can respond rapidly to inflammation and participate in the progression of AAV. Recently, the role of monocyte-macrophage in AAV has been studied with great detail. This article reviews recent research progress of monocyte-macrophage in AAV so as to further understand the disease.

Correlation between hepatic fibrosis and anti-neutrophil cytoplasmic anti-bodies(ANCA)in schistosomiasis patients / 中国血吸虫病防治杂志

Objective To investigate the association between hepatic fibrosis and anti-neutrophil cytoplasmic antibodies (ANCA)in the patients with schistosomiasis. Methods Totally 145 schistosomiasis patients confirmed by Jingzhou First Peo-ple's Hospital during the period of February 2014 to June 2016 were randomly selected as an experimental group,and 100 healthy people during the same period were randomly selected as a control group. The serum samples of the two groups were col-lected and the level of ANCA was detected by indirect immunofluorescence,and the levels of hyaluronic acid,laminin,procol-lagen type Ⅲ,and collagen type Ⅳ were detected by radioimmunoassay. The levels of the above indexes of the two groups were compared. Results The positive rate of ANCA was 28.96%(42/145)in the experimental group and 1.00%(1/100)in the healthy control group,and the difference between them was statistically significant(P < 0.05). The levels of hyaluronic acid, laminin,procollagen type Ⅲ and collagen type Ⅳ in the experimental group were significantly higher than those in the control group,and the differences were statistically significant(all P < 0.05),and the proportions of those with abnormal levels of hyal-uronic acid,laminin,procollagen type Ⅲ and collagen type IV in the experimental group were significantly higher than those in the control group,and the differences were statistically significant(all P < 0.05). In the experimental group,among the 42 pa-tients with ANCA positives,the proportions of those with abnormal levels of hyaluronic acid and laminin were significantly high-er than the proportions of those with ANCA negatives,and in the former,the levels of the four indicators of liver fibrosis were all higher than the normal values,and the average levels of hyaluronic acid and laminin in the patients with ANCA positives were significantly higher than those in the patients with ANCA negatives(all P < 0.05). Conclusion The patients with schistosomia-sis hepatic fibrosis could be positive for the autoimmune antibody ANCA,and ANCA may be associated with the autoimmune process of liver fibrosis.

Long-term Prognosis of Anti-Neutrophil Cytoplasmic Antibody-Negative Renal Vasculitis: Cohort Study in Korea

Few studies have reported on the long-term prognosis of anti-neutrophil cytoplasmic antibody (ANCA)-negative renal vasculitis. Between April 2003 and December 2013, 48 patients were diagnosed with renal vasculitis. Their ANCA status was tested using indirect immunofluorescence and enzyme-linked immunosorbent assays. During a median (interquartile range) follow-up duration of 933.5 (257.5-2,079.0) days, 41.7% of patients progressed to end stage renal disease (ESRD) and 43.8% died from any cause. Of 48 patients, 6 and 42 were ANCA-negative and positive, respectively. The rate of ESRD within 3 months was higher in ANCA-negative patients than in ANCA-positive patients (P = 0.038). In Kaplan-Meier survival analysis, ANCA-negative patients showed shorter renal survival than did ANCA-positive patients (log-rank P = 0.033). In univariate Cox-proportional hazard regression analysis, ANCA-negative patients showed increased risk of ESRD, with a hazard ratio 3.190 (95% confidence interval, 1.028-9.895, P = 0.045). However, the effect of ANCA status on renal survival was not statistically significant in multivariate analysis. Finally, ANCA status did not significantly affect patient survival. In conclusion, long-term patient and renal survival of ANCA-negative renal vasculitis patients did not differ from those of ANCA-positive renal vasculitis patients. Therefore, different treatment strategy depending on ANCA status might be unnecessary.

Rapidly Progressive Glomerulonephritis -A Review of 26 Cases- / 대한신장학회잡지

OBJECTIVES: Rapidly progres s ive glomerulonephritis (RPGN) is a clinico- pathologic entity characterized by extens ive crescent formation(usually involving 50% or more of glomeruli) as the principal his tologic finding and a rapid deterioration of kidney function, which can lead to end s tage renal disease within a few weeks. T he etiology and incidence of RPGN has been well defined in Europe and North America, however, there has been no report of a large series in Korea. T he aim of the present s tudy was to analyze the etiology and clinico- pathologic features of 26 patients with RPGN, seen during 1983-1997. METHODS: T wenty-six patients with RPGN(crescents in > 50% of glomeruli) were obs erved during a period of las t 14 years. Male to female ratio was 1:1.4, and the mean age was 30(6-75) years. Mean time from the initial symptoms to the ESRD was 3.1 months . RESULTS: The incidence of RPGN in our series was 2.1% of primary glomerulonephritis. Immunecomplex mediated disease was presented in 14 cases (54%), including 6 sys temic lupus erythematos us, 3 post- streptococcal glomerulonephritis, 3 Henoch- Schonlein purpura, and 2 IgA nephropathy. Pauci- immune disease was presented in 12 cases (46%), including 3 Wegener' s granulomatos is, one necrotizing crescentic glomerulonephritis, and 8 idiopathic crescentic glomerulonephritis. However, there was none of anti-GBM- mediated disease in our s tudy. ANCA were found in 6 patients. All 3 patients with WG were C- ANCA pos itive, whereas one patient with PSGN, necrotizing cres centic GN, and idiopathic crescentic GN were P- ANCA pos itive, respectively. Initial clinical and laboratory features included edema(80%), hypertens ion(72%), oliguria(68%), a decreased renal function(serum creatinine > 5mg/dL, 35%), and gros s hematuria(36%). Renal biopsy showed large crescents more than 80% of the glomeruli in 14 cases (54%) which were predominantly fibrocellular. Fifteen patients (58%) were treated with prednis olone alone, and 12 of them received puls e doses of corticosteroids. Five patients were treated with prednisolone and cyclophos phamide IV pulse. Two cases received plasma exchange. During the mean follow-up of 31+/-37 months, 18 patients (69%) developed inexorable progression of renal failure, three(12%) showed recovery of renal function, and two(8%) showed partial improvement, which is followed by varying degrees of renal insufficiency. During follow-up, three patients died : two from res piratory failure with severe pulmonary hemorrhage and one from opportunistic pulmonary infection during immunosuppressive therapy. Poor prognos is is as sociated with hypertension, increased serum creatinine level at the time of diagnosis, large crescents more than 85% of glomeruli, and glomerular scleros is . CONCLUSION: We conclude that an earlier diagnos is including kidney biopsy and the more aggressive treatment are essential in the management of RPGN.