The application of antineutrophil cytoplasmic antibody renal risk score in children with antineutrophil cytoplasmic antibody-associated glomerulonephritis / 中华实用儿科临床杂志

Objective:To investigate the potential of the antineutrophil cytoplasmic antibody (ANCA) renal risk score (ARRS) in predicting the prognosis of children with ANCA-associated glomerulonephritis (AAGN).Methods:Laboratory testing, renal pathology results, treatment and prognosis of 61 children with AAGN diagnosed by renal biopsy from June 2007 to May 2022 in General Hospital of Eastern Theater Command were retrospectively analyzed.The Kaplan-Meier method was used to evaluate the overall and renal survival of children with AAGN, and risk factors of progression to end stage renal disease (ESRD) were analyzed by Cox regression analysis. Results:Among the 61 children with AAGN, there were 14 males and 47 females with the age of (15.65±3.74) years.According to ARRS, AAGN children were assigned into low-risk group (27 cases), medium-risk group (21 cases) and high-risk group (13 cases). During a median follow-up duration of 46.36 (14.58, 95.62) months, the number of ESRD cases in the high-risk group (9 cases) was significantly higher than that of low-risk group (2 cases) and medium-risk group (3 cases) ( χ2=13.079, P<0.001). Kaplan-Meier survival analysis showed that AAGN children in the high-risk group had the worst renal prognosis ( χ2=5.796, P=0.016), while no significant difference was detected in the overall survival among the 3 groups ( χ2=2.883, P=0.237). Multivariate Cox regression showed that estimate glomerular filtration rate(eGFR)≤15 mL/(min·1.73 m 2) ( HR=9.574, 95% CI: 4.205-25.187, P=0.015) and ARRS ( HR=2.115, 95% CI: 1.206-4.174, P=0.012) were independent risk factors for children with AAGN progress to ESRD.Receiver operating characteristic (ROC) curve analysis results showed that the area under the curve of ARRS for predicting the risk of progressing to ESRD in AAGN children was 0.880 (95% CI: 0.759-1.000), and the optimal cutoff value of ARRS was 5.50, with the sensitivity and specificity of 85.71% and 82.98%, respectively. Conclusions:ARRS was an independent risk factor for children with AAGN progress to ESRD, which had a predictive value for the progression of AAGN to ESRD.

The role of the NOD-like receptor thermal protein domain associated protein 3 inflammasome in children with antineutrophil cytoplasmic antibody-associated vasculitis / 中华实用儿科临床杂志

Objective:To investigate the role of the NOD-like receptor thermal protein domain associated protein 3(NLRP3)inflammasome and its downstream interleukin(IL)-1β, IL-6, and IL-18 in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis(AAV) in children.Methods:A retrospective study was conducted.Specifically, the localization and expression of the NLRP3 inflammasome in renal tissues of 22 children who were diagnosed with primary AAV and underwent renal biopsy in the Department of Pediatric Nephrology and Rheumatology, the First Affiliated Hospital of Sun Yat-Sen University from September 2003 to September 2020 were detected by the immunohistochemical method.The IL-1β, IL-6 and IL-18 levels in serum and urine were measured by enzyme-linked immunosorbent assay.The measurement data conforming to normal distribution were compared by the t test between two groups and by the single factor ANOVA test among multiple groups.The measurement data that did not conform to normal distribution were compared by the Wilcoxon signed rank sum test.Classification variables were examined by the χ2 test. Pearson correlation coefficient or Spearman rank correlation coefficient were used to analyze the correlation among variables. Results:NLRP3 was widely expressed in the tubulointerstitium, and the expression level in the active group was higher than that in the control group, the semi-quantitative scores of NLRP3 in the renal tubule and glomeruli in the active group were higher than those in the control group ( F=0.859, 8.320, all P<0.05). In the active group, the semi-quantitative score of NLRP3 in the renal tubule was higher than that in the glomeruli( F=3.517, P<0.05). The semi-quantitative score of NLRP3 in the renal tubule was positively correlated with the pediatric vasculitis activity score at renal biopsy ( r=0.471, P=0.027)and negatively correlated with the estimated glomerular filtration rate at renal biopsy ( r=-0.548, P=0.008)in the active group.The serum IL-1β, serum IL-18 and urinary IL-6 levels in the active group were higher than those in the remission group and the control group ( F=16.449, 16.449, 0.637, 29.891, 27.612, 7.464, all P<0.05). The serum IL-18 level in the remission group was higher than that in the control group( F=18.671, P<0.05). In the active group, a positive correlation was found between the serum IL-1β level and the semi-quantitative score of NLRP3 in the renal tubule( r=0.805, P=0.002), between the serum IL-6 level and the C-reactive protein level at renal biopsy ( r=0.728, P=0.017), and between the urinary IL-6 level and the crescent proportion at renal biopsy ( r=0.677, P=0.032). The serum IL-18 level in the active group was positively correlated with the semi-quantitative score of NLRP3 in the renal tubule, pediatric vasculitis activity score and glomerular sclerosis proportion at renal biopsy, and negatively correlated with the estimated glomerular filtration rate at renal biopsy ( r=0.644, 0.612, 0.695, -0.577, all P<0.05). The urinary IL-18 level was positively correlated with the complement C 4 level at renal biopsy ( r=0.855, P<0.05). Conclusions:The NLRP3 inflammasome and its downstream IL-1β, IL-6, and IL-18 may be involved in the pathogenesis and progression of AAV, and can be used as one of the reference indicators for disease activity assessment.

Clinical analysis of 15 cases with myeloperoxidase antineutrophil cytoplasmic antibody associated hypertrophic pachymeningitis / 中华内科杂志

To study the clinical features of myeloperoxidase(MPO) antineutrophil cytoplasmic antibody (ANCA) associated hypertrophic pachymeningitis (HP). Clinical data of 15 cases diagnosed with MPO-ANCA vasculitis complicated with HP were retrospectively analyzed. Nine cases were males and the other 6 were females, with an average age of (58±8) years. All cases presented with chronic headache. Contrast-enhanced magnetic resonance imaging (MRI) scan showed local or diffused thickening of cerebral and/or spinal dura matter while brain parenchyma were normal. Nine cases developed multiple cranial nerve paralysis, with trigeminal nerve and auditory nerve involved most commonly. The main clinical manifestations were facial pain, hearing loss and tinnitus. Two cases were complicated with hypertrophic spinal pachymeningitis (HSP) and 4 cases were complicated with pulmonary diseases. Positive serum perinuclear pattern ANCA (pANCA) and MPO could be found in all cases, positive serum IgG 4 was seen in two patients. erythrocyte sedimentation rate(ESR;25-116 mm/1h) and C-reactive protein (CRP;29.02-146.00 mg/L) were both elevated in 14 cases. Nine cases had elevated intracranial pressure[180-235 mmH 2O (1 mmH 2O=0.009 8 kPa)] and abnormal protein level (457.6-3710.0 mg/L) in cerebrospinal fluid. Six cases were treated with glucocorticoids (prednisone 20-60 mg/d) and 9 cased with glucocorticoids and immunosuppressants (methotrexate 15 mg/week or cyclophosphamide 100 mg/d po). All patients achieved remission. MPO-ANCA associated HP is a special type of central nervous system involvement in ANCA associated vasculitis (AAV). It rarely involves the lung or kidney. Steroids and immunosuppressive agents are effective. In HP with unknown underlying diseases, it is suggested to screen ANCA and IgG 4 tests for AAV or IgG 4-related disease.

Nursing care of a patient with antineutrophil cytoplasmic antibody associated vasculitis and autoimmune hemolytic anemia, hemophagocytic syndrome / 中国实用护理杂志

Objective:To summarize the nursing care of a patient with antineutrophil cytoplasmic antibody associated vasculitis and autoimmune hemolytic anemia, hemophagocytic syndrome.Methods:A patient with antineutrophil cytoplasmic antibody associated vasculitis and autoimmune hemolytic anemia, hemophagocytic syndrome on March 24, 2020 in Jingmen Second People′s Hospital was selected. The key points in nursing were targeted nursing such as high fever, for complications(hypothermia and shock); actively monitor systemic damage and preventive care of complications; including prevention of exacerbation of hemolysis and prevention of complications after blood transfusion;observation and nursing of prevention of bleeding and disseminated intravascular coagulation;renal function monitoring, evaluation and prevention of complications; nursing of respiratory failure and prevention of complications,and medication nursing.Results:After careful treatment and care, the patient's body temperature returned to normal, and respiratory failure was corrected. There was no worsening of hemolysis, bleeding, disseminated intravascular coagulation, or renal failure. Hemoglobin, platelets, erythrocyte sedimentation rate, and serum ferritin were normal after repeated examinations, and the patient was discharged smoothly.Conclusions:According to the clinical characteristics and individual conditions of the patient,systemic damage monitoring and prevention of complications,accurate assessment and observation of the condition,and targeted protective measures are the keys to reducing systemic damage,complications and successful nursing care.

Severe Pulmonary Embolism, Thrombosis of Lower Extremity, Unexpected Mild Renal Disorder in MPO-ANCA Associated Vasculitis: A Case Report / 中国医学科学杂志(英文版)

Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) associated vasculitis is an autoimmune disease usually with severe multiple dysfunction syndrome, especially prominent acute renal failure. A 65-year-old woman was admitted with progressive dyspnoea for six months and fever, sputum with blood, pain of the lower extremities and intermittent claudication for two days, indicating multiple organ involvement (respiratory system, blood vessels). The renal involvement was relatively mild, presenting with microscopic haematuria. The chest computed tomography demonstrated multiple pulmonary embolisms. Ultrasound and computed tomography angiography for the lower extremity vessels showed venous and arterial thrombosis. Exclusion of other diseases that can cause multiple organ damage and thrombosis, the positive perinuclear ANCA and MPO-ANCA strongly support the diagnosis of MPO-ANAC-associated vasculitis. The patient's physical condition has been greatly improved by treatment with corticosteroids and anticoagulation.

The novel fibrosis index at diagnosis may predict all-cause mortality in patients with antineutrophil cytoplasmic antibody-associated vasculitis without substantial liver diseases

OBJECTIVES: Antineutrophil cyto plasmic antibody-associated vasculitis (AAV) is a fatal disease. Currently, predictors of mortality due to AAV are based on the distribution of organ involvement. The novel fibrosis index (NFI) is an index composed of laboratory results that reflect the degree of liver fibrosis. This study aimed to evaluate whether NFI can predict poor outcomes in patients with AAV without substantial liver disease. METHODS: A total of 210 patients with immunosuppressive drug-naïve AAV were retrospectively reviewed. NFI was calculated as follows: NFI=(serum bilirubin × (alkaline phosphatase)2)/(platelet count×(serum albumin)2). NFI cut-off was set at 1.24 (the highest quartile). Poor outcomes were defined as all-cause mortality, relapse, and end-stage renal disease (ESRD). RESULTS: During the median 34.5 months of follow-up, 21 patients (10%) died, 72 patients (34.3%) relapsed, and 38 patients (18.1%) had ESRD due to AAV progression. The median calculated NFI was 0.61, and it was higher in AAV patients with all-cause mortality than in those without mortality, but the difference was not statistically significant (1.26 vs. 0.59). AAV patients with NFI at diagnosis ≥1.24 exhibited a significantly lower cumulative patient survival rate than those with NFI at diagnosis <1.24 (p=0.002). Multivariate Cox hazard model analysis showed that NFI at diagnosis ≥1.24 was an independent predictor of all-cause mortality in AAV (hazard ratios [HR] 2.850, 95% confidence interval [CI] 1.026, 7.910). CONCLUSIONS: NFI ≥1.24, which may be an independent predictive marker for all-cause mortality in AAV patients without substantial liver disease.

Digital infarct and mononeuritis in a middle aged female: always suspect Antineutrophil cytoplasmic antibodies associated vasculitis

Vasculitis is a process caused by inflammation of blood vessel walls and results in a variety of disorders. Small-vessel vasculitis (vasculitis involving arteries, venules and capillaries) should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Testing for Antineutrophil cytoplasmic antibody (ANCA) is the basis of classification of small vessel vasculitis into ANCA associated and non - ANCA associated vasculitis. Apart from cutaneous manifestations like palpable purpura and vasculitic urticaria, digital gangrene in a patient with evidence of mononeuritis multiplex is highly suggestive of ANCA associated vasculitis (AAV). Clinically most of these vasculitides have overlapping clinical presentations and similar treatment. Early diagnosis and rapid initiation of treatment of AAV is recommended rather than ordering for definitive tests (e.g. histopathology or angiograms) since delay in treatment can result in serious end organ damage (pulmonary or renal).

Microscopic polyangiitis and antiphospholipid syndrome. An uncommon association / Poliangitis microscópica y síndrome antifosfolípido. Una asociación infrecuente

ABSTRACT Antiphospholipid syndrome is frequently associated with systemic lupus erythematosus and other autoimmune diseases. However, coexistence with primary vasculitis has been poorly reported. The case is presented of a 67-year-old patient with a history of recurrent abortion and chronic pulmonary thromboembolism who was admitted due to haemopty sis. At the initial evaluation, a massive alveolar haemorrhage and glomerulonephritis were diagnosed. The results of the antibodies were positive for ANCA with P-type pattern, anti-myeloperoxidase antibodies, and antiphospholipid antibodies (anti-β2 IgG glycoprotein 1 and lupus anticoagulant). Diagnosis of ANCA positive vasculitis-type microscopic polyangiitis was made in association with antiphospholipid syndrome. Given the clinical context, it was decided to initiate intravenous methylprednisolone in pulses for 3 consecutive days, fol lowed by oral prednisone, and as maintenance therapy, rituximab and anticoagulation with warfarin were instituted. The clinical evolution of the patient was satisfactory, with symp tom control being achieved, as well as a significant improvement of renal and pulmonary function, with a decrease in the Birmingham vasculitis activity score (BVAS).

RESUMEN El síndrome antifosfolípido se asocia frecuentemente con lupus eritematoso sistémico y otras enfermedades autoinmunes. Sin embargo, la coexistencia con vasculitis primaria ha sido poco reportada. Se presenta el caso de una paciente de 67 arios de edad con historia de aborto recurrente y tromboembolismo pulmonar crónico, quien es admitida para estudio de hemoptisis. A la evaluación inicial se diagnosticó una hemorragia alveolar masiva y glomerulonefritis. El resultado de los anticuerpos fue positivo para anticuerpos anticitoplasma de neutrófilos (ANCA) con patrón tipo perinuclear, anticuerpos anti-mieloperoxidasa y anticuerpos antifosfolípidos (anti (32 glicoproteína 1 IgG y anticoagulante lúpico), configurándose el diagnóstico de vasculitis asociada a ANCA de tipo poliangitis microscópica en asociación con síndrome antifosfolípido. Dado el contexto clínico, se decidió iniciar metilprednisolona intravenosa en pulsos por 3 días consecutivos, seguida de prednisona oral, y como terapia de mantenimiento se instauró rituximab y anticoagulación con warfarina. La evolución clínica de la paciente fue satisfactoria, alcanzando control de síntomas e importante mejoría de la función renal y pulmonar, con disminución del score BVAS.

Research progress of combined rheumatoid factor ANCA, IL-1, IL-6, TNF-αdetection in diagnosing rheumatoid arthritis / 国际检验医学杂志

Rheumatoid Arthritis is a kind of chronic autoimmune diseases mainly involving joint, which is characterized by systemic inflammatory response.Studies have shown that Rheumatoid arthritis patients showed associated antineutrophil cytoplasmic antibody (ANCA) and inflammatory cytokines such as interleukin (IL) , tumor necrosis factor (TNF) increased, the positive of ANCA and the increase of IL-1, IL-6, TNF-αare especially seen in the Rheumatoid arthritis patients who have inflammatory reaction, and which are roughly equal to the articular synovitis pathological mechanism of wind dampmess heat accumulation pattern in the traditional Chinese medicine.The treatment for Rheumatoid arthritis patients, Western treatment is use the methotrexate (MTX) as basic drugs, combined with other drugs to control the positive of ANCA and the higher of IL-1, IL-6, TNF-α.And combining traditional Chinese medicine treatment, the effect is not only at to increase Western effection but also be able to reduce the side effects of Western medicine.Therefore, the research of combined rheumatoid factor ANCA, IL-1, IL-6, TNF-αdetection is benefit to diagnosing rheumatoid arthritis.

Antineutrophil Cytoplasmic Antibody-Associated Vasculitis in Korea: A Narrative Review

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involve small vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations and pathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), and eosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currently the algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeable advancements in studies on the pathophysiology of AAV and the classification criteria for AAV in Western countries. However, studies analysing clinical features of Korean patients with AAV have only been conducted and reported since 2000. One year-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitis activity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortality or poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infection free survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed 1.43 compared to the general Korean population.

Classification of Antineutrophil Cytoplasmic Antibody-associated Vasculitis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of systemic vasculitides, that are characterized by inflammation in the small vessels, ranging from capillaries to arterioles or venules. AAV is divided into three variants based on the clinical manifestations and histological findings such as microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). MPA often induces rapid progressive necrotising glomerulonephritis, and occasionally induces diffuse alveolar hemorrhage. In contrast, GPA preferentially affects the respiratory tracts from the bronchus to the nasal cavity. GPA can also involve the kidneys, but the frequency of renal involvement is less than MPA. EGPA is based on allergic components such as asthma, peripheral eosinophilia, migratory eosinophilic pneumonia and eosinophil infiltration. Since 1982, when the association between ANCA and systemic vasculitis was first reported, several classification criteria for AAV have been proposed. This review describes the classification criteria for and nomenclature of AAV from the 1990 American College of Rheumatology (ACR) classification criteria to the 2012 revised Chapel Hill consensus conference (CHCC) nomenclature of Vasculitides. New classification trials for AAV such as AAV based on the ANCA-types (myeloperoxidase-ANCA vasculitis, proteinase 3-ANCA vasculitis and ANCA negative vasculitis) and the ACR/European League Against Rheumatism (EULAR) 2017 provisional classification criteria for GPA were also introduced. In addition, the histopathological classification of ANCA-associated glomerulonephritis and the revised 2017 international consensus on testing of ANCAs in GPA and MPA are also discussed.

Antineutrophil cytoplasmic antibody-positive scleritis: Clinical profile of patients from a tuberculosis-endemic region

Purpose: To report the clinical profile of a series of antineutrophil cytoplasmic antibody (ANCA)-associated scleritis in Indian population. Methods: We conducted a retrospective review of medical records of 33 eyes of 26 consecutive patients with scleritis, who tested positive for either antibody to proteinase 3 [anti-PR3/cytoplasmic antineutrophil cytoplasmic antibody (cANCA)] or myeloperoxidase [anti-MPO/perinuclear anti-neutrophil cytoplasmic antibody (pANCA)] between 2006 and 2015. Results: The mean age at presentation was 54.1 (11.1) years and 61.5% of the patients were female. Underlying systemic disorder was found in 46.2% of patients and includes granulomatosis with polyangitis (30.8%) and tuberculosis (15.4%). Necrotizing scleritis (48.5%) was the most common scleritis observed, followed by diffuse anterior scleritis (42.4%). Positive cANCA was found in 65.4% of patients and 34.6% was found positive for pANCA. Four of the six patients with positive Mantoux test were started on anti-tuberculosis treatment (ATT) by pulmonologist. Cyclophosphamide was the most common immunosuppressive and 11.5% of the patients required combination of two immunosuppressives. Seventeen eyes developed cataract and four eyes required patch graft. Female gender was more frequently associated with pANCA-associated scleritis than cANCA (P = 0.037). Incidence of necrotizing scleritis was higher in patients with positive cANCA, but this difference was not statistically significant (P = 0.806). cANCA-positive patients had statistically significant higher association with systemic rheumatic diseases (P = 0.021). Conclusion: Necrotizing scleritis is the most common subtype of scleritis in ANCA-positive individuals and even in the absence of systemic involvement. All patients with ANCA positivity should be thoroughly screened to rule out any evidence of tuberculosis, especially in tuberculosis-endemic region before planning aggressive immunomodulatory therapy.

Eosinophilic granulomatosis with polyangiitis: a challenge for differential diagnosis / Granulomatosis eosinofílica con poliangitis: un desafío para el diagnóstico diferencial

Abstract Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a rare small and medium vessels vasculitis, consisting of asthma, migratory pulmonary infiltrates and eosinophilia. Its low occurrence makes it difficult to achieve an early diagnosis, and hence a directed treatment in order to control it and avoid complications. We report a 31 year-old man with refractory asthma, who developed arthritis and multiplex mononeuritis. Before EGPA's diagnosis, he had just received asthma treatment (steroids, bronchodilators, antileukotriene and omalizumab); but once EGPA is confirmed and correct treatment was started, there was a remarkable clinical improvement.

Resumen La granulomatosis eosinofílica con poliangitis (EGPA), anteriormente conocida como síndrome de Churg-Strauss, es una vasculitis poco frecuente de vasos pequeños y medianos, que consiste en asma, infiltrados pulmonares migratorios y eosinofilia. Su baja aparición dificulta el diagnóstico precoz y, por lo tanto, un tratamiento dirigido para controlarlo y evitar complicaciones. Presentamos a un hombre de 31 años con asma refractaria, que desarrolló artritis y mononeuritis múltiple. Antes del diagnóstico de EGPA, acababa de recibir tratamiento para el asma (esteroides, broncodilatadores, antileucotrienos y omalizumab); pero una vez que se confirmó la EGPA y se inició el tratamiento correcto, hubo una mejoría clínica notable.

C-Reactive Protein to Serum Albumin Ratio Is an Independent Predictor of All-Cause Mortality in Patients with ANCA-Associated Vasculitis

PURPOSE: We investigated whether C-reactive protein (CRP) to serum albumin ratio (CAR) could be an independent predictor of all-cause mortality in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). MATERIALS AND METHODS: We retrospectively reviewed the medical records of 170 patients with AAV. We collected clinical and laboratory data. We also examined AAV-related and traditional risk factors of all-cause mortality. To assess the hazard ratios of variables, we performed univariable and multivariable Cox hazard model analyses. RESULTS: The mean age was 55.0 years and 53 patients (31.2%) were male among 170 patients with AAV (88 microscopic polyangiitis, 43 granulomatosis with polyangiitis, and 39 eosinophilic granulomatosis with polyangiitis). ANCA was detected in 129 patients (75.9%). The initial mean CRP and serum albumin were 41.1 (mg/L) and 3.6 (g/dL), and the mean CAR at diagnosis was 14.8. The most common risk factor of mortality was hypertension (42.4%), followed by chronic kidney disease ≥stage 3 (25.9%). Fourteen patients (8.2%) died during the mean follow-up of 56.7 months. In both multivariable Cox hazard model analyses, CAR at diagnosis was identified as an independent predictor of all-cause of mortality comparable to diabetes mellitus (DM). Moreover, patients with CAR ≥10.35 and having DM exhibited a higher frequency of all-cause mortality than those without. CONCLUSION: CAR at diagnosis can be an independent predictor of all-cause mortality, comparable to DM, the conventional risk factor of mortality.

Advances on ANCA-associated vasculitis / 复旦学报（医学版）

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) consists of autoimmune diseases characterized by necrotizing inflammation of small vessels and presence of ANCA.As a kind of life-threatening disease,AAV tends to involve a plenty of systems,and is subject to relapse even after complete remission.In this review,we focus on the latest progress of epidemiology,etiology,pathogenesis and clinical diagnosis of AAV.

A report of 4 cases of myeloperoxidase antin eutrophil cytoplasmic antibody associated hypertrophic pachymeningitis with literature review / 中华风湿病学杂志

Objective To investigate the clinical features of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) associated hypertrophic pachymeningitis (HP).Methods Clinical data of 4 casesdiagnosed with MPO-ANCA vasculitis complicated with HP in our hospital were analyzed retrospectively and the related literaturewere reviewed.Results Four male patients with an age range from 44 to 66 years were diagnosed with ANCA-associated HP.The main clinical manifestations included headache and withvarious degree ofmultiple cranial paralysis.During active phase of the disease,all patients showed perinuclear(p)-ANCA positive,elevated levels of inflammatory biomarkers and titers of MPO-ANCA,whereas renal function,cytoplasmic (c)-ANCA and protease 3 (PR3)-ANCA were negative.Contrast-enhanced cranial magnetic resonance imaging (MRI) scan showed obviously thickened dura mater and sinusitis or mass in paranasal sinus.Four patients were sensitive to glucocorticoid.Three patients had a relapse during glucocorticoid tapering and were undercontrol when the dosage of glucocorticoid was increased and immunosuppressive agents were added.Levels of inflammatory biomarkers,titers of MPO-ANCA and p-ANCA recovered to normal,and the dural thickness on MRI was reduced in the remission stage.Conclusion MPO-ANCA associated HP is a type of central nervous system involvement in ANCA associated vasculitis (AAV).It involves the upper respiratory tract more frequently,and less frequently progresses to systemic AAV.This should be taken into consideration when middle-aged and elderly patients presented with headache and multiple cranial neuropathies.Enhanced MRI is the preferred examination for diagnosis,and dural biopsy should be done when necessary.

Clinical value of antineutrophil cytoplasmic antibody in eosinophilic granulomatosis with polyangiitis / 中华风湿病学杂志

Objective To investigate the value of antineutrophil cytoplasmic antibody (ANCA) in clinical phenotype of eosinophilic granulomatosis with polyangiitis (EGPA).Methods The clinical data of 64 patients with EGPA from Peking Union Medical College Hospital between 2007 to 2016 were retrospectively analyzed,and the patients were followed up.Characteristics of patients with ANCA positive and ANCA negative were compared by independent-samples t test,Mann-Whitney U test and Chi-square test.Results Among 64 patients with EGPA,12(19％) were serum ANCA positive and 52(81％) were negative.The incidence of fever (77％ vs 35％,x2=9.403,P=0.002) and renal involvement,including proteinuria (67％ vs 25％,x2=7.678,P=0.006),hematuria (58％ vs 8％,x2=17.57,P＜0.01),renal inadequacy (33％ vs 4％,x2=9.978,P=0.002),and the BVAS score higher than 15 (92％ vs 60％,x2=4.440,P=0.035) in ANCA positive group were higher than ANCA negative group,while the presence of allergic rhinitis (17％ vs 56％,x2=5.969,P=0.015),mucocutaneous lesion (33％ vs 65％,x2=4.152,P=0.042) and cardiac involvement (8％ vs 44％,x2=3.361,P=0.021) in the ANCA-positive group was lower when compared with ANCA-negative patients.The positive ratio of rheumatoid factor (RF) (100％ vs 42％,x2=7.723,P=0.006),and the level of erythrocyte sedimentation rate (ESR) (50 vs 35.5 mm/1 h,P=0.034) in ANCA-positive group were higher than in ANCA negative group.There was no significant difference in pathological characteristics between the two groups.According to the treatment and prognosis,there were no significant differences between the two groups in the usage and dosage of steroids and immunosuppressant,the remission rate and recurrence rate of the disease,and the death rate due to the primary disease.Conclusion The clinical manifestations of EGPA are complicate.Whether ANCA is positive or not may be related to the clinical phenotypes.More attention should be paid to renal involvement in ANCA positive patients while cardiac involvement in ANCA negative patients.

ANCA-Associated Vasculitis Presenting with Hypertrophic Pachymeningitis

No abstract available.

Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody Positive Diffuse Alveolar Hemorrhage Associated with Methimazole

Diffuse alveolar hemorrhage (DAH) is a life-threatening condition associated with many disorders. Here, we report a case of 59-year-old female who had diffuse alveolar hemorrhage associated with methimazole. She had been treated with methimazole for two weeks due to the recurrence of Grave's disease, before visiting the emergency room. She had to be intubated on the 3rd day of hospitalization because of unabated massive hemoptysis and rapid progression of diffuse alveolar infiltration on chest radiographs. Since her clinical condition improved substantially after cessation of methimazole and steroid pulse therapy, she was extubated on the 9th day of hospitalization and then discharged. After discharge, DAH did not recur with cessation of steroid and she had radioactive iodine therapy for her Grave's disease. This was a rare and interesting case of life-threatening DAH associated with cytoplasmic-antineutrophil cytoplasmic antibody and methimazole.

Pathogenesis of diseases with antineutrophil cytoplasmic antibody and its clinical significance / 中国基层医药

Objective To investigate the pathogenesis of diseases with antineutrophil cytoplasmic antibody (ANCA)and its diagnostic value in primary small vessel vasculitis.Methods 57 patients with serum ANCA positive were involved in this study,and ELISA was employed to assay anti-MPO.Patients with ANCA positive,anti-MPO positive and/or anti-PR3 positive were involved in group A.Patients with ANCA positive,anti-MPO negative and anti-PR3 negative were involved in group B.X2 was used to analyze the differences between the two groups.Results The etiology of 57 ANCA positive patients included primary small vessel vasculitis (20 cases,35.1%),non inflam-matory connective tissue disease(19 cases,33.3%),non connective tissue disease(18 cases,31.6%).A group of primary small vessel vasculitis accounted for 58.6%,which was significantly higher than 10.7% of the B group (χ2 =14.354,P<0.01);while the B group of non inflammatory connective tissue disease accounted for 50%,which was significantly higher than 17.2%of the A group (χ2 =6.879,P<0.01).Conclusion ANCA should be found in many kinds of diseases,so combined detection of anti-MPO and anti-PR3 should be employed to improve the diag-nosis specificity in primary small vessel vasculitis.Furthermore,non vessel vasculitis connective tissue disease should be excluded in patients with ANCA positive,anti-MPO and anti-PR3 negative.