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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.
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The indirect immunofluorescence (IIF) technique for antineutrophil cytoplasmic antibodies (ANCA) detection is subject to substantial differences across laboratories. This study aimed to assess the impact of improvements in the IIF-ANCA technique on the positivity rate of ANCA tests. A cross-sectional study was performed with serum samples from patients with ANCA-associated vasculitis (AAV), autoimmune hepatitis (AIH), and ulcerative colitis (UC). A paired analysis was performed for IIF-ANCA results using the traditional method and a modified protocol after a series of specific adjustments in the technique based on the protocol of IIF-ANCA test performed at a nation-wide private laboratory in Brazil. ANCA specificity was assessed by ELISA for anti-proteinase 3 (PR3) and anti-myeloperoxidase (MPO) antibodies. Sixty-one patients were evaluated. The positivity rate of IIF-ANCA tests at disease presentation performed at the University reference laboratory was 32.3% in AAV, AIH, and UC patients, whereas the positivity rates of IIF-ANCA and ELISA tests in other laboratories were 75.0 and 72.7%, respectively. After modifications in the IIF-ANCA technique, there was a significant increase in the positivity rate (14.8 vs 34.3%; P=0.0002) and in median titers [1/40 (1/30-1/160) vs 1/80 (1/40-1/80); P=0.0003] in AAV, AIH, and UC patients. UC had the highest increment in positive results from 5.3 to 36.8%. There was poor agreement between MPO- or PR3-ANCA and both IIF-ANCA techniques. In conclusion, modifications in the IIF-ANCA protocol led to a significant improvement in its positivity rate and titers.
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The clinical manifestations of subacute combined degeneration of spinal cord (SCD) in children are complex and vary greatly. Due to the fact that some patients with SCD may be complicated with autoimmune diseases, the high early misdiagnosis and missed diagnosis rates are observed. One case of 13-year old female with severe anemia, multiple joint swelling and pain in left limbs and paralysis of bilateral lower limbs with the extremely low level of serum vitamin B12 and poly-glandular involvement as well as a variety of positive auto-antibodies (anti-intrinsic factor antibody, anti-parietal cell antibody, thyroid peroxidase antibody, thyroid globulin antibody and perinuclear anti-neutrophil cytoplasmic antibody) was retrospectively analyzed. The patient was diagnosed as SCD with autoimmune disease (undifferentiated connective tissue disease and autoimmune polyglandular syndrome). The patient′s condition gradually alleviated after high-dose intravenous methylprednisolone, immunoglobulin, naproxen (then changed to hydroxychloroquine 1 month later), vitamin B12 and levothyroxine sodium tablets supplementation, blood transfusion and rehabilitation. SCD with autoimmune diseases is rare in children, and the clinical manifestations vary greatly. Early recognition and early treatment can improve the prognosis of SCD. The clinical data of this child were retrospectively analyzed, so as to improve the understanding of the disease by clinicians.
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Renal involvement in COVID-19 infection is varied and worsens its outcome and prognosis. However, the association of COVID-19 infection with glomerulonephritis is exceptional. We report a 46-year-old woman with COVID-19 who had an acute kidney injury and ANCA associated glomerulonephritis two weeks after the onset of the disease. The kidney biopsy showed a crescentic glomerulo-nephritis and the presence of anti-glomerular basement membrane antibodies (GBM-Abs). She was treated with steroids and oral cyclophosphamide with good response without requiring plasmapheresis. Plasma anti GBM-Abs were negative. This case suggests that the presence of anti-GBM-Abs in the kidney, was temporally related to COVID-19 pulmonary damage. The absence of plasma antibodies is probably due to transient production and glomerular adsorption, but with unknown pathogenic role.
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Humans , Female , Middle Aged , COVID-19/complications , Glomerulonephritis/complications , Autoantibodies , Basement Membrane/pathology , Antibodies, Antineutrophil CytoplasmicABSTRACT
ABSTRACT Introduction: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Patients with SLE exhibit multiple serum autoantibodies, including anti-neutrophil cytoplasmic antibodies (ANCAs). There are two main techniques to detect ANCAs: indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assay (ELISA). In this study, an attempt was made to determine the frequency and clinical associations of ANCAs in patients with SLE. Methods: A cross-sectional study was conducted in a tertiary care hospital in Colombia that included 74 patients with SLE. The presence of ANCAs was assessed using IIF with ethanol-fixed slides, and ELISA was used to detect antibody specificities for myeloperoxidase (MPO)-ANCA and proteinase 3 (PR3)-ANCA. Results: Of the 74 patients with SLE evaluated, 60 (81.1%) of them were ANCA-positive by IIF. By contrast, only one patient showed specificity for PR3-ANCA by ELISA. The relevance of ANCA positivity by IIF and clinical and serological features was significant for renal involvement (p = .0174), and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) (p = .0308). Conclusion: ANCAs are common in the serum of patients with SLE, as detected by ethanol-fixed slides with IIF staining. However, detection of specificity to PR3 and/or MPO is rare, thus highlighting the importance of detecting these autoantibodies by different techniques.
RESUMEN Introducción: El lupus eritematoso sistémico (LES) es una enfermedad autoinmune sistémica. Los pacientes con LES muestran múltiples autoanticuerpos séricos, incluyendo los anticuerpos anticitoplasma de neutrófilo (ANCA, por sus siglas en inglés). Existen 2 técnicas principales para la detección de ANCA: inmunofluorescencia indirecta (IFI) y ensayo por inmunoadsorción ligado a enzimas (ELISA). En este estudio nuestro objetivo fue determinar la frecuencia y las asociaciones clínicas de los ANCA en pacientes con LES. Métodos: Realizamos un estudio transversal de 74 pacientes con LES en un hospital de alta complejidad de Colombia. La presencia de ANCA se evaluó por IFI, utilizando láminas con fijación de etanol, y con ELISA para determinar las especificidades para mieloperoxidasa (MPO)-ANCA y proteinasa 3 (PR3)-ANCA. Resultados: Fueron evaluados 74 pacientes con LES, 60 (81,1%) de ellos fueron positivos para ANCA. Por el contrario, solo un paciente mostró especificidad para PR3-ANCA por ELISA. La relación entre la positividad para ANCA por IFI y las características clínicas y serológicas fue estadísticamente significativa para compromiso renal (p = 0,0174) y para el índice de actividad de la enfermedad (Systemic Lupus Erythematosus Disease Activity Index [SLEDAI]) (p = 0,0308). Conclusiones: Los ANCA detectados mediante fijación con etanol por técnicas de IFI, son comunes en pacientes con LES. Sin embargo, la detección de especificidades para PR3 o MPO es rara; se destaca la importancia de la evaluación de estos autoanticuerpos mediante diferentes técnicas.
Subject(s)
Humans , Adult , Immunoproteins , Blood Proteins , Skin and Connective Tissue Diseases , Connective Tissue Diseases , Antibodies, Antineutrophil Cytoplasmic , Amino Acids, Peptides, and Proteins , Lupus Erythematosus, SystemicABSTRACT
Abstract Antineutrophil cytoplasmic antibodies (ANCAs) are associated with small vessel vasculitis but their prevalence is not rare in other immune diseases. In lupus nephritis (LN), their pathological role and clinical relevance have been the target of controversial views. We present a case of acute kidney injury and nephrotic syndrome in a young woman with diffuse global proliferative and membranous nephritis on her kidney biopsy, showing a full-house immunofluorescence pattern, very allusive of class IV + V LN, but lacking associated clinical criteria and laboratory findings to support the diagnosis of systemic lupus erythematosus (SLE). Furthermore, the patient presented with high titers of ANCA, steadily decreasing alongside the renal function and proteinuria improvements, with mycophenolate mofetil (MMF) and steroid treatment. The authors believe this is a case of lupus-like nephritis, in which ANCAs are immunological markers, although they are not directly involved in the pathogenesis.
Resumo Os anticorpos anticitoplasma de neutrófilos (ANCAs) estão associados à vasculite de pequenos vasos, no entanto, a sua prevalência não é rara em outras doenças imunológicas. Na nefrite lúpica (LN), o seu papel patológico e relevância clínica têm sido alvo de pontos de vista controversos. Apresentamos um caso de lesão renal aguda e síndrome nefrótica em uma jovem com nefrite proliferativa difusa e membranosa em sua biópsia renal, muito alusivo a NL classe IV + V, com um padrão full house na imunofluorescência, mas sem critérios clínicos e achados laboratoriais para corroborar o diagnóstico de lúpus eritematoso sistêmico (LES). Não obstante, a paciente apresentou títulos elevados de ANCA, que diminuiram progressivamente com a melhoria da função renal e da proteinúria, após tratamento com micofenolato de mofetil (MMF) e esteróide. Os autores acreditam que se trata de um caso de nefrite semelhante à nefrite lúpica, em que os ANCAs são marcadores imunológicos, embora não estejam diretamente envolvidos na patogênese.
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Abstract Some cases of patients with IgA nephropathy diagnosed via kidney biopsy and antineutrophil cytoplasmic antibody (ANCA) positivity have been reported. This article describes a case series comprising patients with IgA nephropathy and ANCA positivity seen at a medical center in the city of São Paulo, Brazil, from 1996 to 2016. A total of 111 patients underwent diagnostic kidney biopsies for IgA nephropathy. Five were ANCA-positive at the time of diagnosis; their mean age was 45 ± 15.3 years and they were predominantly females with a mean proteinuria of 2.2 ± 0.9 g/day and a median serum creatinine level of 2.5 (2.0 - 8,6) mg/dL; all had hematuria. Four of the five were cANCA-positive (80%); all had normal serum C3 and C4 levels; and 80% were positive for ANA. One case presented an association with infection, but no associations were found with medication. One patient had granuloma and another had a collapsing lesion. This article describes the cases of five ANCA-positive patients (with predominantly cANCA positivity) submitted to diagnostic kidney biopsies for IgA nephropathy; one patient had a collapsing lesion, but progressed well.
Resumo Alguns casos clínicos de biópsia renal diagnóstica de nefropatia por IgA em pacientes com títulos séricos positivos de anticorpo anticitoplasma de neutrófilos (ANCA) vêm sendo publicados. Descreve-se uma série de casos de nefropatia por IgA com ANCA positivo de centro único da cidade de São Paulo, Brasil, no período de 1996 a 2016. No período estudado, houve 111 pacientes com biópsia renal com diagnóstico de nefropatia por IgA; destes, 5 tinham ANCA positivo ao diagnóstico com média de idade de 45 ± 15,3 anos, predominando o sexo feminino, com média de proteinúria de 2,2 ± 0,9 g/dia, hematúria presente em 100% dos casos e mediana de creatinina sérica de 2,5 (2,0 - 8,6) mg/dL. O cANCA foi o padrão mais encontrado, em 4 dos 5 casos (80%), com os níveis séricos das frações de complemento C3 e C4 normais em todos e FAN positivo em 80% dos casos. Houve associação com infecções em um caso, mas sem associação com medicações. À microscopia óptica, um dos pacientes tinha granuloma e outro, lesão colapsante. Em resumo, descreve-se cinco casos de pacientes com biopsia renal diagnóstica de nefropatia por IgA com ANCA sérico positivo predominando cANCA, destacando um paciente desse grupo com microscopia óptica com lesão colapsante que, apesar disso, teve boa evolução.
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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.
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Objective:To study the clinical features of infective endocarditis (IE) with positive anti-neutrophil cytoplasmic antibodies (ANCA) in order to improve the level of diagnosis and treatment.Methods:Eighteen IE cases with positive ANCA admitted to the First Affiliated Hospital of Zhengzhou University from June 2016 to July 2021 were collected. The demographic information, clinical symptom, laboratory tests, imaging examinations, treatment and clinical outcomes were analyzed retrospectively. Statistical program for social sciences (SPSS) 20.0 statistical software was used for analysis. Enumeration data were expressed as the number of cases and percentage (%), and measurement data were expressed as Mean± SD. Results:Twelve cases were male and 6 cases were female, with an average age of (50±16) years. Sixteen patients had positive PR3-ANCA, in which 2 cases had positive myeloperoxidase (MPO)-ANCA. The major clinical manifestations included fever (88.9%, 16/18), anemia (72.2%, 13/18), splenomegaly (44.4%, 8/18), cardiac murmur (33.3%, 6/18), arthralgia (22.2%, 4/18), liver damage (22.2%, 4/18), thromboembolic events (16.7%, 3/18), Osler's node (11.1%, 2/18) and renal dysfunction (11.1%, 2/18). Higher C-reactive protein (CRP), erythrocyte sedimentation (ESR) and procalcitionin (PCT) were detected in 83.3% (15/18) patients. The positive rate of blood culture was 50.0%(9/18) and streptococcus was the most common pathogen (77.8%, 7/9). Echocardiograms of all patients showed abnormal vegetation, most commonly involving the mitral valve (66.7%, 12/18) and aortic valve (33.3%, 6/18). Two patients were misdiagnosed as ANCA associated vasculitis (AAV), but the other one was diagnosed as AAV with IE as the first manifestation. Except for one case who died of multiple organ failure, all cases reached clinical recovery after surgery and antibiotic therapy.Conclusion:IE patients with positive ANCA may present with the clinical manifestations similar to AAV. We should highly alert to avoid misdiagnosis and treatment.
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Objective:To explore the clinical and histopathologic features of lupus nephritis (LN) patients with positive antineutrophil cytoplasmic antibody (ANCA), so as to provide more theoretical basis to recognize and treat this disease.Methods:Clinical data of biopsy-proven LN patients with ANCA test in the First Affiliated Hospital of Sun Yat-sen University from November 1, 2002 to September 11, 2020 were collected and analyzed retrospectively. The difference of clinical data, laboratory examination, and pathological examination of renal biopsy between ANCA-positive group and ANCA-negative group. The clinicopathological characteristics between different ANCA serotypes were compared.Results:A total of 1 304 patients with LN confirmed by renal biopsy and ANCA test results were enrolled. Eighty ANCA-positive patients from 1 304 LN patients were screened. There are 55(68.8%) ANCA-positive LN patients with positive anti-myeloperoxidase antibodies (MPO). There were 14(17.5%) ANCA-positive LN patients with positive anti-proteinase 3 antibodies (PR3), and 11(13.8%) ANCA-positive patients with double positive antibodies of MPO and PR3. ANCA-positive LN patients had significantly higher serum creatinine [135.5(68.0, 361.8) μmol/L vs 88.0(64.0, 165.0) μmol/L, P=0.004] and blood urea nitrogen [12.35(6.35, 21.18) mmol/L vs 8.60 (5.50, 15.70) mmol/L, P=0.026] as well as lower estimated glomerular filtration rate [45.70(13.83, 84.10) ml·min -1·(1.73 m 2) -1 vs 66.75(38.43, 96.22) ml·min -1·(1.73 m 2) -1, P=0.001] than ANCA-negative patients (stratified sampling of 160 patients). ANCA-positive LN patients had higher chronicity index than ANCA-negative LN patients [3(2, 7) vs 2(0, 5), P=0.006]. There were statistically significant difference in hemoglobin, serum creatinine and estimated glomerular filtration rate among ANCA-positive group, ANCA-negative group, and MPO-ANCA and PR3-ANCA double positive group. MPO-ANCA and PR3-ANCA double positive LN patients had the lowest hemoglobin and estimated glomerular filtration rate, and highest serum creatinine among the three groups (all P<0.05). Conclusions:ANCA-positive LN patients have worse renal function and higher renal histological chronicity index than ANCA-negative LN patients, especially for patients with double positive MPO-ANCA and PR3-ANCA. More stringent monitoring and therapy may be needed in this subgroup of LN patients.
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Objective:To investigate the characteristics and risk factors of infection in newly diagnosed patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods:The clinical data of AAV patients (followed up for at least 6 months) in Affiliated Hospital of Qingdao University from September 2012 to September 2020 were retrospectively collected. According to whether infection occurred during follow-up, the patients were divided into infection group and non-infection group. The clinical characteristics and infection status of the two groups were analyzed, and the Cox regression analysis model was used to explore the influencing factors of infection.Results:A total of 236 AAV patients were enrolled in this study, including 128 females (54.2%) and 108 males (45.8%), with a median age of 66.00 (59.76, 71.99) years. There were 202 patients (85.6%) with positive myeloperoxidase (MPO)-ANCA and 34 patients (14.4%) with positive protease 3 (PR3) -ANCA. There were 77 cases in the infection group and 159 cases in the non-infection group. A total of 121 infections occurred in 77 patients, and 54 infections (44.6%) occurred within 6 months after initial diagnosis. In the infection group the proportion of patients with hypertension history, pulmonary underlying diseases and patients who received hormone pulse therapy or plasma exchange, the incidence of lung, kidney, heart and gastrointestinal involvement, the level of serum creatinine and five factors score (FFS) at initial diagnosis were significantly higher than those in the non-infection group (all P<0.05), while the estimated glomerular filtration rate (eGFR) was significantly lower ( P<0.05). Lung (73.6%) was the main infection organ of AAV patients. The most common pathogenic microorganisms were bacteria (64.0%), mainly Pseudomonas aeruginosa and Staphylococcus aureus, followed by fungi (33.7%, mainly Candida albicans). Multivariate Cox regression analysis showed that lung involvement ( HR=1.682, 95% CI 1.034-2.734, P=0.036) and gastrointestinal involvement ( HR=2.976, 95% CI 1.219-7.267, P=0.017) were the independent influencing factors for infection in AAV patients. Conclusions:AAV patients have a higher incidence of infection within 6 months after initial diagnosis. The most common organ of infection in AAV patients is the lung, and the common pathogens are Pseudomonas aeruginosa, Staphylococcus aureus and Candida albicans. Lung involvement and gastrointestinal involvement are the independent risk factors for infection in AAV patients.
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Objective:To investigate the clinical manifestations, pathological characteristics, treatment and prognosis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) in 13 children.Methods:The clinical and pathological data of 13 cases of AAV in children′s Hospital of Nanjing Medical University from June 2000 to December 2021 were retrospectively analyzed.Results:Among the 13 cases, 12 cases were diagnosed with microscopic polyangiitis (MPA) and 1 case was granulomatosis with polyangiitis (GPA), including 10 females and 3 males. The onset age ranged from 3 years and 11 months to 13 years and 10 months. The most frequently involved organ was the kidney (12 cases, 92.3%), followed by respiratory system (7 cases, 53.8%), skin (5 cases, 38.5%), digestive system (4 cases, 30.8%), nervous system (4 cases, 30.8%) and cardiovascular system (3 cases, 23.1%). There were 10 cases with orthotic anemia, 7 cases with positive antinuclear antibody, and 3 cases with mildly decreased complement C3. Among the 12 children with renal impairment, 9 cases were accompanied by abnormal renal function at the beginning of the disease. Renal biopsy was classified according to the Berden as follows: sclerotic in 5 cases, crescentic 3 cases, focal in 2 cases and mixed in 2 cases. All children were treated with glucocorticoid combined with immunosuppressant. During the follow-up time from 8 months to 128 months, 4 cases acquired complete remission, 8 cases achieved partial remission and 1 case recurred after complete remission, and 7 cases progressed to chronic kidney disease stage 5. Three children with complete remission underwent repeated renal biopsy, including 2 cases of mixed type and 1 case of crescent type initially, and all changed to focal type.Conclusions:AAV in children occurs mainly in school-age female, and most of AAV in children is MPA. The clinical manifestations are various. Most of them have renal damage and anemia, and lung damage is also common. Patients with skin purpura onset may be misdiagnosed as Henoch-Schonlein purpura, and AAV with ANA positive or complement reduction should exclude systemic lupus erythematosus. Once the renal function is abnormal in AAV, especially estimated glomerular filtration rate<60 ml·min -1·(1.73 m 2) -1 and the pathological classification is sclerotic type or crescent type, it is difficult to reverse even after active treatment. Early diagnosis and treatment are very important for AAV.
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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.
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Objective: Cardiac arrest (CA) has been observed in some patients with Takotsubo syndrome (TTS), most of whom had CA at the initial presentation of TTS. The objective of this report was to discuss the factors underlying the onset of this syndrome.Case presentation: A 72-year-old woman with refractory antineutrophil cytoplasmic autoantibody-associated lung disease was referred to our hospital. Twenty minutes after bronchoscopic examination, cardiopulmonary arrest suddenly occurred. Resuscitation immediately resumed her heartbeat and spontaneous breathing. Subsequent 12-lead electrocardiography, echocardiography, and left ventricular angiography revealed TTS.Conclusion: This case indicates that bronchoscopy can cause severe TTS, especially in patients with systemic inflammation.
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Objetivo: Estimar a prevalência do anticorpo anticitoplasma de neutrófilos (ANCA) positivo em pacientes submetidos à hemodiálise, assim como também associar essa prevalência às características sociodemográficas e clínicas destes pacientes. Métodos: Estudo transversal realizado em 78 pacientes de uma Clínica de Hemodiálise no Sul de Santa Catarina nos meses de agosto a novembro de 2013. Foi realizada entrevista, coleta de dados em prontuário eletrônico e pesquisa do anticorpo anticitoplasma de neutrófilos por imunofluorescência indireta WAMA em plasma heparinizado. Resultados: A prevalência de anticorpo anticitoplasma de neutrófilos reagente em amostra titulada 1:10 foi de 24,4%, sendo que 10,3% apresentaram padrão c-ANCA e 14,1% apresentaram padrão p-ANCA, do total. Não foram observadas associações com características clínicas e sociodemográficas. Conclusão: Com base na alta prevalência de anticorpo anticitoplasma de neutrófilos positivo encontrada neste estudo, salienta-se a necessidade de mais estudos nesse grupo de pacientes para definição da contribuição deste exame no diagnóstico e prognóstico da doença renal e suas complicações
Objective: To estimate the Antibodies, Antineutrophil Cytoplasmic (ANCA) prevalence in patients submitted to hemodialysis, as well as associate this prevalence to sociodemographic and clinical characteristics of these patients. Methods: Cross-sectional study performed with 78 users from a Hemodialysis Clinic in Southern Santa Catarina on the period from August to November 2013. Interviews, data collection of electronic records and antibodies, antineutrophil cytoplasmic research by WAMA indirect immunofluorescence on heparinized plasma were performed. Results: The prevalence of ANCA reagent 1:10 titrated sample was 24.4%, and 10.3% had c-ANCA pattern and 14.1% had p-ANCA pattern, from total sample. No associations were observed with clinical and sociodemographic characteristics. Conclusions: Based on the high antibodies, antineutrophil cytoplasmic prevalence in this study, and no statistically significant association with clinical and sociodemographic characteristics, we emphasize the need for more studies on this group of patients to determine the contribution of this exam in the diagnosis and prognosis of kidney disease and its complications.
Subject(s)
Humans , Renal Dialysis , Antibodies, Antineutrophil Cytoplasmic , Renal Insufficiency/complicationsABSTRACT
ABSTRACT BACKGROUND: Perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) and anti-Saccharomyces cerevisiae antibodies (ASCAs) have long been used to differentiate between Crohn's disease (CD) and ulcerative colitis (UC), more recently having been used as prognostic indicators. OBJECTIVE: To determine the diagnostic accuracy of serological markers in the identification of pediatric CD and UC in Sao Paulo, Brazil, as well as to correlate those markers with characteristics demographic and clinical of these two diseases. METHODS: Retrospective cross-sectional multi-center study involving pediatric patients with inflammatory bowel disease (IBD). We identified ASCAs serological markers and p-ANCA, correlating their presence with demographic and clinical data, not only in the patients with IBD but also in a group of age-matched gastrointestinal disease-free controls. RESULTS: A total of 122 patients, 74 with IBD (46% males), treated at four pediatric gastroenterology referral centers, the mean age of 13±7 years, 49 (66%) with CD, and 25 (34%) with UC. The control Group comprised 48 patients (54% males). The proportion of patients testing positive for p-ANCA was significantly higher in the UC group (69.9%) compared to the CD group (30.4%), as well as being significantly higher in the CD group versus the control Group (P<0.001 for both). The proportion of patients testing positive for ASCA IgA (76.2%) and ASCA IgG (94.4%) markers was also significantly higher in the CD group than in the control Group (P<0.001), and such positivity correlated significantly with the use of immunomodulatory medications such as azathioprine and anti-tumor necrosis factor agents (azathioprine 38.9%, anti-TNF 55.6%; P=0.002). In the CD group, the proportion of patients testing positive for the ASCA IgA was significantly higher among those who underwent surgery than among those who did not (26.86±17.99; P=0.032). CONCLUSION: In pediatric patients with IBD in Sao Paulo, Brazil, serological tests proving to be highly specific, although not very sensitive, for the diagnosis of IBD. However, the serological markers showed a positive correlation with the severity of the disease.
RESUMO CONTEXTO: Os anticorpos citoplasmáticos anti-neutrófilos perinuclear (p-ANCA) e anticorpos anti-Saccharomyces cereviciae (ASCAs) são utilizados para diferenciar a doença de Crohn (DC) da colite ulcerativa (CU) e mais recentemente para correlacioná-los com o prognóstico da doença. OBJETIVO: 1) Determinar a acurácia diagnóstica dos marcadores sorológicos na identificação de DC e CU pediátrica em São Paulo, Brasil. 2) Correlacioná-los com as características demográficas e clínicas destas duas doenças. MÉTODOS: Estudo multicêntrico transversal em pacientes com diagnóstico estabelecido de doença inflamatória intestinal (DII) determinando a presença dos marcadores sorológicos ASCAs e p-ANCA, correlacionando seus resultados com os dados demográficos e clínicos, e também em pacientes controles isentos de doenças gastrointestinal. RESULTADOS: 122 pacientes, 74 com DII (46% masculinos) em quatro centros de referência em Gastroenterologia Pediátrica, média de idade 13±7 anos, 49 (66%) com DC e 25 (34%) com CU e 48 controles (54% masculinos). O marcador p-ANCA apresenta maior porcentagem de detecção na CU (69,6%), mas também na DC (30,4%) quando comparado ao grupo controle (P<0,001). Os marcadores ASCA IgA (76,2%) e IgG (94,4%) apresentam maiores porcentagens de detecção na DC, quando comparada ao controle (P<0,001) e que a positividade do marcador esteve relacionada ao uso de medicações em pacientes portadores de DC que realizaram cirurgia (26,86±17,99; P=0,032). CONCLUSÃO: Os resultados dos testes sorológicos em crianças com DII em São Paulo, Brasil, foram altamente específicos, mas pouco sensíveis para auxiliar no diagnóstico, embora com correlação positiva com a gravidade da doença.
ABSTRACT
La vasculitis asociada a propiltiouracilo (PTU) y ANCA es una enfermedad rara con una incidencia de 0,47-0,71 casos por cada 10 000 pacientes. Afecta principalmente a los vasos de menor calibre. Piel, riñones y pulmones son los más afectados. El diagnóstico requiere alta sospecha clínica y se apoya en estudios histopatológicos de las lesiones en piel y serología positiva para ANCA. La suspensión del medicamento suele bastar como manejo de la enfermedad. Mujer de 45 años de edad, con historia de consumo de PTU durante 2 años, desde su diagnóstico de hipertiroidismo; presentando placas purpúricas dolorosas en ambos miembros inferiores.
SUMMARY ANCA vasculitis associated to propylthiouracil (PTU) is a rare condition with an incidence of 0.47-0.71 cases per 100,000 patients. It affects mainly minor caliber blood vessels. The skin, kidneys and lungs are the organs most affected. To make the diagnosis it is necessary to have a high index of suspicion and rely on histopathologic findings of skin lesions plus a positive ANCA test. Stopping the inducing drug is usually necessary to manage this condition. We report the case of a 45-year-old woman who consumed PTU for two years for the treatment of hyperthyroidism presenting with painful purpuric plaques in the lower limbs.
ABSTRACT
Objective:To determine the prognostic values of clinical and laboratory features at the time of presentation on renal survival of patients with myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody(ANCA)-associated glomerulonephritis (MPO-ANCA-GN).Methods:A total of 172 patients with MPO-ANCA-GN and hospitalized at the First Affiliated Hospital of Nanjing Medical University from January 2005 to December 2018 were enrolled. The baseline clinical characteristics and renal biopsy pathological data were analyzed, and the renal prognosis was followed up. The clinical and pathological characteristics of different renal prognosis in all patients and 112 patients who underwent renal biopsy were analyzed, and the related factors affecting renal survival were further discussed.Results:Among these 172 patients, 81 were males and 91 were females. The median serum creatinine at diagnosis was 343.7(174.2, 606.6) μmol/L and the median estimated glomerular filtration rate (eGFR) was 15.81(7.61, 38.04) ml·min -1·(1.73 m 2) -1. In total, 76 patients (44.2%) received initial renal replacement therapy (RRT). During a median follow-up duration of 20(3, 60) months, 73 patients (42.4%) progressed to end-stage renal disease (ESRD) and required dialysis, including 6 (8.2%) patients who entered RRT during follow-up and 67 (91.8%) patients who received RRT at the beginning. Among the 112 patients who underwent renal biopsy, the proportion of patients who progressed to ESRD in the sclerotic group was the highest (15/25, 60.0%). The baseline serum creatinine level ( P<0.001), urine red blood cell count ( P=0.012) and the proportion of glomerular sclerosis ( P=0.002) in the non-dialysis dependent group were significantly lower than those in the dialysis dependent group, while the levels of eGFR ( P<0.001), serum albumin ( P=0.002) and hemoglobin ( P<0.001) were higher than those of the dialysis-dependent group. Kaplan-Meier survival analysis showed that the renal survival rate of the focal group was the highest ( χ2=19.488, P<0.001, log-rank test), while the renal survival rate of the sclerotic group was significantly lower than that of the crescentic group ( χ2=5.655, P=0.017); higher levels of serum creatinine (>320 μmol/L, χ2=77.229, P<0.001) and urine red blood cell count (>300 cells/μl, χ2=8.511, P=0.004), lower levels of rheumatoid factor (<20 IU/ml, χ2=8.610, P=0.003), serum albumin (<30 g/L, χ2=11.060, P=0.001) and hemoglobin (<90 g/L, χ2=21.921, P<0.001) were associated with lower renal survival rate; in terms of treatment, the renal survival rate of the glucocorticoids plus mycophenolate mofetil group was significantly higher than that of the glucocorticoids plus cyclophosphamide ( χ2=5.056, P=0.025) or the glucocorticoids alone group ( χ2=16.459, P<0.001). Multivariate Cox regression showed that baseline serum creatinine >320 μmol/L ( HR=8.803, 95% CI 3.087-25.106, P<0.001) and serum albumin <30 g/L ( HR=2.566, 95% CI 1.246-5.281, P=0.011) were the related factors affecting renal survival. Conclusion:Serum creatinine and albumin levels of MPO-ANCA-GN patients at diagnosis may be the related factors that affect the patient's renal prognosis.
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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.
ABSTRACT
OBJECTIVES: To determine the frequency of the antineutrophil cytoplasmic antibodies (ANCA), antiproteinase-3 and antimyeloperoxidase, in primary sclerosing cholangitis (PSC) with or without inflammatory bowel disease (IBD+ or IBD-) and in different types of autoimmune hepatitis (AIH). Additionally, to verify the agreement between ANCA patterns by indirect immunofluorescence and their antigenic specificities by ELISA. METHODS: For this study, 249 patients were enrolled (42 PSC/IBD+; 33 PSC/IBD-; 31 AIH type-1; 30 AIH type-2; 31 AIH type-3; 52 primary biliary cirrhosis; 30 healthy controls) whose serum samples were tested for ANCA autoantibodies. RESULTS: There were fewer female subjects in the PSC/IBD- group (p=0.034). Atypical perinuclear-ANCA was detected more frequently in PSC/IBD+ patients than in PSC/IBD- patients (p=0.005), and was significantly more frequent in type-1 (p<0.001) and type-3 AIH (p=0.012) than in type-2 AIH. Proteinase-3-ANCA was detected in 25 samples (only one with cytoplasmic-ANCA pattern), and more frequently in PSC/IBD+ than in PSC/IBD- patients (p=0.025). Myeloperoxidase-ANCA was identified in eight samples (none with the perinuclear-ANCA pattern). Among the 62 reactive samples for atypical perinuclear-ANCA, 13 had antigenic specific reactions for proteinase-3 and myeloperoxidase. CONCLUSIONS: PSC/IBD+ differed from PSC/IBD- in terms of sex and proteinase 3-ANCA and atypical perinuclear-ANCA reactivity, the latter of which was more frequently detected in type-1 and type-3 AIH than in type-2 AIH. There was no agreement between ANCA patterns and antigenic specificities in IBD and autoimmune liver diseases, which reinforces the need for proteinase-3 and myeloperoxidase antibody testing.