Oalib search

Abstract: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are characterized by destruction of small vessels, granulomatous inflammation of the respiratory tract and necrotizing glomerulonephritis. This review describes the clinical diagnosis and therapy as well as the patho-physiology of ANCA-associated vasculitides with a specific focus on the interplay of ANCAs with activated neutrophils and the deleterious pathophysiological consequences of neutrophil-endothelium interaction.

保存相关文章

Case Report: Patient with Hepatitis C, p-ANCA, and Cryoglobulin Antibodies Presenting with Necrotizing Crescentic p-ANCA Glomerulonephritis
Anjay Rastogi,Bishoy Yanny,Farid Arman,Jean Hou,Marian Kaldas,Michelle Sangalang,Naomi So,Niloofar Nobakht,Ramy M. Hanna,Sammy Saab,Umut Selamet
- , 2018, DOI: 10.1159/000491629

Abstract: Hepatitis C (HCV) infection has a prevalence of 3 million infected individuals in the United States, according to recent Center for Disease Control reports, and can have various renal manifestations. Cryoglobulins, antibodies that precipitate at colder temperatures in vitro, are a relatively common cause of renal disease in HCV infection. The cryoglobulin proteins can form occlusive aggregates in small glomerular capillary lumina or deposit in other areas of the glomerulus, resulting in hypocomplementemia, proteinuria, hematuria, and renal injury. The typical biopsy pattern is that of membranoproliferative glomerulonephritis (MPGN). There are, however, other HCV-related patterns of glomerular injury. Anti-neutrophil cytoplasmic antibodies (ANCA) are known to exist in HCV-infected patients. In many reported cases, ANCA serologic testing may appear positive due to cross-reactivity of the immune assays; however, the biopsy findings do not support ANCA-associated crescentic glomerulonephritis (GN)/vasculitis as the primary cause of glomerular injury. There are rare reports of microscopic polyangiitis (MPA) p-ANCA vasculitis, in patients with HCV infection. In comparison with the MPGN pattern of cryoglobulinemic glomerular injury, biopsies from these HCV-infected patients with concomitant MPA revealed a crescentic GN, associated with normal serum complement levels. We present a case of HCV-associated glomerular disease with the surprising biopsy finding of necrotizing and crescentic p-ANCA GN, with a background, low-grade mesangial immune complex GN. Thus, p-ANCA disease should also be considered in HCV-infected patients, in addition to the more typical lesions of MPGN or cryoglobulinemic GN

保存相关文章

肉芽肿性多血管炎伴乳房脓肿、肺脏受累1例
Granulomatosis with Polyangiitis with Breast Abscess and Lung Involvement: A Case Report [PDF]
赵欣玥, 梁睿雯
Advances in Clinical Medicine (ACM) , 2025, DOI: 10.12677/acm.2025.1571954

Abstract: 目的：报道1例肉芽肿性多血管炎合并乳房脓肿、肺脏受累患者，提高对该病的重视。病例摘要：患者中年女性，因“左乳胀痛20余天”入院。患者以乳房胀痛、破溃起病，随后出现高热、肺脓肿、结膜炎、耳廓红肿等表现。体格检查：结膜充血，双耳廓红肿，右侧为著；左侧乳头及左腋下皮肤周围破溃，可见脓性分泌物渗出。双肺呼吸音粗，可闻及局部湿性啰音。心脏、腹部查体未见异常。治疗及转归：入院先后给予“哌拉西林他唑巴坦、阿米卡星、复方磺胺甲噁唑、利奈唑胺、美罗培南”等药物抗感染治疗效果不佳；确诊后给予甲泼尼龙琥珀酸钠40 mg/d及环磷酰胺0.2 g*3次静脉滴注；后因患者出现软骨受累，将甲泼尼龙琥珀酸钠加量至60 mg/d。经治疗后患者症状减轻，复查CRP、ESR下降，胸部CT部分病灶较前范围减小；嘱出院后继续口服甲泼尼龙12 mg、每日三次，环磷酰胺50 mg隔天一次治疗。
Objective: To report a case of granulomatosis with polyangiitis complicated with breast abscess and lung involvement, and to raise awareness of this disease. Case Summary: A middle-aged female patient was admitted to the hospital due to “left breast swelling and pain for more than 20 days”. The patient presents with breast swelling and pain, followed by symptoms such as high fever, lung abscess, conjunctivitis, and swelling of the auricle. Physical Examination: Conjunctival congestion, redness, and swelling of both earlobes, with a focus on the right side. The skin around the left nipple and left armpit is ulcerated, with purulent discharge visible. The breathing sounds of both lungs are coarse, and local moist rales can be heard. No abnormalities were found during the physical examination of the heart and abdomen. Treatment and Outcome: Upon admission, the patient was given drugs such as piperacillin-tazobactam, amikacin, compound sulfamethoxazole, linezolid, and meropenem for anti-infection treatment, but the effect was not satisfactory. After diagnosis, the patient was given an intravenous infusion of methylprednisolone sodium succinate 40 mg/d and cyclophosphamide 0.2 g*3 times. Later, due to cartilage involvement in the patient, methylprednisolone succinate sodium was added at a dose of 60 mg/d. After treatment, the patient’s symptoms improved, CRP and ESR decreased upon re-examination, and some lesions on chest CT were reduced compared to before. After discharge, the patient continued to take oral methylprednisolone 12 mg three times a day and cyclophosphamide 50 mg once every other day for treatment.

保存相关文章

Atualiza？？o do tratamento das vasculites associadas a anticorpo anticitoplasma de neutrófilos
Santana, Alfredo Nicodemos Cruz;Woronik, Viktoria;Halpern, Ari Stiel Radu;Barbas, Carmen S V;
Jornal Brasileiro de Pneumologia , 2011, DOI: 10.1590/S1806-37132011000600016

Abstract: in its various forms, antineutrophil cytoplasmic antibody (anca)-associated vasculitis (aav) is characterized by a systemic inflammation of the small and medium-sized arteries (especially in the upper and lower respiratory tracts, as well as in the kidneys). the forms of aav comprise wegener's granulomatosis (now called granulomatosis with polyangiitis), microscopic polyangiitis, renal aav, and churg-strauss syndrome. in this paper, we discuss the phases of aav treatment, including the induction phase (with cyclophosphamide or rituximab) and the maintenance phase (with azathioprine, methotrexate, or rituximab). we also discuss how to handle patients who are refractory to cyclophosphamide.

保存相关文章

Clinical Course and Outcome Predictors in Pauci-Immune ANCA-Positive Renal-Limited Vasculitis
Fija？ko, Mirjana,Jaki？, Marko,Rup？i？, Vesna,Rup？i？, Vinka,Vizjak, Vedrana
- , 2011,

Abstract: Sa？etak Our aim was to assess the clinical course and outcome of ANCA-positive, pauciimmune renal limited vasculitis, their correlation with laboratory and histopathologic parameters recorded at initial and follow up testing, and to identify the possible outcome predictors. The study included 17 patients with renal biopsy, clinical, serologic and histopathologic parameters meeting the criteria for pauci-immune ANCA-positive glomerulonephritis without extrarenal manifestations of the disease. Creatinine clearance, 24-hour proteinuria and ANCA titer by ELI SA method were determined at disease onset, during treatment and at the end of follow up. In 15 patients, the diagnosis was verified by kidney biopsy. Data were processed by Spearman correlation coefficient and Mann-Whitney test, and survival by Kaplan-Meier test. Lower percentage of glomeruli affected with vasculitis, better initial renal function as measured by serum creatinine or creatinine clearance, and lower chronicity on biopsy were identified as favorable indicators of kidney function outcome. Therapy responders had highest initial and lowest final 24-h proteinuria. The highest level of final 24-h proteinuria was recorded in dialysis dependent patients. The cumulative one-year and two-year patient and kidney survival rate was 64% and 50%, and 64% and 38%, respectively

保存相关文章

Type 1 autoimmune hepatitis presenting with severe autoimmune neutropenia
Evangelos Cholongitas,George Dalekos,Ioannis Goulis,Kaliopi Zachou,Petros Doumtsis,Theodora Oikonomou
- , 2018, DOI: 10.20524/aog.2017.0186

Abstract: Autoimmune hepatitis (AIH) is a progressive, chronic liver disease characterized by unresolving hepatocellular inflammation of autoimmune origin. The clinical spectrum may vary from asymptomatic presentation, to non-specific symptoms such as fatigue, arthralgias, nausea and abdominal pain, to acute severe liver disease. AIH is characterized by the presence of interface hepatitis and portal plasma cell infiltration on histological examination, hypergammaglobulinemia, and positive autoantibodies. AIH is associated with other autoimmune diseases and its course is often accompanied by various non-specific hematological disorders. However, the coexistence of autoimmune neutropenia (AIN) is infrequent. We present a case of a female patient diagnosed with type 1 AIH and agranulocytosis on presentation. A diagnosis of AIN was established, based on the patient’s sex, the underlying liver disease, the absence of alternative causes, the presence of atypical anti-neutrophil cytoplasmic antibodies in patient’s serum and the favorable and dose-dependent treatment of both pathologic entities with corticosteroids and mycophenolate mofetil

保存相关文章

Cutaneous Vasculitis as Early Presentation of Eosinophilic Granulomatosis with Polyangiitis without Systemic Symptoms
Aris Chandran Abdullah,Jyi Jong Tang,Norain Karim,Sin Fah Chung,Wahinuddin Sulaiman
- , 2019, DOI: 10.5001/omj.2019.67

Abstract: Eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) is a rare vasculitis in children. It commonly presents with respiratory symptoms, especially asthma, allergic rhinitis, and peripheral eosinophilia. Involvement of other systems, such as renal and cardiac, may carry a poor prognosis. Anti-neutrophil cytoplasmic antibodies have been found less frequently in children with this condition. We report a case of a 15-year-old male who presented with indurated and pruritic papules on both legs and peripheral eosinophilia without other system involvement. Histopathological findings from a skin biopsy were suggestive of eosinophilic granulomatosis with polyangiitis. Anti-neutrophil cytoplasmic antibodies were negative

保存相关文章

抗中性粒细胞胞质抗体相关性血管炎继发间质性肺病的研究进展
Research Progress of Anti-Neutrophil Cytoplasmic Antibodies Associated Vasculitis Secondary Interstitial Lung Disease [PDF]
席磊, 白祥梅, 杨柳, 王玲
Advances in Clinical Medicine (ACM) , 2023, DOI: 10.12677/ACM.2023.133428

Abstract: 抗中性粒细胞胞质抗体(Anti-Neutrophil Cytoplasm Antibody, ANCA)相关性血管炎(Anti-neutrophil cytoplasmic Antibodies Associated Vasculitis, AAV)是结缔组织疾病(Connective Tissue Disease, CTD)相关的间质性肺疾病(Interstitial Lung Disease, ILD)的一种，因其发病机制尚且不能明确，诊断较为困难，复发率高等特点，目前仍需要进一步研究，本文将从近年来AAV-ILD的发病机制、血清标志物及治疗等方面的研究进展进行系统性论述。
Anti-neutrophil cytoplasmic antibodies associated vasculitis is a kind of interstitial lung disease related to connective tissue diseases. Due to its unclear pathogenesis, difficult diagnosis and high recurrence rate, further research is still needed. This paper will systematically discuss the patho-genesis, serum markers and treatment of AAV-ILD in recent years.

保存相关文章

ANCA: Serology in Wegener′s granulomatosis
Pradhan Vandana,Badakere S,Ghosh K,Almeida A
Indian Journal of Medical Sciences , 2005,

Abstract: BACKGROUND AND OBJECTIVES Wegener′s granulomatosis (WG) is being increasingly diagnosed in India, which exists in two forms, the ′limited Wegener′s granulomatosis′ (LWG) having upper respiratory tract (URT) and lower respiratory tract (LRT) involvement and the ′classical Wegener′s granulomatosis′ (CWG), with the triad of URT, LRT involvement along with kidney involvement. Cytoplasmic ANCA (C-ANCA) or anti-Proteinase3 (anti-PR3), which is highly diagnostic for WG, rarely perinuclear ANCA (P-ANCA) may exist. AIMS To detect anti-neutrophil cytoplasmic antibodies (ANCA) and correlate it with serological, hematological parameters, and the Birmingham Vasculitis Activity Score (BVAS). SETTINGS AND DESIGN Twenty-three clinically and histopathologically proven WG (16 CWG, 7 LWG) were studied. MATERIAL AND METHODS C-ANCA and P-ANCA patterns were identified by immunofluorescence and specificities were confirmed by ′α granule′ enzyme linked immunosorbent assay (ELISA), anti-PR3, anti-MPO (myeloperoxidase) and anti-Lactoferrin (anti-LF) by ELISA. RESULTS LRT involvement was seen in 91.3%, URT in 78.3%, and renal manifestations in 69.6% cases. The BVAS in CWG was significantly higher than BVAS in the LWG. Decreased hemoglobin, increased WBC counts, ESR, CRP and Creatinine were seen in CWG as compared to LWG. The C-ANCA was present in 65.2% patients and P-ANCA in 13% cases. Anti-PR3 was seen in 69.6% patients and anti-LF in 17.4% cases. Severity of disease and ANCA was higher in CWG than in LWG. CONCLUSIONS Vasculitis syndromes are known to overlap and many go undetected; therefore ANCA testing, along with the clinical and histopathological observations may be helpful in early detection and management of WG cases.

保存相关文章

第1页/共1000条

每页显示条