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Ronald J. Falk - One of the best experts on this subject based on the ideXlab platform.
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Necrotizing Arteritis and Small Vessel Vasculitis
The Autoimmune Diseases, 2014Co-Authors: J. Charles Jennette, Ronald J. FalkAbstract:Publisher Summary There are three major categories of systemic Vasculitis—namely, large Vessel Vasculitis or chronic granulomatous arteritis, medium-sized Vessel Vasculitis or necrotizing arteritis, and Small Vessel Vasculitis or necrotizing polyangiitis. This chapter focuses on medium-sized Vessel Vasculitis and Small Vessel Vasculitis. The evaluation of patients with cutaneous palpablepurpura revealed Vasculitis involving predominantly Small Vessels. The pathologic findings in SVV of extensive acute inflammation with numerous neutrophils and conspicuous leukocytoclasia that resembled the Arthus reaction suggested a possible “hypersensitivity” pathogenesis. The chapter further discusses polyarteritis nodosa (PAN). The hallmark of PAN is necrotizing inflammation of medium-sized or Small arteries. Because different organs can be affected in different patients, the clinical manifestations of even relatively specific types of Vasculitis are extremely variable among patients. Therefore, the diagnosis of systemic Vasculitis, including autoimmune Vasculitis, is difficult, and requires skillful integration of clinical, pathologic, and laboratory data. Although difficult, precise diagnosis is essential for proper management, because the prognosis and appropriate treatment vary substantially among different categories of Vasculitis.
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The clinical course of ANCA Small-Vessel Vasculitis on chronic dialysis.
Kidney international, 2009Co-Authors: Sofia Lionaki, J. Charles Jennette, Ronald J. Falk, Susan L. Hogan, Caroline E. Jennette, Julie B. Hamra, Patrick H. NachmanAbstract:Antineutrophil cytoplasmic autoantibody (ANCA)-associated Small-Vessel Vasculitis frequently affects the kidney. Here we describe the rates of infection, disease relapse, and death in patients with ANCA Small-Vessel Vasculitis before and after end-stage renal disease (ESRD) in an inception cohort study and compare them to those of patients with preserved renal function. All patients had biopsy-proven ANCA Small-Vessel Vasculitis. Fisher's exact tests and Wilcoxon rank sum tests were used to compare the characteristics by ESRD status. ESRD follow-up included time on dialysis with transplants censored. Over a median follow-up time of 40 months, 136 of 523 patients reached ESRD. ESRD was associated with new-onset ANCA Small-Vessel Vasculitis in 51% of patients, progressive chronic kidney disease without active Vasculitis in 43%, and renal relapse in 6% of patients. Relapse rates of ANCA Small-Vessel Vasculitis, reported as episodes/person-year, were significantly lower on chronic dialysis (0.08 episodes) compared with the rate of the same patients before ESRD (0.20 episodes) or with patients with preserved renal function (0.16 episodes). Infections were almost twice as frequent among patients with ESRD on maintenance immunosuppressants and were an important cause of death. Given the lower risk of relapse and higher risk of infection and death, we suggest that immunosuppression be geared to patients with ESRD who present with active Vasculitis.
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Removing Antibody and Preserving Glomeruli in ANCA Small-Vessel Vasculitis
Journal of the American Society of Nephrology : JASN, 2007Co-Authors: Sofia Lionaki, Ronald J. FalkAbstract:If anti-neutrophil cytoplasmic autoantibodies (ANCA) participate in the pathogenesis of Small-Vessel Vasculitis, then it would stand to reason that removal of these antibodies should ameliorate disease. There is substantial evidence that ANCA are capable of activating leukocytes in vitro .[1][1],[2
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Controversies in Small Vessel Vasculitis--comparing the rheumatology and nephrology views.
Current opinion in rheumatology, 2007Co-Authors: Ronald J. Falk, Gary S. HoffmanAbstract:Over the past 20 years, remarkable progress has been made in understanding the pathogenesis and treatment of patients with Small Vessel Vasculitis. Nephrologists and rheumatologists play primary roles in the care of these patients. Whilst there are many areas of agreement between these specialists with respect to understanding and practical care of these patients, there are a number of important areas that differ in the practice patterns of nephrologic and rheumatologic practice. These areas include the nomenclature of these diseases, the role of antineutrophil cytoplasmic antibodies in the pathogenesis, the route and duration of initial cyclophosphamide administration, and, most importantly, the total duration of maintenance therapy following disease remission. Two experienced clinicians, a rheumatologist and a nephrologist, spar off in support of their opinions.
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Thoughts about the classification of Small Vessel Vasculitis.
Journal of nephrology, 2004Co-Authors: Ronald J. Falk, J. Charles JennetteAbstract:The classification of Small Vessel Vasculitis has markedly changed over the past 150 years. With the discovery of anti-neutrophil cytoplasmic autoantibodies (ANCA), renewed interest in the field has spawned investigations into the immunopathogenesis of Small Vessel Vasculitis. This review will describe the historical basis of the classification of Vasculitis, the current nosology based on the Chapel Hill Nomenclature for Vasculitis and then both pathogenic and clinical reasons that support the use of the term "ANCA Small Vessel Vasculitis" to describe the pauci-immune necrotizing Small Vessel vasculitides, including microscopic polyangiitis, Wegener's granulomatosis, the Churg-Strauss syndrome and pauci-immune necrotizing and crescentic glomerulonephritis.
J. Charles Jennette - One of the best experts on this subject based on the ideXlab platform.
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Necrotizing Arteritis and Small Vessel Vasculitis
The Autoimmune Diseases, 2014Co-Authors: J. Charles Jennette, Ronald J. FalkAbstract:Publisher Summary There are three major categories of systemic Vasculitis—namely, large Vessel Vasculitis or chronic granulomatous arteritis, medium-sized Vessel Vasculitis or necrotizing arteritis, and Small Vessel Vasculitis or necrotizing polyangiitis. This chapter focuses on medium-sized Vessel Vasculitis and Small Vessel Vasculitis. The evaluation of patients with cutaneous palpablepurpura revealed Vasculitis involving predominantly Small Vessels. The pathologic findings in SVV of extensive acute inflammation with numerous neutrophils and conspicuous leukocytoclasia that resembled the Arthus reaction suggested a possible “hypersensitivity” pathogenesis. The chapter further discusses polyarteritis nodosa (PAN). The hallmark of PAN is necrotizing inflammation of medium-sized or Small arteries. Because different organs can be affected in different patients, the clinical manifestations of even relatively specific types of Vasculitis are extremely variable among patients. Therefore, the diagnosis of systemic Vasculitis, including autoimmune Vasculitis, is difficult, and requires skillful integration of clinical, pathologic, and laboratory data. Although difficult, precise diagnosis is essential for proper management, because the prognosis and appropriate treatment vary substantially among different categories of Vasculitis.
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The clinical course of ANCA Small-Vessel Vasculitis on chronic dialysis.
Kidney international, 2009Co-Authors: Sofia Lionaki, J. Charles Jennette, Ronald J. Falk, Susan L. Hogan, Caroline E. Jennette, Julie B. Hamra, Patrick H. NachmanAbstract:Antineutrophil cytoplasmic autoantibody (ANCA)-associated Small-Vessel Vasculitis frequently affects the kidney. Here we describe the rates of infection, disease relapse, and death in patients with ANCA Small-Vessel Vasculitis before and after end-stage renal disease (ESRD) in an inception cohort study and compare them to those of patients with preserved renal function. All patients had biopsy-proven ANCA Small-Vessel Vasculitis. Fisher's exact tests and Wilcoxon rank sum tests were used to compare the characteristics by ESRD status. ESRD follow-up included time on dialysis with transplants censored. Over a median follow-up time of 40 months, 136 of 523 patients reached ESRD. ESRD was associated with new-onset ANCA Small-Vessel Vasculitis in 51% of patients, progressive chronic kidney disease without active Vasculitis in 43%, and renal relapse in 6% of patients. Relapse rates of ANCA Small-Vessel Vasculitis, reported as episodes/person-year, were significantly lower on chronic dialysis (0.08 episodes) compared with the rate of the same patients before ESRD (0.20 episodes) or with patients with preserved renal function (0.16 episodes). Infections were almost twice as frequent among patients with ESRD on maintenance immunosuppressants and were an important cause of death. Given the lower risk of relapse and higher risk of infection and death, we suggest that immunosuppression be geared to patients with ESRD who present with active Vasculitis.
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CHAPTER 65 – Necrotizing Arteritis and Small Vessel Vasculitis
The Autoimmune Diseases, 2006Co-Authors: J. Charles JennetteAbstract:Publisher Summary There are three major categories of systemic Vasculitis—namely, large Vessel Vasculitis or chronic granulomatous arteritis, medium-sized Vessel Vasculitis or necrotizing arteritis, and Small Vessel Vasculitis or necrotizing polyangiitis. This chapter focuses on medium-sized Vessel Vasculitis and Small Vessel Vasculitis. The evaluation of patients with cutaneous palpablepurpura revealed Vasculitis involving predominantly Small Vessels. The pathologic findings in SVV of extensive acute inflammation with numerous neutrophils and conspicuous leukocytoclasia that resembled the Arthus reaction suggested a possible “hypersensitivity” pathogenesis. The chapter further discusses polyarteritis nodosa (PAN). The hallmark of PAN is necrotizing inflammation of medium-sized or Small arteries. Because different organs can be affected in different patients, the clinical manifestations of even relatively specific types of Vasculitis are extremely variable among patients. Therefore, the diagnosis of systemic Vasculitis, including autoimmune Vasculitis, is difficult, and requires skillful integration of clinical, pathologic, and laboratory data. Although difficult, precise diagnosis is essential for proper management, because the prognosis and appropriate treatment vary substantially among different categories of Vasculitis.
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Thoughts about the classification of Small Vessel Vasculitis.
Journal of nephrology, 2004Co-Authors: Ronald J. Falk, J. Charles JennetteAbstract:The classification of Small Vessel Vasculitis has markedly changed over the past 150 years. With the discovery of anti-neutrophil cytoplasmic autoantibodies (ANCA), renewed interest in the field has spawned investigations into the immunopathogenesis of Small Vessel Vasculitis. This review will describe the historical basis of the classification of Vasculitis, the current nosology based on the Chapel Hill Nomenclature for Vasculitis and then both pathogenic and clinical reasons that support the use of the term "ANCA Small Vessel Vasculitis" to describe the pauci-immune necrotizing Small Vessel vasculitides, including microscopic polyangiitis, Wegener's granulomatosis, the Churg-Strauss syndrome and pauci-immune necrotizing and crescentic glomerulonephritis.
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Recurrent ANCA-associated Small Vessel Vasculitis after transplantation: A pooled analysis.
Kidney international, 1999Co-Authors: Patrick H. Nachman, J. Charles Jennette, Susan L. Hogan, Mårten Segelmark, Kerstin Westman, Karen K. Satterly, Ronald J. FalkAbstract:Recurrent ANCA-associated Small Vessel Vasculitis after transplantation: A pooled analysis. Background Recurrent antineutrophil cytoplasmic antibody (ANCA)-associated Small Vessel Vasculitis (ANCA-SVV) after renal transplantation has been described in case series. However, general information regarding the frequency, character, and predictors of recurrent disease after transplantation is currently lacking. We considered the rate of relapse, whether a positive ANCA at the time of transplantation predicted relapse, and whether cyclosporine A prevented recurrent disease. Methods We performed a pooled analysis of published data, added to the experience at the Universities of North Carolina (14 patients) and Lund, Sweden (11 patients). To avoid reporting bias, only case series were included for analysis. Subgroup analysis was performed by disease category (Wegener's granulomatosis, microscopic polyangiitis, or necrotizing crescentic glomerulonephritis) and ANCA staining pattern. Results ANCA-SVV recurred in 17.3% of all patients ( N = 127), in 20% of cyclosporine A-treated patients ( N = 85), and in 25.6% of patients with circulating ANCA at the time of transplantation ( N = 39). There was no statistically significant difference in the relapse rate between patients treated and those not treated with cyclosporine A ( P = 0.45), between those with and without circulating ANCA at the time of transplant ( P = 0.75), or between patients with Wegener's granulomatosis and those with microscopic polyangiitis or necrotizing crescentic glomerulonephritis alone ( P = 0.62). Conclusion There is a substantial relapse rate in the ANCA-SVV population. Therapy with cyclosporine A does not protect against recurrent ANCA-SVV, and the presence of a positive ANCA at the time of transplantation does not preclude transplantation. These conclusions must be substantiated with a prospective study of renal transplantation in patients with ANCA-SVV so as to optimize their management.
Yoshihisa Nojima - One of the best experts on this subject based on the ideXlab platform.
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A case of ANCA-negative renal Small-Vessel Vasculitis with tubulointerstitial infiltration of IgG4-positive plasma cells
Modern rheumatology, 2014Co-Authors: Toru Sakairi, Satoshi Okabe, Keiju Hiromura, Shinsuke Motegi, Noriyuki Sakurai, Hidekazu Ikeuchi, Yoriaki Kaneko, Akito Maeshima, Junko Hirato, Yoshihisa NojimaAbstract:A 62-year-old male patient presented with progressive renal dysfunction for 2 months. He had elevated serum C-reactive protein and IgG4 levels with absence of anti-neutrophil cytoplasmic antibodies. A renal biopsy showed severe tubulointerstitial nephritis (TIN) with extensive infiltration of IgG4-positive plasma cells, suggesting a diagnosis of IgG4-related kidney disease (IgG4-RKD). However, the identification of a few crescentic glomeruli and necrotizing Vasculitis of an interlobular artery lead to a diagnosis of renal Small-Vessel Vasculitis. This case indicates that a careful examination is required to distinguish between IgG4-RKD and TIN caused by renal Small-Vessel Vasculitis.
Carol A. Langford - One of the best experts on this subject based on the ideXlab platform.
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Small-Vessel Vasculitis: therapeutic management.
Current rheumatology reports, 2007Co-Authors: Carol A. LangfordAbstract:Small-Vessel Vasculitis is defined by the presence of blood Vessel inflammation involving the arterioles, venules, or capillaries. This can be seen in a broad spectrum of settings, but it is most commonly associated with Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Although prednisone combined with cyclophosphamide induces remission and prolongs survival in these diseases, this regimen is toxic and does not prevent relapse. Current therapeutic approaches seek to minimize cyclophosphamide exposure through the use of staged induction-maintenance regimens or cyclophosphamide alternatives for induction of nonsevere disease. Emerging trials with biologic agents are exploring new treatment options.
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Advances in the treatment of Small Vessel Vasculitis
Rheumatic diseases clinics of North America, 2006Co-Authors: Eamonn S. Molloy, Carol A. LangfordAbstract:The primary systemic Small Vessel vasculitides include Wegener’s granulomatosis (WG), Churg-Strauss syndrome (CSS), and microscopic polyangiitis (MPA). These conditions also are referred to by some investigators as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This term emphasizes the similarities between these conditions, including the predominant size of Vessel involved, renal histology, and association with ANCA. Because clinicopathologic and therapeutic differences do exist between these diseases, they are discussed separately herein. The pathogenesis of the Small Vessel vasculitides is not understood completely. Neutrophils, augmented by endothelial cell activation from proinflammatory cytokines, such as tumor necrosis factor a (TNF-a), play a role in vascular injury. Cell-mediated immune responses also are involved as indicated by the finding of T cells and monocytes within vasculitic lesions and the presence of granulomas in WG and CSS. ANCA may be involved in the pathogenesis of AAV, in particular in the activation of cytokine-primed neutrophils and monocytes. Detection of ANCA plays a key role in the assessment of patients who have Small Vessel Vasculitis; proteinase 3 (PR3)-specific ANCA characteristically is found in WG and myeloperoxidase (MPO)-specific ANCA usually is present in MPA and CSS.
Nadia Anguel - One of the best experts on this subject based on the ideXlab platform.
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Outcomes of patients admitted to intensive care units for acute manifestation of Small-Vessel Vasculitis: a multicenter, retrospective study
Critical care (London England), 2016Co-Authors: Antoine Kimmoun, E. Baux, Vincent Das, Nicolas Terzi, Patrice Talec, Pierre Asfar, Stephan Ehrmann, Guillaume Geri, Steven Grangé, Nadia AnguelAbstract:Background The outcomes of patients admitted to the intensive care unit (ICU) for acute manifestation of Small-Vessel Vasculitis are poorly reported. The aim of the present study was to determine the mortality rate and prognostic factors of patients admitted to the ICU for acute Small-Vessel Vasculitis.
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Outcomes of patients admitted to intensive care units for acute manifestation of Small-Vessel Vasculitis: a multicenter, retrospective study
Critical Care, 2015Co-Authors: Antoine Kimmoun, E. Baux, Vincent Das, Nicolas Terzi, Patrice Talec, Pierre Asfar, Stephan Ehrmann, Guillaume Geri, Steven Grangé, Nadia AnguelAbstract:BACKGROUND: The outcomes of patients admitted to the intensive care unit (ICU) for acute manifestation of Small-Vessel Vasculitis are poorly reported. The aim of the present study was to determine the mortality rate and prognostic factors of patients admitted to the ICU for acute Small-Vessel Vasculitis. METHODS: This retrospective, multicenter study was conducted from January 2001 to December 2014 in 20 ICUs in France. Patients were identified from computerized registers of each hospital using the International Classification of Diseases, Ninth Revision (ICD-9). Inclusion criteria were (1) known or highly suspected granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis (respectively, ICD-9 codes M31.3, M30.1, and M31.7), or anti-glomerular basement membrane antibody disease (ICD-9 codes N08.5X-005 or M31.0+); (2) admission to the ICU for the management of an acute manifestation of Vasculitis; and (3) administration of a cyclophosphamide pulse in the ICU or within 48 h before admission to the ICU. The primary endpoint was assessment of mortality rate 90 days after admission to the ICU. RESULTS: Eighty-two patients at 20 centers were included, 94% of whom had a recent (