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"Antineutrophil cytoplasmic autoantibodies"

Domenico Santoro, Carmela Vadalà, Rossella Siligato, Michele Buemi, Salvatore Benvenga
Autoimmune thyroiditis (AIT) is generally associated with hypothyroidism. It affects ~2% of the female population and 0.2% of the male population. The evidence of thyroid function- and thyroid autoantibody-unrelated microproteinuria in almost half of patients with AIT and sometimes heavy proteinuria as in the nephrotic syndrome point to a link of AIT with renal disease. The most common renal diseases observed in AIT are membranous nephropathy, membranoproliferative glomerulonephritis, minimal change disease, IgA nephropathy, focal segmental glomerulosclerosis, antineutrophil cytoplasmic autoantibody (ANCA) vasculitis, and amyloidosis...
2017: Frontiers in Endocrinology
Aleksandar Jankovic, Vesna Maslarevic-Radovic, Petar Djuric, Jelena Tosic-Dragovic, Ana Bulatovic, Nikola Simovic, Milos Mitrovic, Verica Stankovic-Popovic, Vesna Dopudja-Pantic, Snezana Arandjelovic, Nada Dimkovic
INTRODUCTION: Microscopic polyangiitis (MPA) is one of the causes of the pulmonary-renal syndrome associated with elevated non-specific markers of inflammation and antineutrophil cytoplasmic autoantibody (ANCA) positivity in 50-75%. De novo occurrence of the disease in patients on chronic hemodialysis (HD) has not been described. CASE PRESENTATION: We presented patient who developed MPO-ANCA-associated MPA with lung and musculoskeletal involvement after 4 years on regular HD due to bilateral nephrectomy...
2017: Frontiers in Immunology
Kimihiko Goto, Kentaro Nakai, Hideki Fujii, Shinichi Nishi
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a life-threatening disease characterized by rapidly progressive glomerulonephritis (RPGN) and diffuse alveolar hemorrhage (DAH). Glucocorticoids and immunosuppressants are commonly used to treat this disease but may induce irreversible side effects, particularly in elderly patients. We herein report the case of a 76-year-old woman with RPGN. After methylprednisolone pulse therapy, DAH occurred, and she required ventilatory support. After plasma exchange, her serum creatinine level improved, and she was discharged with home oxygen therapy...
2017: Internal Medicine
David Bergeron, Annie Dionne, Raphaël Marchand, Robert Laforce
No abstract text is available yet for this article.
March 2017: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Katarzyna Życińska, Anna Borowiec
No abstract text is available yet for this article.
2016: Kardiologia Polska
Damien Noone, Diane Hebert, Christoph Licht
The antineutrophil cytoplasm autoantibody (ANCA)-associated vasculitides (AAV), although rare in childhood, can have devastating effects on affected organs, especially the kidney. In this review we present an update on the pathogenesis and treatment of ANCA vasculitis, with a particular emphasis on the role of the alternative pathway of complement. The rationale and evidence for the current treatment strategies are summarized. Targeting the activation of neutrophils by the anaphylatoxin C5a may serve as an additional therapeutic strategy, however the results of clinical studies are awaited...
September 5, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Hong Xiao, Peiqi Hu, Ronald J Falk, J Charles Jennette
BACKGROUND: Antineutrophil cytoplasmic autoantibodies (ANCA) are associated with a spectrum of necrotizing vasculitis including granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited necrotizing and crescentic glomerulonephritis. Clinical observations and in vitro and in vivo experimental evidence strongly indicate that ANCA are pathogenic. SUMMARY: The etiology and pathogenesis of ANCA-associated vasculitis (AAV) are multifactorial, with contributions from genetic factors, environmental exposures, infections, characteristics of the innate and adaptive immune system, and the intensity and duration of the injury...
March 2016: Kidney Diseases
Mohammad A Ursani, Joan Appleyard, Onome Whiteru
BACKGROUND Pyogenic arthritis, pyoderma gangrenosum (PG), acne, and suppurative hidradenitis (PA-PASH) syndrome has been linked to an auto-inflammatory pathway. We report a case that is an atypical presentation of a rare syndrome, which supports literature suggesting that different phenotypes of PG-related syndromes may be a variation of the same pathogenic spectrum. Interestingly, our patient displayed a positive proteinase-3 antibody (PR-3). The clinical relevance of this is unclear. In recent literature, antineutrophil cytoplasmic autoantibodies (ANCA) positivity has been reported in various inflammatory conditions other than ANCA-associated vasculitis (AAV)...
August 17, 2016: American Journal of Case Reports
Yinghua Chen, Yuemei Ding, Zhengzhao Liu, Haitao Zhang, Zhihong Liu, Weixin Hu
BACKGROUND: The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied. METHODS: Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed. RESULTS: The most common initial symptom of EGPA was asthma (57...
July 26, 2016: BMC Nephrology
Habib Khan, Sanjay Chaubey, Rynda Nitiahpapand, Jatin Desai
Cardiac involvement is rare in antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis but can involve aortic and mitral valves. We present an unusual case of a 65-year-old woman who presented 16 years after an aortic valve replacement with severe mitral regurgitation with ACNA-associated vasculitis. The extensive nature of the pathologic condition, which extended to the tricuspid valve, prevented the replacement of the mitral valve during a surgical procedure. This is a rare case in which florid valvulopathy was observed in association with vasculitis...
August 2016: Annals of Thoracic Surgery
Gholamreza Pouladfar, Zahra Jafarpour, Amir Hossein Babaei, Bahman Pourabbas, Bita Geramizadeh, Anahita Sanaei Dashti
Visceral leishmaniasis (VL), a life-threatening parasitic infection, is endemic in the Mediterranean region. Diagnosis of VL is based on epidemiologic, clinical, and laboratory findings. However, sometimes, clinical features and laboratory findings overlap with those of autoimmune diseases. In some cases, autoantibodies are detected in patients with VL and this could be a potential diagnostic pitfall. In this study, we have reported on a three-year-old girl from a VL-endemic area in Iran, who presented with prolonged fever and splenomegaly...
2016: Case Reports in Infectious Diseases
Yinghua Chen, Liu Yang, Kang Li, Zhengzhao Liu, Dehua Gong, Haitao Zhang, Zhihong Liu, Weixin Hu
Our aim was to investigate the clinical efficacy of double filtration plasmapheresis (DFPP) in the treatment of antineutrophil cytoplasmic autoantibody-(ANCA) associated vasculitis (AAV) with severe renal involvement. Fifteen AAV patients who had severe renal failure (median SCr 5.6(IQR 5.2-9.0) mg/dL) and needed initial renal replacement therapy (RRT) were treated with DFPP and immunosuppressive therapy. Two plasma volumes were processed during each DFPP session. The changes of serum ANCA and renal function were investigated...
April 2016: Therapeutic Apheresis and Dialysis
Falk Hiepe, Andreas Radbruch
Autoantibodies are secreted by plasma cells and have an essential role in driving the renal manifestations of autoimmune diseases such as systemic lupus erythematosus and antineutrophil cytoplasmic autoantibody-associated vasculitis. Effective depletion of autoreactive plasma cells might be the key to curative treatment of these diseases. Two major plasma-cell compartments exist: short-lived plasmablasts or plasma cells, which result from differentiation of activated B cells, and long-lived plasma cells, which result from secondary immune responses...
April 2016: Nature Reviews. Nephrology
A Kerstein, A Müller, D Kabelitz, P Lamprecht
Patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) have an expansion of effector memory T‑cells in peripheral blood. The enlarged effector memory cell population contains distinct cell subsets, including T‑helper type 1 (Th1) CD4+ T‑cells lacking co-stimulatory CD28 expression and Th17 cells in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and Th2 type and Th17 cells in eosinophilic granulomatosis with polyangiitis (EGPA). The cytokine response of autoreactive proteinase 3 (PR3)-specific effector memory T‑cells is skewed towards an increase of Th2 type, Th17 and Th22 cell fractions in GPA...
March 2016: Zeitschrift Für Rheumatologie
A Kerstein, A Müller, D Kabelitz, P Lamprecht
Patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV) have an expansion of effector memory T‑cells in peripheral blood. The enlarged effector memory cell population contains distinct cell subsets, including T‑helper type 1 (Th1) CD4(+) T‑cells lacking co-stimulatory CD28 expression and Th17 cells in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) and Th2 type and Th17 cells in eosinophilic granulomatosis with polyangiitis (EGPA). The cytokine response of autoreactive proteinase 3 (PR3)-specific effector memory T‑cells is skewed towards an increase of Th2 type, Th17, and Th22 cell fractions in GPA...
February 25, 2016: Zeitschrift Für Rheumatologie
Beatrice Feragalli, Cesare Mantini, Marco Sperandeo, Michele Galluzzo, Giovanni Belcaro, Armando Tartaro, Antonio R Cotroneo
The respiratory system may be involved in all systemic vasculitides, although with a variable frequency. The aim of our review is to describe radiographic and high-resolution CT (HRCT) findings of pulmonary vasculitides and to correlate radiological findings with pathological results. Lung disease is a common feature of antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides, including granulomatosis with polyangiitis (Wegener's), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) and microscopic polyangiitis...
2016: British Journal of Radiology
Xiang-Yang Li, Ying-Shan Liang, Pearl Pai
OBJECTIVES: The kidneys are frequently involved in antineutrophil cytoplasmic autoantibody (ANCA) associated small-vessel vasculitis (AASVV). The pathological hallmark of ANCA-associated glomerulonephritis (AAGN) is a pauci-immune necrotising crescentic glomerulonephritis. The histopathology of AAGN may change during the course of the disease as a consequence of immunosuppressive therapy. Herein, we report the pathological evolution of a case of AAGN. METHODS: We report a female presented with renal-limited AASVV, hypocomplementemia and nephrotic syndrome...
May 2016: Clinical and Experimental Rheumatology
Alessandra Ferrarini, Cecilia Benetti, Pietro Camozzi, Alessandro Ostini, Giacomo D Simonetti, Gregorio P Milani, Mario G Bianchetti, Sebastiano A G Lava
UNLABELLED: Acute hemorrhagic edema of young children is a rare leukocytoclastic vasculitis that has been reported exclusively in small retrospective cases series, case reports, or quizzes. Considering that retrospective experience deserves confirmation in at least one observational prospective study, we present our experience with 16 children (12 boys and 4 girls, 5-28 months of age) affected by acute hemorrhagic edema. The patients were in good general conditions and with a low-grade or even absent fever...
April 2016: European Journal of Pediatrics
Lindsay Lally, Robert Spiera
PURPOSE OF REVIEW: The present review discusses the evidence supporting the use of B-cell-targeted therapy in the treatment of various forms of systemic vasculitis with a focus on the use of rituximab (RTX), in the antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV). RECENT FINDINGS: Long-term follow-up of the two studies establishing the efficacy of a RTX-based induction regimen for severe AAV have demonstrated noninferiority of a single course of RTX compared with conventional therapy for remission maintenance...
January 2016: Current Opinion in Rheumatology
Cees G M Kallenberg
PURPOSE OF REVIEW: Antineutrophil cytoplasmic autoantibodies (ANCAs) are considered important diagnostic tests in the work-up of patients suspected of vasculitis. Here we discuss new developments in the methodology of testing, the pitfalls in using these tests as diagnostic tools, and the value of serial ANCA testing for the follow-up of patients with ANCA-associated vasculitis including treatment decisions. RECENT FINDINGS: Both the indirect immunofluorescence (IIF) test and antigen-specific assays should be performed...
January 2016: Current Opinion in Rheumatology
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