109 results match your criteria pauci-immune small-vessel

Proteinuria Indicates Decreased Normal Glomeruli in ANCA-Associated Glomerulonephritis Independent of Systemic Disease Activity.

J Clin Med 2021 Apr 6;10(7). Epub 2021 Apr 6.

Department of Nephrology and Rheumatology, University Medical Center Göttingen, 37075 Göttingen, Germany.

Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Read More

View Article and Full-Text PDF

Systemic lupus erythematosus and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome in a 77-year-old man: A case report.

World J Clin Cases 2021 Jan;9(3):707-713

Department of Nephrology, Shenzhen Longhua District Central Hospital, Shenzhen 518110, Guangdong Province, China.

Background: Systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies. Their overlap constitutes a rare rheumatologic disease. To date, only dozens of such cases with biopsy-proven glomerulonephritis have been reported worldwide typically in women of childbearing age. Read More

View Article and Full-Text PDF
January 2021

Thrombotic Microangiopathy and Venous Thrombosis in a Patient With Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis.

Cureus 2020 Nov 23;12(11):e11665. Epub 2020 Nov 23.

Department of Pathology, Methodist Dallas Medical Center, Dallas, USA.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic pauci-immune small vessel vasculitis. Its various presentations make AAV diagnosis challenging. Here, we present a case of AAV with thrombotic microangiopathy (TMA) and deep venous thrombosis (DVT). Read More

View Article and Full-Text PDF
November 2020

Successful Induction Treatment With Rituximab of Isolated Pauci-Immune Pulmonary Capillaritis Presenting as Diffuse Alveolar Hemorrhage in a Pediatric Patient.

Chest 2020 11;158(5):e225-e227

Department of Pediatric Pulmonology and Sleep Medicine, Children's Hospital Los Angeles, University of Southern California School of Medicine, Los Angeles, CA.

Diffuse alveolar hemorrhage often presents as dyspnea, cough, or hemoptysis, and it is mediated by both immune and nonimmune processes. Isolated pauci-immune capillaritis (IPPC) is a rare diagnosis in which capillaritis, small-vessel vasculitis of the lung, is found on biopsy in the absence of an underlying systemic disorder. Traditionally, IPPC has been treated similarly to anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis with cyclophosphamide and glucocorticoids. Read More

View Article and Full-Text PDF
November 2020

Simultaneous development of IgA vasculitis and eosinophilic granulomatosis with polyangiitis.

Mod Rheumatol Case Rep 2020 01 23;4(1):63-69. Epub 2019 Oct 23.

Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

Immunoglobulin A (IgA) vasculitis (IgAV) is a small vessel vasculitis presenting cutaneous purpura, arthralgias and/or arthritis, acute enteritis and glomerulonephritis caused by deposition of the IgA-mediated immune complex. Eosinophilic granulomatosis with polyangiitis (EGPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by eosinophil-rich and granulomatous inflammation in small to medium-sized vessels. Both IgAV and EGPA are classified as autoimmune systemic vasculitis, but the pathogenesis of immune complex-mediated IgAV and that of pauci-immune EGPA are different. Read More

View Article and Full-Text PDF
January 2020

Microscopic polyangiitis.

Vnitr Lek 2020 ;66(4):249-252

Microscopic polyangiitis is a rare, systemic, necrotizing, pauci-immune, ANCA associated small vessel vasculitis, with no evidence of granulomatous inflammation. Diagnosing microscopic polyangiitis is often difficult because of it´s presentation by a number of non-specific symptoms. We treated a 35-year old patient, who was admitted for migrating arthritis and fever with papulous rash. Read More

View Article and Full-Text PDF
November 2020

Environmental risk factors associated with ANCA associated vasculitis: A systematic mapping review.

Autoimmun Rev 2020 Nov 15;19(11):102660. Epub 2020 Sep 15.

Trinity Health Kidney Centre, Trinity Translational Medicine Institute, Trinity College Dublin, Ireland; ADAPT Centre, Trinity College Dublin, Ireland. Electronic address:

Background: Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare multi-system autoimmune disease, characterised by a pauci-immune necrotising small-vessel vasculitis, with a relapsing and remitting course. Like many autoimmune diseases, the exact aetiology of AAV, and the factors that influence relapse are unknown. Evidence suggests a complex interaction of polygenic genetic susceptibility, epigenetic influences and environmental triggers. Read More

View Article and Full-Text PDF
November 2020

Renal involvement in eosinophilic granulomatosis with polyangiitis (EGPA): a multicentric retrospective study of 63 biopsy-proven cases.

Rheumatology (Oxford) 2021 01;60(1):359-365

Department of Nephrology, Hôpital Européen Georges Pompidou, AP-HP, Paris, France.

Objective: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small-vessel vasculitis characterized by asthma, hypereosinophilia and ANCA positivity in 40% of patients. Renal involvement is rare and poorly described, leading to this renal biopsy-proven based study in a large EGPA cohort.

Methods: We conducted a retrospective multicentre study including patients fulfilling the 1990 ACR criteria and/or the 2012 revised Chapel Hill Consensus Conference criteria for EGPA and/or the modified criteria of the MIRRA trial, with biopsy-proven nephropathy. Read More

View Article and Full-Text PDF
January 2021

Diffuse alveolar hemorrhage with histopathologic manifestations of pulmonary capillaritis: Three case reports.

World J Clin Cases 2020 Jun;8(12):2662-2666

Department of Pediatrics, the First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi Zhuang Autonomous Region, China.

Background: Diffuse alveolar hemorrhage (DAH) is a multicause pulmonary capillary hemorrhage or pulmonary vascular small vessel injury (mainly capillaries, including arteries and veins), causing pulmonary microcirculation blood to accumulate in the alveolar space. DAH is classified by the histological absence or presence of pulmonary capillaritis (PC) and is rarely reported in the literature.

Case Summary: This is a report of three girls aged 6-11 years with DAH and PC. Read More

View Article and Full-Text PDF

Narrative Review of Hypercoagulability in Small-Vessel Vasculitis.

Kidney Int Rep 2020 May 13;5(5):586-599. Epub 2020 Jan 13.

Department of Internal Medicine, Section on Nephrology, Wake Forest School of Medicine, Winston Salem, North Carolina, USA.

Pauci-immune necrotizing and crescentic glomerulonephritis (GN) is the most common etiology of rapidly progressive GN. Clinical presentation in those afflicted is usually related to rapid loss of kidney function. We report the case of a 70-year-old woman who came to medical attention for signs and symptoms related to lower-extremity deep vein thrombosis (DVT). Read More

View Article and Full-Text PDF

Serological testing in small vessel vasculitis.

Rheumatology (Oxford) 2020 05;59(Suppl 3):iii51-iii54

Department of Clinical Sciences, Nephrology, Skane University Hospital, Lund University, Lund, Sweden.

Serological analysis has a central role in the diagnostic work-up of patients with suspected small vessel vasculitis, both for establishing a specific diagnosis and for the monitoring of response to therapy. Autoantibodies can be detected in all forms of primary small vessel vasculitis as well as in the most common forms of secondary vasculitis. For primary vasculitis the most important serological test is for ANCA. Read More

View Article and Full-Text PDF

Immune deposits in skin vessels of patients with acute hemorrhagic edema of young children: A systematic literature review.

Pediatr Dermatol 2020 Jan 22;37(1):120-123. Epub 2019 Nov 22.

Pediatric Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland.

Background: Acute hemorrhagic edema of young children is a benign skin-limited vasculitis mainly affecting children 2 to 24 months of age, which is often considered the infantile variant of immunoglobulin A vasculitis (Henoch-Schönlein purpura). In most cases, the diagnosis is made on a clinical basis without a skin biopsy.

Methods: A systematic review of the literature was performed to examine the reported prevalence of vascular immune deposits in skin biopsies of patients with acute hemorrhagic edema of young children. Read More

View Article and Full-Text PDF
January 2020

Complement alternative pathway in ANCA-associated vasculitis: Two decades from bench to bedside.

Autoimmun Rev 2020 Jan 15;19(1):102424. Epub 2019 Nov 15.

Service de Néphrologie-Dialyse-Transplantation, Université d'Angers, CHU Angers, Angers, France; CRCINA, INSERM, Université de Nantes, Université d'Angers, Angers, France. Electronic address:

Anti-neutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitides (AAVs) are small vessel vasculitides involving predominantly ear-nose-throat, kidneys, lungs and nerves. AAVs are life-threatening diseases, especially in their most severe forms such as necrotizing crescentic glomerulonephritis (GN) and/or intra-alveolar hemorrhage. Unlike immune complex GN or anti-glomerular basement membrane GN, AAVs are classified as pauci-immune GN. Read More

View Article and Full-Text PDF
January 2020

Unilateral eyelid edema and mucosal involvement as the first presentations of Wegener granulomatosis.

Caspian J Intern Med 2019 ;10(3):343-346

Nassau University Medical Center, Department of Internal Medicine, 2201 Hempstead Turnpike, East Meadow, NY, 11554, 516-978-0158, USA.

Background: Wegener granulomatosis or granulomatosis with polyangiitis is a pauci-immune small vessel vasculitis which is usually associated with anti-neutrophil cytoplasmic antibodies (ANCA) mainly in old men. This small vessel vasculitis is usually characterized by necrotizing granulomatous inflammation with multiorgan involvement. Kidneys could be involved as the main and life-threatening condition in Wegener granulomatosis. Read More

View Article and Full-Text PDF
January 2019

CMV gastric ulcer in a patient with pauci immune crescentic glomerulonephritis on rituximab - a rare combination.

Postgrad Med 2019 Nov 17;131(8):619-622. Epub 2019 Sep 17.

Gastroenterology, Mercy St. Vincent Medical Center, Toledo, OH, USA.

Cytomegalovirus (CMV) infections are asymptomatic in immunocompetent patients but in immunocompromised patients, CMV infections have varying manifestations depending on their location. Patient who are organ transplant recipients, taking immunosuppressive therapy for a long time are at increased risk of CMV infections. CMV-induced gastric ulcer is very rare but many cases have been reported in the literature. Read More

View Article and Full-Text PDF
November 2019

Central Nervous System Involvement in ANCA-Associated Vasculitis: What Neurologists Need to Know.

Front Neurol 2018 10;9:1166. Epub 2019 Jan 10.

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

To provide a comprehensive review of the central nervous system (CNS) involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including the pathogenesis, clinical manifestations, ancillary investigations, differential diagnosis, and treatment. Particular emphasis is placed on the clinical spectrum and diagnostic testing of AAV. AAV is a pauci-immune small-vessel vasculitis characterized by neutrophil-mediated vasculitis and granulomatousis. Read More

View Article and Full-Text PDF
January 2019

Genetics and pathogenesis of small-vessel vasculitis.

Alan D Salama

Best Pract Res Clin Rheumatol 2018 02 17;32(1):21-30. Epub 2018 Nov 17.

UCL Department of Renal Medicine, Centre for Experimental Nephrology, Royal Free Hospital, London, NW3 2PF, United Kingdom. Electronic address:

Small-vessel vasculitides are uncommon autoimmune diseases characterised by inflammation and necrosis of arterioles, capillaries and venules, frequently described as various (previously eponymous) clinical syndromes. Some are associated with vessel wall immune complex deposition, whereas others are pauci-immune but paradoxically often associated with circulating anti-neutrophil cytoplasmic antibodies (ANCA). Most is known about the pathogenesis of the pauci-immune ANCA-associated syndromes, which are gradually becoming better understood with regard to their genetic predisposition and the critical pathways mediating disease initiation, as well as their particular phenotypic features. Read More

View Article and Full-Text PDF
February 2018

Alveolar Hemorrhage in Vasculitis (Primary and Secondary).

Semin Respir Crit Care Med 2018 08 7;39(4):482-493. Epub 2018 Nov 7.

Department of Respiratory Medicine, Hospices Civils de Lyon, National Reference Center for Rare Pulmonary Diseases, Lyon, France.

Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Read More

View Article and Full-Text PDF

Primary Vasculitis in Childhood: GPA and MPA in Childhood.

Front Pediatr 2018 16;6:226. Epub 2018 Aug 16.

The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Childhood onset anti-neutrophilic cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a rare group of primary systemic vasculitides affecting medium and small blood vessels. AAV includes granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and renal limited ANCA vasculitis. These disorders are associated with severe clinical manifestations, frequent relapses and a high cumulative morbidity, and often present with multisystem involvement. Read More

View Article and Full-Text PDF

ANCA associated vasculitis: experience of a tertiary care referral center.

J Bras Nefrol 2019 Jan-Mar;41(1):55-64. Epub 2018 Aug 9.

Jersey Shore University Medical Center, Seton Hall-Hackensack-Meridian School of Medicine, Neptune, NJ, USA.

Background And Objectives: Anti-neutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis is a small vessel vasculitis with insufficient epidemiologic estimates in the United States. We aimed to determine demographic and clinical features of ANCA associated vasculitis patients presenting to a large tertiary care referral center in Upstate New York. Design, setting, participants, and measurements: A retrospective analysis of cases with pauci-immune GN on renal biopsy and clinical diagnosis of ANCA vasculitis presenting over 11 years was conducted. Read More

View Article and Full-Text PDF
December 2019

ANCA-Positive pauci-immune crescentic glomerulonephritis in a patient with systemic lupus erythematosus.

J Bras Nefrol 2017 Oct-Dec;39(4):454-457

Laboratorios Integrados S.A, Lima, Perú.

The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury symptoms with indication for renal replacement therapy in the context of clinical SLE diagnosis. A kidney biopsy was conducted and it was found that most glomeruli showed some segmental sclerosis with synechia to the Bowman's capsule. Read More

View Article and Full-Text PDF
September 2018

The Prevalence and Management of Pauci-Immune Glomerulonephritis and Vasculitis in Western Countries.

Kidney Dis (Basel) 2016 Mar 5;1(4):224-34. Epub 2015 Dec 5.

Department of Nephrology, Laiko Hospital, Athens, Greece; Transplantation Unit, Laiko Hospital, Athens, Greece.

Background: Pauci-immune glomerulonephritis is the most common cause of aggressive glomerulonephritis and occurs as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis. It is characterized by paucity of staining for immunoglobulins, by immunofluorescence along with fibrinoid necrosis and crescent formation by light microscopy, while the vast majority of patients have anti-neutrophil cytoplasmic antibodies (ANCA) in their circulation, which also participate in the pathogenesis of the disease.

Summary: Pauci-immune glomerulonephritis often manifests with rapidly deteriorating kidney function, which may be accompanied by distinctive clinical features of systemic necrotizing small-vessel vasculitis of one the following clinical phenotypes: microscopic polyangiitis, granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis. Read More

View Article and Full-Text PDF

The clinical presentation and therapy of diseases related to anti-neutrophil cytoplasmic antibodies (ANCA).

Autoimmun Rev 2016 Oct 29;15(10):978-82. Epub 2016 Jul 29.

Department of Medical and Health Sciences, Linköping University, Linköping, Sweden; Department of Nephrology, Linköping University, Linköping, Sweden. Electronic address:

Anti-neutrophil cytoplasmic antibodies (ANCA) are a family of autoantibodies that react with proteins predominantly expressed in cytoplasmic granules of polymorphonuclear neutrophil granulocytes (PMNs). ANCA was initially detected using indirect immunofluorescence, allowing for different patterns such as p-ANCA (perinuclear) and c-ANCA (cytoplasmic) to be distinguished. Today it is common to detect the antibodies by immunochemical assays such as ELISA using purified proteins as antigens. Read More

View Article and Full-Text PDF
October 2016

Neutrophil Extracellular Traps in ANCA-Associated Vasculitis.

Front Immunol 2016 30;7:256. Epub 2016 Jun 30.

Department of Medical and Health Sciences, Linköping University, Linköping, Sweden; Department of Nephrology, Linköping University, Linköping, Sweden.

A group of pauci-immune vasculitides, characterized by neutrophil-rich necrotizing inflammation of small vessels and the presence of antineutrophil cytoplasmic antibodies (ANCAs), is referred to as ANCA-associated vasculitis (AAV). ANCAs against proteinase 3 (PR3) (PR3-ANCA) or myeloperoxidase (MPO) (MPO-ANCA) are found in over 90% of patients with active disease, and these ANCAs are implicated in the pathogenesis of AAV. Dying neutrophils surrounding the walls of small vessels are a histological hallmark of AAV. Read More

View Article and Full-Text PDF

Central nervous system vasculitis in pediatric microscopic polyangiitis.

Acta Reumatol Port 2016 Oct-Dec;41(4):372-375

King George's Medical University, Lucknow.

Microscopic polyangiitis is a small vessel vasculitis, associated with myeloperoxidaseantineutrophil cytoplasmic antibody. It rarely occurs in children. Central nervous system involvement in pediatric microscopic polyangiitis is not a well known entity with perhaps only five cases till date. Read More

View Article and Full-Text PDF
January 2018

Hypersensitivity pneumonitis associated with azathioprine therapy in a patient with granulomatosis with polyangiitis.

Rheumatol Int 2016 Jul 7;36(7):1027-32. Epub 2016 May 7.

Division of Pulmonary and Critical Medicine, Department of Internal Medicine, Catholic University of Daegu School of Medicine, Daegu, Korea.

Granulomatosis with polyangiitis (GPA), an autoimmune disease characterized by inflammatory granulomas and necrotizing small-vessel vasculitis, primarily affects the respiratory tract and kidneys. Azathioprine (AZA) is a purine analog that is commonly used for maintaining GPA remission after induction therapy with cyclophosphamide. While the dose-dependent side effects of AZA are common and well known, hypersensitivity reactions such as pulmonary toxicity are rare. Read More

View Article and Full-Text PDF

The transition of renal histopathology after immunosuppressive therapy in a woman with renal limited ANCA-associated vasculitis: a case report and literature review.

Clin Exp Rheumatol 2016 May-Jun;34(3 Suppl 97):S115-20. Epub 2016 Feb 2.

Department of Nephrology, University of Hong Kong-Shenzhen Hospital, Shenzhen; and Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong, China.

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. Read More

View Article and Full-Text PDF
September 2016

Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis.

Biomed Res Int 2015 25;2015:402826. Epub 2015 Nov 25.

Division of Nephrology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA.

Rapidly progressive glomerulonephritis (RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN). From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG), anti-GBM GN, and immune complex GN. Read More

View Article and Full-Text PDF
September 2016