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Vasculitides

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https://www.readbyqxmd.com/read/28447661/the-nonsystemic-vasculitic-neuropathies
#1
REVIEW
Michael P Collins, Robert D Hadden
Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy...
April 27, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28442217/vasculopathic-and-vasculitic-dermatoses
#2
REVIEW
Alejandro A Gru, Andrea L Salavaggione
The inflammatory and non-inflammatory disorders affecting the blood vessels are an important cause of death among patients. Additionally, some of the findings could be very subtle, while the consequences of misdiagnosis tragic. There is a limited number of factors by which the cutaneous blood vessels can be affected and the most common examples in the clinical practice will be discussed here: non-inflammatory purpuras; vascular-occlusive disorders; urticarias; vasculitides; and neutrophilic dermatoses.
April 7, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28432167/a-tale-of-two-vasculitides-biopsy-proven-giant-cell-arteritis-followed-by-the-independent-development-of-renal-limited-microscopic-polyangiitis
#3
Roman Zuckerman, Mayur Patel, Deborah R Alpert
We describe an 80-year-old woman who presented with headaches, bilateral jaw claudication and visual symptoms. She was diagnosed with giant cell arteritis, which was confirmed by temporal artery biopsy. She was treated with high-dose prednisone followed by a slow taper, with complete resolution of symptoms. Approximately 4 years later, she developed progressively worsening renal function associated with haematuria. Serological workup revealed a high-titre perinuclear antinuclear cytoplasmic antibody and antibodies to myeloperoxidase...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28420068/pentraxin-3-levels-are-associated-with-vasculitis-and-disease-activity-in-childhood-onset-systemic-lupus-erythematosus
#4
S Sahin, A Adrovic, K Barut, S Durmus, R Gelisgen, H Uzun, O Kasapcopur
Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28406765/maintenance-treatment-of-anca-associated-vasculitides
#5
REVIEW
Loic Guillevin
ANCA-associated vasculitis(AAV)covers a group of systemic necrotising vasculitides characterised by inflammation of small vessels, sometimes with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). Potentially lethal if not promptly diagnosed and treated, AAV in most patients can be induced into remission with the current treatment modalities. However, the risk of relapse remains high, necessitating prolonged immunosuppressive or immunomodulating maintenance therapy, whose optimal duration remains undetermined...
April 5, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28379475/are-the-1990-american-college-of-rheumatology-vasculitis-classification-criteria-still-valid
#6
Benjamin Seeliger, Jan Sznajd, Joanna C Robson, Andrew Judge, Anthea Craven, Peter C Grayson, Ravi S Suppiah, Richard A Watts, Peter A Merkel, Raashid A Luqmani
Objectives.: Advances in diagnostic techniques have led to better distinction between types of vasculitis, potentially affecting the utility of the 1990 ACR classification criteria for vasculitis. This study tested the performance of these criteria in a contemporary vasculitis cohort. Methods.: The Diagnosis and Classification in Vasculitis Study provided detailed clinical, serological, pathological and radiological data from patients with primary systemic vasculitis and clinical context-specific comparator conditions...
March 31, 2017: Rheumatology
https://www.readbyqxmd.com/read/28378796/corrigendum-analysis-of-the-common-genetic-component-of-large-vessel-vasculitides-through-a-meta-immunochip-strategy
#7
F David Carmona, Patrick Coit, Güher Saruhan-Direskeneli, José Hernández-Rodríguez, María C Cid, Roser Solans, Santos Castañeda, Augusto Vaglio, Haner Direskeneli, Peter A Merkel, Luigi Boiardi, Carlo Salvarani, Miguel A González-Gay, Javier Martín, Amr H Sawalha
No abstract text is available yet for this article.
April 5, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28377447/update-on-systemic-vasculitides
#8
REVIEW
Siddiq Anwar, M Yousuf Karim
Management of systemic vasculitis has been revolutionised over the last decade with the introduction of targeted biological agents. With an increase in both the prevalence and the recognition of vasculitis as well as the high cost of these agents, it is important to ensure their most optimal utilisation. The goals of vasculitis therapy include the induction and maintenance of remissions, preventing relapses, reducing the toxicity of therapy with the aim of reducing morbidity and mortality as well as improving the quality of life of those afflicted...
April 4, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28375834/the-role-of-ultrasound-in-the-diagnosis-and-follow-up-of-large-vessel-vasculitis-an-update
#9
Giuseppe Germanò, Sara Monti, Cristina Ponte, Niccolo' Possemato, Roberto Caporali, Carlo Salvarani, Pierluigi Macchioni, Nicolò Pipitone
Large-vessel vasculitides comprise giant cell arteritis and Takayasu's arteritis. In both conditions, early changes consist of transmural inflammation of the arterial wall, while later complications include lumen changes, such as stenoses or aneurysms. Colour-Doppler sonography has the ability to depict the arterial wall as well as the lumen, and is therefore ideally suited both to diagnose early vasculitis and to monitor patients over time. In this review article, we addressed the following issues: 1) the role of colour-Doppler sonography in the diagnosis of large-vessel vasculitis and its common pitfalls; 2) whether colour-Doppler sonography can increase the yield of temporal artery biopsy in giant cell arteritis; 3) the role of colour-Doppler sonography in monitoring patients with LVV over time; and 4) how colour-Doppler sonography performs compared to other imaging techniques...
March 29, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28361331/anca-associated-vasculitis-pathogenesis-a-commentary
#10
REVIEW
Eric J Gapud, Philip Seo, Brendan Antiochos
PURPOSE OF REVIEW: The ANCA-associated vasculitides are a group of small vessel vasculitides characterized by autoantibodies recognizing the neutrophil cytoplasmic antigens PR3 and MPO. We examine the current clinical and molecular immunology understanding of ANCA-associated vasculitides and discuss the current needs in our understanding of the pathogenic mechanisms of these rare diseases. RECENT FINDINGS: The majority of efforts to understand the pathogenesis of these diseases have focused on dissecting neutrophil biology because the neutrophil is the primary expressor of ANCA autoantigens...
April 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28355983/mesenteric-inflammatory-veno-occlusive-disease-of-the-spleen-metasynchronous-with-two-arterial-thrombotic-events-in-systemic-lupus-erythematosus
#11
D Allali, G Puppa, C Chizzolini
Vasculitides, particularly those affecting small vessels, are known to complicate systemic lupus erythematosus (SLE); however, isolated venulitis of the mesenteric bed has rarely been reported. Here we relate the case of a 46-year-old woman with SLE who presented with acute abdominal pain due to artery thrombosis and extended splenic ischemia requiring splenectomy. The histological examination revealed diffuse venulitis in the absence of arterial vasculitis consistent with the definition of mesenteric inflammatory veno-occlusive disease (MIVOD)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28337408/eosinophilic-granulomatosis-with-polyangiitis-formerly-known-as-churg-strauss-syndrome-as-a-differential-diagnosis-of-hypereosinophilic-syndromes
#12
Yuri Albuquerque Pessoa Santos, Bruno Rangel Antunes Silva, Pollyanna Natividade Zanconato Barros Assis Lira, Luiz Carlos Aguiar Vaz, Thiago Thomaz Mafort, Leonardo Palermo Bruno, Agnaldo José Lopes
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28332442/ultrasound-imaging-in-the-diagnosis-of-large-vessel-vasculitis
#13
Michael Czihal, Christian Lottspeich, Ulrich Hoffmann
Nowadays noninvasive vascular imaging has an important role in the diagnostic work-up of the large vessel vasculitides (LVV), most importantly giant cell arteritis (GCA) and Takayasu arteritis. Among the imaging modalities available, ultrasound (US) has several important advantages, including low costs, rapid and repetitive availability without exposure to radiation, and high spatial resolution for assessment of large and medium-sized arteries. Therefore, US can be regarded the first line imaging method in suspected LVV...
March 23, 2017: VASA. Zeitschrift Für Gefässkrankheiten
https://www.readbyqxmd.com/read/28306595/update-on-maintenance-therapy-for-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#14
Ora Singer, W Joseph McCune
PURPOSE OF REVIEW: The antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitides are a group of rare systemic diseases. The past several years have seen major therapeutic advances in the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The success rate in induction of remission is high, but reducing the high incidence of relapses remains a therapeutic challenge. RECENT FINDINGS: Studies have shown no improvement in relapse rates in GPA and MPA over the past 2 decades...
May 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28277489/analysis-of-the-common-genetic-component-of-large-vessel-vasculitides-through-a-meta-immunochip-strategy
#15
F David Carmona, Patrick Coit, Güher Saruhan-Direskeneli, José Hernández-Rodríguez, María C Cid, Roser Solans, Santos Castañeda, Augusto Vaglio, Haner Direskeneli, Peter A Merkel, Luigi Boiardi, Carlo Salvarani, Miguel A González-Gay, Javier Martín, Amr H Sawalha
Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are major forms of large-vessel vasculitis (LVV) that share clinical features. To evaluate their genetic similarities, we analysed Immunochip genotyping data from 1,434 LVV patients and 3,814 unaffected controls. Genetic pleiotropy was also estimated. The HLA region harboured the main disease-specific associations. GCA was mostly associated with class II genes (HLA-DRB1/HLA-DQA1) whereas TAK was mostly associated with class I genes (HLA-B/MICA). Both the statistical significance and effect size of the HLA signals were considerably reduced in the cross-disease meta-analysis in comparison with the analysis of GCA and TAK separately...
March 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28242394/successful-management-of-arterial-pseudoaneurysm-caused-by-stent-fracture
#16
Maham Rahimi, Brent Robertson, Lynne M Doctor, Jonathan Bath
Pseudoaneurysm is a relatively common complication of arterial injury. Arterial pseudoaneurysms have many different etiologies, including trauma, iatrogenic injury, vascular repair, infection, and vasculitides. In this case report, we present a pseudoaneurysm created by a fracture of superficial femoral artery stent secondary to a mechanical fall. To our knowledge, this is the first report of a symptomatic pseudoaneurysm caused by a superficial femoral artery stent fracture. The large, unruptured pseudoaneurysm in this case was successfully treated with a covered stent and pseudoaneurysm exclusion...
February 24, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28229830/neutrophil-extracellular-traps-formation-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-association-with-eosinophilic-inflammation
#17
Joanna Natorska, Michał Ząbczyk, Jakub Siudut, Piotr Krawiec, Lucyna Mastalerz, Anetta Undas
OBJECTIVES: Eosinophilic granulomatosis with polyangiitis (EGPA) is associated with an inflammation and the presence of antineutrophil cytoplasmic antibodies (ANCA). Thus, we investigated the impact of ANCAs and eosinophilic inflammation on neutrophil activation and extracellular traps (NETs) formation. METHODS: We recruited 29 patients in the remission of EGPA (17 ANCA-negative and 12 ANCA-positive, including 7 p-ANCA-positive and 5 c-ANCA-positive patients). Healthy donors' neutrophils were stimulated with EGPA patients' serum...
February 9, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28225490/clinical-characteristics-and-outcome-of-spanish-patients-with-anca-associated-vasculitides-impact-of-the-vasculitis-type-anca-specificity-and-treatment-on-mortality-and-morbidity
#18
MULTICENTER STUDY
Roser Solans-Laqué, Guadalupe Fraile, Monica Rodriguez-Carballeira, Luis Caminal, Maria J Castillo, Ferran Martínez-Valle, Luis Sáez, Juan J Rios, Xavier Solanich, Joaquim Oristrell, Francisco Pasquau, Eva Fonseca, Monica Zamora, Jose L Callejas, Begoña Frutos, Monica Abdilla, Patricia Fanlo, Isabel García-Sánchez, Miguel López-Dupla, Bernardo Sopeña, Almudena Pérez-Iglesias, Josep A Bosch
The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA)...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28184292/rituximab-therapy-in-pemphigus-and-other-autoantibody-mediated-diseases
#19
REVIEW
Nina A Ran, Aimee S Payne
Rituximab, a monoclonal antibody targeting the B cell marker CD20, was initially approved in 1997 by the United States Food and Drug Administration (FDA) for the treatment of non-Hodgkin lymphoma. Since that time, rituximab has been FDA-approved for rheumatoid arthritis and vasculitides, such as granulomatosis with polyangiitis and microscopic polyangiitis. Additionally, rituximab has been used off-label in the treatment of numerous other autoimmune diseases, with notable success in pemphigus, an autoantibody-mediated skin blistering disease...
2017: F1000Research
https://www.readbyqxmd.com/read/28157661/eosinophilic-endomyocardial-disease
#20
Victor Y Liu, Raymundo A Quintana-Quezada, L Maximilian Buja
Eosinophilic granulomatosis with polyangiitis is the rarest of the antineutrophil cytoplasmic antibody-associated vasculitides. We describe the case of a 58-year-old woman with a history of asthma found to have a left ventricular apical thrombus. Cardiac catheterization with endomyocardial biopsy was consistent with eosinophilic endomyocardial disease. The disease affects several organ systems and shares many features with more common conditions, making it a difficult diagnosis.
March 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
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