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Vasculitides

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https://www.readbyqxmd.com/read/28720136/bilateral-multiple-pulmonary-artery-aneurysms-associated-with-cavitary-pulmonary-tuberculosis-a-case-report
#1
Pedro Pallangyo, Frederick Lyimo, Smita Bhalia, Hilda Makungu, Bashir Nyangasa, Flora Lwakatare, Pal Suranyi, Mohamed Janabi
BACKGROUND: Pulmonary artery aneurysms constitute <1% of aneurysms occurring in the thoracic cavity. Congenital cardiac defects are responsible for the majority (>50%) of cases, however, pulmonary artery aneurysm is a rare sequelae of pulmonary tuberculosis reported in about 5% of patients with chronic cavitary tuberculosis on autopsy. The natural history of this potentially fatal condition remains poorly understood and guidelines for optimal management are controversial. CASE PRESENTATION: A 24-year-old man, a nursing student of African descent, was referred to us from an up-country regional hospital with a 4-week history of recurrent episodes of breathlessness, awareness of heartbeats and coughing blood 3 weeks after completing a 6-month course of anti-tuberculosis drugs...
July 19, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28710880/an-international-survey-on-anti-neutrophil-cytoplasmic-antibodies-anca-testing-in-daily-clinical-practice
#2
Jan Damoiseaux, Ingmar Heijnen, Christel Van Campenhout, Catharina Eriksson, Nicole Fabien, Manfred Herold, Renate G van der Molen, William Egner, Dina Patel, Aresio Plaza-Lopez, Antonella Radice, Marie José Rego de Sousa, Markku Viander, Yehuda Shoenfeld
BACKGROUND: Detection of anti-neutrophil cytoplasmic antibodies (ANCA) is important for the diagnosis of the ANCA-associated vasculitides (AAV). For AAV, especially ANCA directed against myeloperoxidase (MPO) and proteinase 3 (PR3) are most relevant. ANCA with less well-defined specificities may, however, also be detected in other inflammatory and non-inflammatory conditions. METHODS: A questionnaire, initiated by the European Autoimmunity Standardisation Initiative (EASI), was used to gather information on methods and testing algorithms used for ANCA in clinical laboratories of 12 European countries (EASI survey)...
July 15, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28710538/-manifestations-of-autoimmune-disorders-in-otorhinolaryngology-classical-symptoms-and-diagnostic-approach
#3
B Hofauer, A Chaker, K Thürmel, A Knopf
Manifestations of autoimmune disorders represent a diagnostic challenge. Many autoimmune disorders show typical manifestations in the ear, nose and throat and should be taken into account in the differential diagnosis. Sjogren's syndrome, various vasculitides and sarcoidosis represent the most common autoimmune disorders in otorhinolaryngology, but IgG4-related diseases, relapsing polychondritis and autoimmune disorders of the mucosa are also observed. Autoimmune diseases can lead to sensorineural deficits...
July 14, 2017: HNO
https://www.readbyqxmd.com/read/28678392/adding-azathioprine-to-remission-induction-glucocorticoids-for-eosinophilic-granulomatosis-with-polyangiitis-microscopic-polyangiitis-or-polyarteritis-nodosa-without-poor-prognosis-factors-a-randomized-controlled-trial
#4
Xavier Puéchal, Christian Pagnoux, Gabriel Baron, Thomas Quémeneur, Antoine Néel, Christian Agard, François Lifermann, Eric Liozon, Marc Ruivard, Pascal Godmer, Nicolas Limal, Arsène Mékinian, Thomas Papo, Anne-Marie Ruppert, Anne Bourgarit, Boris Bienvenu, Loïck Geffray, Jean-Luc Saraux, Elisabeth Diot, Bruno Crestani, Xavier Delbrel, Laurent Sailler, Pascal Cohen, Véronique Le Guern, Benjamin Terrier, Matthieu Groh, Claire Le Jeunne, Luc Mouthon, Philippe Ravaud, Loïc Guillevin
OBJECTIVE: Most patients with nonsevere systemic necrotizing vasculitides (SNVs) achieve remission with glucocorticoids alone but one-third relapse within 2 years. This study aimed to determine whether azathioprine adjunction to glucocorticoids could achieve a higher sustained remission rate of newly diagnosed nonsevere eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA) or polyarteritis nodosa (PAN). METHODS: All patients included in this double-blind trial received glucocorticoids, gradually tapered over 12 months, and were randomized to receive azathioprine or placebo for 12 months, with stratification according to SNV (EGPA or MPA/PAN)...
July 5, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28667559/vasculitis-in-the-central-nervous-system
#5
Anastasia Bougea, Nikolaos Spantideas
Central nervous system (CNS) vasculitides are a heterogeneous group of disorders characterized by an inflammatory cell infiltration and necrosis of blood vessel walls in the brain, spinal cord, and the meninges. The CNS complications are likely to be fatal without judicious use of immunosuppression; thus, early diagnosis may prevent from damage and disability. This chapter updates our knowledge on CNS vasculitis-related immunological mechanisms, neurological complications, diagnosis, and management.
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28662606/patients-with-vasculitides-admitted-to-the-intensive-care-unit-implications-from-a-single-center-retrospective-study
#6
Yael Haviv, Ora Shovman, Nicola Luigi Bragazzi, Kassem Sharif, Yarden Yavne, Yehuda Shoenfeld, Howard Amital, Abdulla Watad
BACKGROUND: Vasculitides are a group of disorders characterized by inflammation of vessels. Vasculitides may have life-threatening complications with significant morbidity and mortality; however, information regarding the outcome and prognosis of patients with vasculitides requiring intensive care unit (ICU) is scarce. METHODS: Data of patients with vasculitides admitted to the ICU of the Sheba Medical Center between the years 2000 and 2014 were retrieved retrospectively...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28638968/-large-vessel-vasculitis-giant-cell-arteritis-and-takayasu-arteritis
#7
P M Villiger
According to the Chapel Hill Classification, large vessel vasculitides encompass giant cell arteritis (GCA) and the histologically related Takakaysu arteritis (TAK). The two diseases lack autoantibodies and present with a systemic inflammatory response. GCA typically shows a sudden onset with profound sickness, loss of appetite and of body weight, and temporal headache. Due to the substantial risk of sudden blindness, diagnostic work-up has to be performed immediately and treatment started without delay. A close association between polymyalgia rheumatica (PMR) and GCA is well established...
June 21, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28630775/a-rare-case-of-paraneoplastic-aortitis-associated-with-chronic-myelomonocytic-leukemia
#8
Sylwia Sasinowska, Pamela Traisak, Michael McCormack, Hala Eid
Aortitis is a broad term describing inflammation of the aorta. The most common causes of aortitis are the large-vessel vasculitides giant cell arteritis and Takayasu arteritis. Other etiologies include aortitis associated with other autoimmune disorders, infectious causes, and paraneoplastic and idiopathic cases. We describe a rare case of a large-vessel arteritis occurring in association with chronic myelomonocytic leukemia (CMML). A 68-year-old female with recent diagnosis of CMML presented to our office for evaluation of abnormal chest computed tomography (CT) that showed inflammation surrounding the entirety of thoracic and abdominal aorta, consistent with aortitis...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28623488/vasculitis-for-the-internist-focus-on-anca-associated-vasculitis
#9
REVIEW
Benjamin Chaigne, Loïc Guillevin
Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality...
August 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28619978/rheumatoid-arthritis-causing-diffuse-alveolar-haemorrhage-a-novel-therapeutic-approach
#10
Afaf Osman, Panagis Galiatsatos, Sonali Bose, Sonye Danoff
Pulmonary vascular involvement due to rheumatoid arthritis, presenting as diffuse alveolar haemorrhage (DAH), is a rare phenomenon, especially if there are no signs of systemic vasculitides. Furthermore, how to proceed with the management of these patients is challenging, as in the case of our patient, who had recurrent DAH. We present a case of a patient with known rheumatoid arthritis who had recurrence of DAH that spanned over several years, often presenting with life-threatening respiratory failure. While her DAH presentation improved with high-dose glucocorticoids, to resolve her recurrence, we opted to initiate treatment with rituximab, with a short course of azathioprine...
June 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28612332/cocaine-levamisole-induced-vasculitis-vasculopathy-syndrome
#11
REVIEW
Javier Marquez, Lina Aguirre, Carolina Muñoz, Andres Echeverri, Mauricio Restrepo, Luis F Pinto
PURPOSE OF REVIEW: To understand the clinical spectrum of cocaine-levamisole-induced vasculitis. Worldwide recreational drug consumption is high among the adult population from various social strata. The use of cocaine with levamisole, a frequently added antiparasitic diluent, favors the manifestations of vasculitic lesions, especially in the skin. RECENT FINDINGS: New insights into immunological mechanisms involved in the pathogenesis of the disease. There are still many unknown aspects in the pathogenesis of this disease, such as the immune system interaction with p-ANCAs and the release of inflammatory NETs (neutrophil extracellular traps), which are the origin of auto-antigens and tissue damage, manifesting as vasculitic purpura on the skin...
June 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28612242/histopathologic-classification-of-anti-neutrophil-cytoplasmic-antibody-associated-glomerulonephritis-achievements-limitations-and-perspectives
#12
Andrea Hinojosa-Azaola, Ariadna Jiménez-González
Clinical and histological factors have been identified as predictors of early and late renal outcome in ANCA-associated vasculitides (AAV). The presence and severity of kidney involvement at diagnosis are associated with poor prognosis in both patient and renal survival. Histologic findings remain the gold standard for diagnosing patients with AAV. In order to quantify the extent of the morphological parameters in the renal biopsies and to identify the histopathological lesions that predict renal outcome, several scoring systems have been proposed to systematically assess kidney biopsies in AAV...
June 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28611282/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-year-observation-study
#13
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
May 23, 2017: Oncotarget
https://www.readbyqxmd.com/read/28602810/apheresis-to-treat-systemic-vasculitis
#14
Julie Moussi-Frances, Marion Sallée, Noémie Jourde-Chiche
Apheresis has been used in the treatment of severe systemic vasculitides, in conjunction with immunosuppressive therapies, for over 40 years. The aim is to rapidly remove autoantibodies or circulating immune complexes from the plasma. The two main indications at present are vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) manifesting as severe renal involvement and/or intraalveolar hemorrhage and antiglomerular basement membrane disease (Goodpasture syndrome). The ongoing PLEXIVAS randomized controlled trial is assessing plasmapheresis to treat ANCA-associated vasculitis with or without severe renal involvement or intraalveolar hemorrhage...
June 7, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28602438/vasculitides-induced-by-cocaine-and-or-levamisole
#15
EDITORIAL
Antoine Néel, Christian Agard, Mohamed Hamidou
No abstract text is available yet for this article.
July 10, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28591497/-anca-associated-vasculitides-at-mexico-city-s-metropolitan-eastern-area
#16
Hernando Cervera-Castillo, Francisco Blanco-Favela, Yolanda Floriberta Silva-López, Guadalupe Eugenia Paredez-Rivera, Verónica Torres-Caballero
BACKGROUND: The anti-neutrophil cytoplasmic antibody-associated (ANCA) vasculitides includes granulomatosis with polyangiitis (Wegener's) (GPW), Chrug-Strauss syndrome (CSS) and microscopic polyangiitis (MPA). Since it has low incidence in our field, there are a few published papers. The aim of this study was to report the clinical characteristics, activity and damage of these vasculitides in Mexico City's metropolitan eastern area. METHODS: We studied 28 cases with ANCA-associated vasculitis in the aforementioned area...
July 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28589389/-safety-and-efficacy-of-off-label-use-of-biologic-therapies-in-patients-with-inflammatory-rheumatic-diseases-refractory-to-standard-of-care-therapy-data-from-a%C3%A2-nationwide-german-registry-graid2
#17
F Proft, H Schulze-Koops, M Grunke, E Schrezenmeier, F Halleck, J Henes, L Unger, E Schmidt, C Fiehn, A Jacobi, C Iking-Konert, C Kneitz, R E Schmidt, B Bannert, R E Voll, R Fischer-Betz, I Kötter, H P Tony, J Holle, M Aringer, A Erler, F Behrens, G R Burmester, T Dörner
BACKGROUND: The German Registry of Autoimmune Diseases 2 (GRAID2) is a retrospective, non-interventional, multicenter registry study collecting data from patients with inflammatory, mainly rheumatic diseases refractory to standard of care therapy and treated with an off-label biologic therapy. The retrospective documentation comprised case history, diagnosis, course of disease (including safety and global efficacy). The objective was to evaluate the global clinical outcome and safety of off-label biologic therapy in clinical practice...
June 6, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28582318/recent-advances-in-childhood-vasculitis
#18
Seza Ozen, Nazire Pinar Acar-Ozen
PURPOSE OF REVIEW: The review aims to summarize the recent findings in vasculitis that may have an impact in our understanding or management of these diseases. RECENT FINDINGS: We are learning more about monogenic diseases that closely mimic the pediatric vasculitides. Deficiency of adenosine deaminase 2 can present with a polyarteritis nodosa (PAN)-like picture and should be included in the differential of all pediatric cases of PAN with a family history or in cases with early stroke, or in cases resistant to conventional therapy...
June 2, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28569178/prediction-of-response-to-remission-induction-therapy-by-gene-expression-profiling-of-peripheral-blood-in-japanese-patients-with-microscopic-polyangiitis
#19
Akihiro Ishizu, Utano Tomaru, Sakiko Masuda, Ken-Ei Sada, Koichi Amano, Masayoshi Harigai, Yasushi Kawaguchi, Yoshihiro Arimura, Kunihiro Yamagata, Shoichi Ozaki, Hiroaki Dobashi, Sakae Homma, Yasunori Okada, Hitoshi Sugiyama, Joichi Usui, Naotake Tsuboi, Seiichi Matsuo, Hirofumi Makino
BACKGROUND: Microscopic polyangiitis (MPA), which is classified as an anti-neutrophil cytoplasmic antibody (ANCA)-associated small vessel vasculitis, is one of the most frequent primary vasculitides in Japan. We earlier nominated 16 genes (IRF7, IFIT1, IFIT5, OASL, CLC, GBP-1, PSMB9, HERC5, CCR1, CD36, MS4A4A, BIRC4BP, PLSCR1, DEFA1/DEFA3, DEFA4, and COL9A2) as predictors of response to remission induction therapy against MPA. The aim of this study is to determine the accuracy of prediction using these 16 predictors...
May 31, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28559644/severe-microscopic-polyangiitis-with-unilateral-vocal-cord-paralysis-as-initial-manifestation
#20
Luis Felipe Flores-Suárez, Marco Antonio Alba, Gabriel Tona
CASE DESCRIPTION: A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement. CLINICAL FINDINGS: Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis...
March 30, 2017: Colombia Médica: CM
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