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NCBI: db=pubmed; Term=vasculitis AND ((English[lang] OR French[lang]) AND adult[MeSH] AND "last 90 days"[PDat])
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The ocular involvement did not accompany with the genital ulcer or the gastrointestinal symptoms at the early stage of Behçet's disease.

mar, 07/05/2019 - 13:29
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The ocular involvement did not accompany with the genital ulcer or the gastrointestinal symptoms at the early stage of Behçet's disease.

Mod Rheumatol. 2019 Mar;29(2):357-362

Authors: Suwa A, Horita N, Ishido T, Takeuchi M, Kawagoe T, Shibuya E, Yamane T, Hayashi T, Meguro A, Ishido M, Minegishi K, Yoshimi R, Kirino Y, Kato S, Arimoto J, Fukumoto T, Ishigatsubo Y, Kurosawa M, Kaneko T, Takeno M, Mizuki N

Abstract
OBJECTIVES: This study aimed to identify patients with high-probability of ocular involvement of Behçet's disease (BD).
METHODS: The Japanese Ministry of Health, Labour and Welfare provided dataset of ongoing nationwide BD registration project. A patient who had confirmed BD and who was suspected to have BD was registered. We mainly analyzed newly registered patients who had the data for all demographic and diagnostic parameters regardless of fulfilment of any diagnostic criteria.
RESULTS: Among 3213 patients with confirmed or possible BD, 1382 (43.0%) were men and 1831 (57.0%) were women with a median age of 38 years (interquartile range (IQR) 30-49 years). The median duration between onset and registration was 0 year (IQR 0-3). A binomial multivariable logistic regression analysis revealed that being female (odds ratio (OR) 0.63, 95% confidence interval (CI) 0.53-0.75, p < .001), duration since onset (OR 1.33 per 10 years, 95% CI 1.18-1.51, p < .001), genital ulceration (OR 0.28, 95% CI 0.23-0.34, p < .001), and gastrointestinal symptoms (OR 0.36, 95% CI 0.30-0.44, p < .001) were related to the ocular lesion. Analyses based on data of 2800 patients who satisfied International criteria of BD, age-, sex-, duration-based subgroup analyses, analyses targeting iridocyclitis and retino-uveitis, and analysis including patients with missing data confirmed that the four factors were associated with the probability of eye involvement.
CONCLUSION: The ocular involvement did not accompany with genital ulcer or gastrointestinal symptoms at the early stage of BD.

PMID: 29583036 [PubMed - indexed for MEDLINE]

Asymptomatic rheumatoid meningitis revealed by magnetic resonance imaging, followed by systemic rheumatic vasculitis: A case report and a review of the literature.

mar, 07/05/2019 - 13:29
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Asymptomatic rheumatoid meningitis revealed by magnetic resonance imaging, followed by systemic rheumatic vasculitis: A case report and a review of the literature.

Mod Rheumatol. 2019 Mar;29(2):370-376

Authors: Matsuda S, Yoshida S, Takeuchi T, Fujiki Y, Yoshikawa A, Makino S

Abstract
We report the case of a 66-year-old man with seropositive rheumatoid arthritis who developed neurologically asymptomatic rheumatoid meningitis (RM) revealed by MRI. RM worsened and chest CT showed pericardial effusion, pleural effusion, and bilateral consolidation, and his serum C3 level was decreased. We diagnosed systemic rheumatic vasculitis based on these findings. After a review of more than 20 previously reported cases of RM, this is the first case of RM without central nerve system symptoms.

PMID: 27659704 [PubMed - indexed for MEDLINE]

Nonthrombotic proliferative vasculopathy associated with antiphospholipid antibodies: A case report and literature review.

mar, 07/05/2019 - 13:29
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Nonthrombotic proliferative vasculopathy associated with antiphospholipid antibodies: A case report and literature review.

Mod Rheumatol. 2019 Mar;29(2):388-392

Authors: Lee JS, Kim H, Lee EB, Song YW, Park JK

Abstract
A 20-year-old man presented with recurrent hemoptysis for seven months. A small subpleural nodule in his right lower lobe was found and excised surgically. Based on the presence of antiphospholipid antibodies (aPL) and vascular wall hypertrophy without vasculitis or an intraluminal thrombus, nonthrombotic proliferative vasculopathy (NTPV) affecting pulmonary arteries was diagnosed. Recently, aPL have been postulated to directly induce the proliferation of vascular cells in the intima and media, leading to NTPV. We review 5 cases of NTPV-associated aPL with critical ischemia in the lower extremities and gastrointestinal infarction. NTPV-associated aPL might be distinct from classic antiphospholipid syndrome and should be considered in aPL-positive patients who present with vascular occlusions of medium-sized vessels in the absence of atherosclerotic risk factors and systemic or local inflammation.

PMID: 27535710 [PubMed - indexed for MEDLINE]

[From isolated cutaneous vasculitis to systemic disease : a diagnostic approach to purpura in the adult patient].

sam, 04/05/2019 - 10:25
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[From isolated cutaneous vasculitis to systemic disease : a diagnostic approach to purpura in the adult patient].

Rev Med Suisse. 2019 Apr 03;15(645):713-718

Authors: Horisberger A, Ghosn J, Di Lucca J, Comte D

Abstract
Purpura can be a clinical manifestation of various diseases. The causes of purpura are divided into two main categories: thrombocytopenia and vasculopathies. Cutaneous vasculitis belongs to the latter group. Cutaneous vasculitis should be considered a symptom rather than a medical entity. Some forms of cutaneous vasculitis are limited to the skin and are known as isolated cutaneous vasculitis, while other forms may be part of a systemic disease with a more serious prognosis. It is essential to clarify the type and severity of the disease for optimal patient care. A delay in the identification and start of treatment can be the cause of serious and potentially irreversible complications. Through this article, we will propose a step-by-step approach from diagnosis to patient care.

PMID: 30942968 [PubMed - indexed for MEDLINE]

Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): case series of three patients with multimodal imaging.

sam, 04/05/2019 - 10:25
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Idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN): case series of three patients with multimodal imaging.

Graefes Arch Clin Exp Ophthalmol. 2019 May;257(5):1013-1018

Authors: Chong YJ, Logeswaran A, Sarmad A, Damato EM

Abstract
PURPOSE: The purpose of this study was to report the clinical presentation, disease progression, treatment and complications of IRVAN.
METHOD: Case series PATIENTS: Six eyes from three patients were included.
RESULTS: All eyes were treated with pan-retinal photocoagulation (PRP). One eye received Ozurdex (dexamethasome implant) for persistent macular exudates and oedema. One eye received Avastin injections for retinal neovascularization. Oral steroids were given to all patients at some point during the disease process. One patient had additional immunosuppression with mycophenolate mofetil. Despite aggressive PRP, the visual outcomes varied widely. One patient maintained 6/6 vision bilaterally at 84 months follow-up. The second patient had progressive visual loss secondary to macular exudates and oedema, from 6/9 right eye, 6/6 left eye to 6/18 right eye, 6/60 left eye within 12 months despite Ozurdex injection. The third patient's vision at presentation was 6/5 right eye, and 6/4 left eye. Despite further interventions including Avastin and mycophenolate mofetil, he continued to have progressive neovascularization and recurrent vitreous haemorrhage. At 72 months, his vision had deteriorated to 6/60 right eye, 6/18 left eye.
CONCLUSION: The progression of IRVAN can vary greatly, in spite of aggressive treatment with PRP, oral and intravitreal steroids, immunosuppressant medication and anti-VEGF agents. The variation in disease progression occurs both within the same individual as well as between individuals. An individualised approach to therapy is advocated.

PMID: 30877369 [PubMed - indexed for MEDLINE]

Vitamin C deficiency: rare cause of severe anemia with hemolysis.

sam, 04/05/2019 - 10:25
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Vitamin C deficiency: rare cause of severe anemia with hemolysis.

Int J Hematol. 2019 May;109(5):618-621

Authors: Shaikh H, Faisal MS, Mewawalla P

Abstract
Historically known to be a disease of sailors and soldiers in the seventeenth and eighteenth century, scurvy is a rare nutritional deficiency in the developed world, but it can still be seen among the alcoholics and the malnourished. We present a case of a 39-year-old alcoholic male who presented with progressive fatigue and diffuse purpuric rash with scattered ecchymosis for 2 months. Blood work was remarkable for hemoglobin of 9.1 g/dl, which further dropped to 7 g/dl over the next few days. He was then found to have hemolysis on lab work. After an extensive workup, the common causes of hemolytic anemia were ruled out, vitamin C level was checked, which interestingly resulted as 0 mg/dl. Supplementation with oral vitamin C resulted in the gradual resolution of hemolytic anemia and rash. Hemoglobin improved to 15 g/dl in 4 weeks, with normalization of vitamin C level. The clinical features of scurvy can easily be confused with conditions such as vasculitis, deep venous thrombosis, and systemic bleeding disorders. Therefore, comprehensive workup up is required prior to the diagnosis. Although rare, being a reversible condition, early diagnosis and treatment of scurvy in high-risk populations cannot be stressed enough.

PMID: 30666502 [PubMed - indexed for MEDLINE]

Ustekinumab treatment is associated with decreased systemic and vascular inflammation in patients with moderate-to-severe psoriasis: Feasibility study using 18F-fluorodeoxyglucose PET/CT.

mer, 01/05/2019 - 08:49
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Ustekinumab treatment is associated with decreased systemic and vascular inflammation in patients with moderate-to-severe psoriasis: Feasibility study using 18F-fluorodeoxyglucose PET/CT.

J Am Acad Dermatol. 2019 May;80(5):1322-1331

Authors: Kim BS, Lee WK, Pak K, Han J, Kim GW, Kim HS, Ko HC, Kim MB, Kim SJ

Abstract
BACKGROUND: Evidence suggests that psoriasis might be associated with metabolic syndrome and an increased risk for cardiovascular disease.
OBJECTIVE: To determine whether ustekinumab reduces systemic and vascular inflammation associated with metabolic syndrome and cardiovascular disease, measured using 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT).
METHODS: Patients with psoriasis and healthy controls underwent baseline 18F-FDG PET/CT imaging. Patients with moderate-to-severe psoriasis were treated with ustekinumab and underwent 18F-FDG PET/CT again after a Psoriasis Area and Severity Index of 75 was achieved.
RESULTS: After a Psoriasis Area and Severity Index of 75 was achieved with ustekinumab treatment, standardized uptake values were reduced in the liver, spleen, and 5 parts of the aorta (P < .05).
LIMITATIONS: Our study does not provide outcome data concerning cardiovascular events or metabolic syndrome; it only shows surrogate markers in a limited (Korean) population.
CONCLUSION: Ustekinumab treatment was significantly associated with decreased systemic and vascular inflammation related to metabolic syndrome and cardiovascular disease among patients with psoriasis.

PMID: 29559399 [PubMed - indexed for MEDLINE]

Anti-neutrophil cytoplasmic antibody associated central nervous system vasculitis with brain and spinal cord subarachnoid hemorrhage: A rare case report and review of the literature.

dim, 28/04/2019 - 05:44
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Anti-neutrophil cytoplasmic antibody associated central nervous system vasculitis with brain and spinal cord subarachnoid hemorrhage: A rare case report and review of the literature.

J Clin Neurosci. 2019 Apr;62:253-255

Authors: Harland TA, Seinfeld J, Cava LF, Neumann RT, Roark C, Kumpe D, Case D

Abstract
This case report describes a 48-year old female who presented with altered mental status, lower extremity weakness, low back pain and a recent history of subjective fevers and night sweats found to have posterior parieto-occipital and spinal subarachnoid hemorrhage on imaging. Further work-up revealed vasculitic changes in the intracranial vasculature and the external carotid artery on angiography. She also demonstrated positivity for perinuclear anti-neutrophil cytoplasmic (p-ANCA) antibodies overall consistent with ANCA associated central nervous system vasculitis (AAV). The present case describes a rare and new presentation of AAV that caused both a cerebral and spinal subarachnoid hemorrhage. There has been no documentation of spinal subarachnoid hemorrhage associated with primary or secondary vasculitis in the literature. Ultimately, this case demonstrates the important finding that AAV can have spinal cord manifestations and cervical vasculature involvement along with the more classic intra-cranial vasculitis findings.

PMID: 30594448 [PubMed - indexed for MEDLINE]

Acute myocardial infarction as the first manifestation of Takayasu arteritis: A case report.

jeu, 25/04/2019 - 03:15
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Acute myocardial infarction as the first manifestation of Takayasu arteritis: A case report.

Medicine (Baltimore). 2019 Apr;98(15):e15143

Authors: Zhang T, Peng B, Tu X, Zhang S, Zhong S, Cao W

Abstract
RATIONALE: Takayasu arteritis (TA) is a chronic inflammatory disease involving the aorta and its major branches. Initial diagnosis is usually difficult due to the highly variable symptoms. Acute myocardial infarction (AMI) is a very rare presentation in patients with TA. Moreover, the choice of early management for these patients is not well established.
PATIENT CONCERNS: A 34-year-old woman was taken to the Emergency Department of our hospital, presenting with a sudden onset and persistent retrosternal chest pain radiating to both upper extremities for 2 hours. Blood pressures were different between 2 arms with 151/115 mm Hg on the right arm and 140/100 mm Hg on the left arm.
DIAGNOSES: The patient was diagnosed with TA according to the medical history, physical examination, and vascular imaging.
INTERVENTIONS: Primary percutaneous coronary intervention (PPCI) was performed to restore the coronary flow of left anterior descending. Meanwhile, combination of oral glucocorticoids and immunosuppressive agents was administered to halt disease progression of TA.
OUTCOMES: Chest pain was relieved without rest and exertional angina. The patient achieved long-term remission without symptom relapse during our follow-up.
LESSONS: Percutaneous coronary intervention was essential and effective in AMI of TA. Timely immunosuppressive therapy could improve the long-term outcome.

PMID: 30985685 [PubMed - indexed for MEDLINE]

Anaphylactoid Syndrome of Pregnancy.

jeu, 25/04/2019 - 03:15
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Anaphylactoid Syndrome of Pregnancy.

Nurs Womens Health. 2019 Feb;23(1):38-48

Authors: Barnhart ML, Rosenbaum K

Abstract
Anaphylactoid syndrome of pregnancy (ASP) is a widespread, proinflammatory, anaphylactic-like reaction that can occur when amniotic fluid enters the maternal blood circulation. ASP is characterized by four cardinal findings: respiratory distress, altered mental status, hypotension, and disseminated intravascular coagulation. ASP is commonly associated with maternal and neonatal mortality. Early recognition followed by prompt and aggressive treatment can improve survival rates and are among the most critical activities for nurses and other clinicians caring for women with ASP.

PMID: 30738557 [PubMed - indexed for MEDLINE]

Long-term results after open repair of inflammatory infrarenal aortic aneurysms.

jeu, 25/04/2019 - 03:15
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Long-term results after open repair of inflammatory infrarenal aortic aneurysms.

J Vasc Surg. 2019 02;69(2):440-447

Authors: Wieker CM, von Stein P, Bianchini Massoni C, Rengier F, Böckler D, Geisbüsch P

Abstract
OBJECTIVE: The objective of this study was to investigate the long-term outcome after open repair of inflammatory infrarenal aortic aneurysms.
METHODS: A total of 62 patients (mean age, 68.9 ± 8.8 years; 91.9% male) undergoing open surgery for inflammatory aortic aneurysm from 1995 until 2014 in a high-volume vascular center were retrospectively evaluated. The patients' demographics, preoperative and postoperative clinical characteristics, imaging measurements, and procedural data were collected. Study end points were preoperative and postoperative sac diameter, evolution of periaortic fibrosis and development of hydroureteronephrosis detected by computed tomography (CT) scan, and mortality and morbidity after 30 days and at the time of maximum follow-up.
RESULTS: The mean abdominal aortic aneurysm diameter was 67.3 ± 16.7 mm. A total of 30 patients (48.4%) were asymptomatic, 27 patients (43.5%) were symptomatic, and 5 patients (8.1%) were treated for ruptured aneurysm. In 25 patients (40.3%), an aorta-aortic tube graft was implanted; in 37 patients (59.7%), an aortic bifurcation graft was used. Median operating time was 208 minutes (range, 83-519 minutes). Median aortic clamping time was 31 minutes (range, 14-90 minutes); in 25 patients (40.3%), suprarenal aortic cross-clamping was necessary. Hydroureteronephrosis was preoperatively diagnosed by CT scan in 16 patients (25.8%), with the need for a ureteral stent in 11 patients (17.7%). Aneurysm- and procedure-associated 30-day mortality was 11.3% (n = 7), with septic multiple organ failure in four patients and cardiac arrest in three patients. The overall perioperative complication rate was 33.9% (n = 21 patients). Median follow-up was 71.0 months (range, 0.2-231.6 months). At 1 year, 2 years, 4 years, and 6 years, overall survival was 83.4%, 79.6%, 79.6%, and 72.6%, respectively. Six patients (9.7%) required a reintervention during follow-up, predominantly aneurysm related and caused by aortoenteric fistula and graft infection (three of five patients). Median maximum thickness of preoperative perianeurysmal inflammation on CT was 10 mm (range, 2-22 mm), which decreased in 15 of 16 (94%) patients with available postoperative CT scans. Postoperative median thickness of perianeurysmal inflammation on CT was 6 mm (range, 0-13 mm). Hydroureteronephrosis persisted in two of nine (22.2%) patients at the end of follow-up.
CONCLUSIONS: Surgery in patients with inflammatory abdominal aortic aneurysms is associated with a substantial amount of perioperative complications. After surgery, the perianeurysmal inflammation decreases in most patients on follow-up CT. However, because the inflammatory process does not totally resolve, patients require lifelong surveillance for hydroureteronephrosis and development of aortoenteric fistulas.

PMID: 30503911 [PubMed - indexed for MEDLINE]

Predictors of positive 18F-FDG PET/CT-scan for large vessel vasculitis in patients with persistent polymyalgia rheumatica.

jeu, 25/04/2019 - 03:15
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Predictors of positive 18F-FDG PET/CT-scan for large vessel vasculitis in patients with persistent polymyalgia rheumatica.

Semin Arthritis Rheum. 2019 Feb;48(4):720-727

Authors: Prieto-Peña D, Martínez-Rodríguez I, Loricera J, Banzo I, Calderón-Goercke M, Calvo-Río V, González-Vela C, Corrales A, Castañeda S, Blanco R, Hernández JL, González-Gay MÁ

Abstract
OBJECTIVE: Polymyalgia rheumatica (PMR) is often the presenting manifestation of giant cell arteritis (GCA). Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) scan often discloses the presence of large vessel vasculitis (LVV) in PMR patients. We aimed to identify predictive factors of a positive PET/CT scan for LVV in patients classified as having isolated PMR according to well-established criteria.
METHODS: A set of consecutive patients with PMR from a single hospital were assessed. All of them underwent PET/CT scan between January 2010 and February 2018 based on clinical considerations. Patients with PMR associated to other diseases, including those with cranial features of GCA, were excluded. The remaining patients were categorized in classic PMR (if fulfilled the 2012 EULAR/ACR classification criteria at disease diagnosis; n = 84) or atypical PMR (who did not fulfill these criteria; n = 16). Only information on patients with classic PMR was assessed.
RESULTS: The mean age of the 84 patients (51 women) with classic PMR was 71.4 ± 9.2 years. A PET/CT scan was positive in 51 (60.7%). Persistence of classic PMR symptoms was the most common reason to perform a PET/CT scan. Nevertheless, patients with positive PET/CT scan often had unusual symptoms. The best set of predictors of a positive PET/CT scan were bilateral diffuse lower limb pain (OR = 8.8, 95% CI: 1.7-46.3; p = 0.01), pelvic girdle pain (OR = 4.9, 95% CI: 1.50-16.53; p = 0.01) and inflammatory low back pain (OR = 4.7, 95% CI: 1.03-21.5; p = 0.04).
CONCLUSION: Inflammatory low back pain, pelvic girdle and diffuse lower limb pain are predictors of positive PET/CT scan for LVV in PMR.

PMID: 29903537 [PubMed - indexed for MEDLINE]

Frequency, treatment, evolution, and factors associated with the presence of tracheobronchial stenoses in granulomatosis with polyangiitis. Retrospective analysis of a case series from a single respiratory referral center.

jeu, 25/04/2019 - 03:15
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Frequency, treatment, evolution, and factors associated with the presence of tracheobronchial stenoses in granulomatosis with polyangiitis. Retrospective analysis of a case series from a single respiratory referral center.

Semin Arthritis Rheum. 2019 Feb;48(4):714-719

Authors: Marroquín-Fabián E, Ruiz N, Mena-Zúñiga J, Flores-Suárez LF

Abstract
OBJECTIVES: Granulomatosis with polyangiitis-a primary systemic vasculitis-most frequent manifestations are respiratory. Airway disease can present with stenosis, and although subglottic stenosis (SGS) is well described, narrowing distal to the glottis has been more recently the focus of reporting. Our objectives, therefore, are to describe the frequency, characteristics, and treatment of tracheobronchial stenoses (TBS) in granulomatosis with polyangiitis (GPA) at our institution, a national referral center for respiratory diseases. Also, to identify factors associated with TBS development in GPA.
METHODS: We undertook a retrospective study of all identified TBS cases (n-29) in whom their demographics, clinical and paraclinical features, and treatment were analyzed with descriptive statistics. Comparisons between those who developed and did not develop TBS (n-79) were established with the χ2 test with Yates correction or two-tailed exact Fisher test for comparisons among groups with or without this complication.
RESULTS: Females were predominantly affected by TBS (76%). Most patients had limited GPA (n-20, 69%). TBS appeared at a mean of 29 months after diagnosis of GPA. Main symptoms were dysphonia, stridor, and dyspnea. All TBS patients had tracheal involvement and 12 (41%) additional bronchial stenoses. Other accompanying manifestations by organ/system were rhinosinusal (n-26, 87%), musculoskeletal (n-16, 55%), ocular (n-13, 45%), pulmonary (n-12, 41%), renal (n-8, 27%), mucocutaneous (n-5, 17%), neurological (n-4, 13%). At TBS diagnosis, 17 patients were PR3-ANCA and/or C-ANCA positive, while 9 were MPO-ANCA and/or P-ANCA positive; results on the remaining were either negative or ANCA were not performed at that time. Seventeen patients had simultaneous medical (8 with glucocorticoid therapy, 9 with immunosuppressants) and surgical therapy, while the rest only the latter at the time of TBS diagnosis. Relapses were frequent (one in 18 patients; two in 11, three in 9 and 2 individuals had ≥4 relapses). Factors positively associated with TBS were the presence of general and musculoskeletal symptoms and rhinosinusal disease, while those negatively associated were prednisone oral daily dose >10mgqd and azathioprine intake. The extent of disease, either generalized or limited was not associated with TBS development, nor were ANCA levels.
CONCLUSIONS: TBS are serious complications of GPA which can arise at any stage of the disease. Timely diagnosis plus optimal treatment and follow-up remain unmet needs.

PMID: 29891418 [PubMed - indexed for MEDLINE]

Myeloperoxidase-ANCA-positive granulomatosis with polyangiitis is a distinct subset of ANCA-associated vasculitis: A retrospective analysis of 455 patients from a single center in China.

jeu, 25/04/2019 - 03:15
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Myeloperoxidase-ANCA-positive granulomatosis with polyangiitis is a distinct subset of ANCA-associated vasculitis: A retrospective analysis of 455 patients from a single center in China.

Semin Arthritis Rheum. 2019 02;48(4):701-706

Authors: Chang DY, Li ZY, Chen M, Zhao MH

Abstract
OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA) directed to proteinase 3 (PR3) used to be considered the serologic marker for granulomatosis with polyangiitis (GPA). However, patients with myeloperoxidase (MPO)-ANCA positive GPA have been increasingly reported. The aim of this study was to analyze the clinical and pathological characteristics and outcome of Chinese patients with MPO-ANCA positive GPA.
METHODS: The clinical and renal histology data, renal outcomes, response to treatment, relapse and mortality were compared between patients with MPO-ANCA positive GPA and MPO-ANCA positive microscopic polyangiitis (MPA) as well as proteinase 3 (PR3)-ANCA positive GPA.
RESULTS: 455 patients with ANCA-associated vasculitis (AAV) were recruited in this study. 276/455 patients were classified as MPO-ANCA positive MPA, 4/455 patients were classified as PR3-ANCA positive MPA, 124/455 were MPO-ANCA positive GPA and 51/455 were PR3-ANCA positive GPA. Compared with MPO-ANCA positive MPA patients, MPO-ANCA positive GPA patients had significantly higher level of BVAS and milder renal lesion at diagnosis. The probability of developing ESRD was significantly higher in patients with MPO-ANCA positive MPA than MPO-ANCA positive GPA. MPO-ANCA positive GPA patients were likely to have relapse than MPO-ANCA positive MPA patients. Compared with PR3-ANCA positive GPA patients, MPO-ANCA positive GPA patients had significantly higher proportion of female, less constitutional symptoms and milder renal lesion at diagnosis.
CONCLUSIONS: Patients with MPO-ANCA positive GPA should be regarded as a unique subset of AAV. This subset of AAV patients had relatively milder renal injury. Although ANCA specificities play an important role in differentiating AAV, taking the disease type together to classify AAV may be more rational.

PMID: 29887327 [PubMed - indexed for MEDLINE]

Arterial lesions in giant cell arteritis: A longitudinal study.

jeu, 25/04/2019 - 03:15
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Arterial lesions in giant cell arteritis: A longitudinal study.

Semin Arthritis Rheum. 2019 02;48(4):707-713

Authors: Kermani TA, Diab S, Sreih AG, Cuthbertson D, Borchin R, Carette S, Forbess L, Koening CL, McAlear CA, Monach PA, Moreland L, Pagnoux C, Seo P, Spiera RF, Warrington KJ, Ytterberg SR, Langford CA, Merkel PA, Khalidi NA, Vasculitis Clinical Research Consortium

Abstract
OBJECTIVES: To evaluate large-vessel (LV) abnormalities on serial imaging in patients with giant cell arteritis (GCA) and discern predictors of new lesions.
METHODS: Clinical and imaging data from patients with GCA (including subjects diagnosed by LV imaging) enrolled in a prospective, multicenter, longitudinal study and/or a randomized clinical trial were included. New arterial lesions were defined as a lesion in a previously unaffected artery.
RESULTS: The study included 187 patients with GCA, 146 (78%) female, mean (±SD) age at diagnosis 68.5 ± 8.5 years; 39% diagnosed by LV imaging. At least one arterial lesion was present in 123 (66%) on the first study. The most frequently affected arteries were subclavian (42%), axillary (32%), and thoracic aorta (20%). In 106 patients (57%) with serial imaging, new arterial lesions were noted in 41 patients (39%), all of whom had a baseline abnormality, over a mean (±SD) follow-up of 4.39 (2.22) years. New abnormalities were observed in 33% patients by year 2; clinical features of active disease were present at only 50% of these cases. There were no differences in age, sex, temporal artery biopsy positivity, or disease activity in patients with or without new lesions.
CONCLUSIONS: In this cohort of patients with GCA, LV abnormalities on first imaging were common. Development of new arterial lesions occurred in patients with arterial abnormalities at first imaging, often in the absence of symptoms of active disease. Arterial imaging should be considered in all patients with GCA at diagnosis and serial imaging at least in patients with baseline abnormalities.

PMID: 29880442 [PubMed - indexed for MEDLINE]

Necrotizing Soft Tissue Infection Complicating Levamisole-Induced Vasculitis.

ven, 19/04/2019 - 02:50
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Necrotizing Soft Tissue Infection Complicating Levamisole-Induced Vasculitis.

Am Surg. 2019 Mar 01;85(3):e145-e147

Authors: Whitaker L, Priest O, Pestana IA

PMID: 30947791 [PubMed - indexed for MEDLINE]

Recurrent superficial venous thrombophlebitis because of mutations in the protein C and fibrinogen genes in a young Argentinian female.

mar, 16/04/2019 - 02:39
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Recurrent superficial venous thrombophlebitis because of mutations in the protein C and fibrinogen genes in a young Argentinian female.

Blood Coagul Fibrinolysis. 2019 Mar;30(2):80-84

Authors: Guglielmone HA, Bastos L, Jarchum GD, Alvarez-Bollea MA

Abstract
: Hypodysfibrinogenemia and protein C deficiency are coagulopathies and in this report, we describe a young patient with both defects confirmed by molecular genetic tests. The patient was a 24-year-old woman referred for recurrent thrombophlebitis and finally deep venous thrombosis. Routine coagulation studies revealed mild decrease of protein C (0.49 IU, reference values 0.7-1.40 IU) and hypodysfibrinogenemia (0.88 g/l and 1.83 g/l for activity and antigen, respectively, reference values 2.0-4.0 g/l). Direct sequencing analyses were performed on FGA, FGB, and FGG genes to confirm hypodysfibrinogenemia and on the protein C gene to confirm protein C deficiency. As a result, the patient was shown to be heterozygous p.Ala82Gly in the FGG gene (Fibrinogen Dunedin) and for compound heterozygous missense mutation in protein C gene. To our knowledge, this is the first report on a case of combined dysfibrinogenemia and protein C deficiency confirmed by molecular genetic tests.

PMID: 30632992 [PubMed - indexed for MEDLINE]

Clinicopathological case: rapid cognitive decline and myoclonus associated with fever, arthropathy and scleritis.

mar, 16/04/2019 - 02:39
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Clinicopathological case: rapid cognitive decline and myoclonus associated with fever, arthropathy and scleritis.

Pract Neurol. 2019 Apr;19(2):147-155

Authors: Ellis RJB, Doran M, Jacob A, Ismail A, Davenport R

Abstract
A 65-year-old man presented with transient neurological symptoms, followed by rapid cognitive decline, myoclonus and fevers. He had evidence of scleritis and an arthropathy. This paper reports the clinicopathological conference discussed at the Association of British Neurologists Annual Meeting 2017.

PMID: 30425126 [PubMed - indexed for MEDLINE]

The C-Reactive Protein/Albumin Ratio and Complete Blood Count Parameters as Indicators of Disease Activity in Patients with Takayasu Arteritis.

sam, 13/04/2019 - 00:52
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The C-Reactive Protein/Albumin Ratio and Complete Blood Count Parameters as Indicators of Disease Activity in Patients with Takayasu Arteritis.

Med Sci Monit. 2019 Feb 22;25:1401-1409

Authors: Seringec Akkececi N, Yildirim Cetin G, Gogebakan H, Acipayam C

Abstract
BACKGROUND This study aimed to evaluate the ratio of C-reactive protein (CRP) to albumin, inflammatory markers, and parameters from the complete blood count (CBC) in patients with Takayasu arteritis and the association with disease activity. MATERIAL AND METHODS A retrospective study included thirty-two patients with Takayasu arteritis and 32 healthy controls. Clinical and demographic characteristics of patients with Takayasu arteritis were recorded at baseline, before medication and on remission. Similar data were obtained for the controls at recruitment. Remission was defined as more than six months of stable disease without new vascular lesions in patients who previously had active disease. Kerr's criteria were used to define active Takayasu arteritis. RESULTS In patients with Takayasu arteritis, the erythrocyte sedimentation rate (ESR), CRP, CRP/albumin ratio, red cell distribution width (RDW), neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and monocyte-lymphocyte ratio (MLR) were significantly higher, and albumin and MPV were significantly lower compared with controls. The ESR, CRP, CRP/albumin ratio, NLR, PLR, and MLR were decreased in remission, whereas MPV was increased. CRP and the CRP/albumin ratio were positively correlated and albumin and MPV were negatively correlated with disease activity. The CRP/albumin ratio had the highest correlation with disease activity in Takayasu arteritis. The CRP/albumin ratio, RDW, NLR, PLR, and MLR were positively correlated with CRP and ESR. CONCLUSIONS The CRP/albumin ratio, RDW, NLR, PLR, MLR, and MPV were markers of remission of active disease, and the CRP/albumin ratio, total albumin, and MPV were markers of disease activity in Takayasu arteritis.

PMID: 30792377 [PubMed - indexed for MEDLINE]

Acute Pulmonary Hypertension Crisis after Adalimumab Reduction in Rheumatoid Vasculitis.

sam, 13/04/2019 - 00:52
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Acute Pulmonary Hypertension Crisis after Adalimumab Reduction in Rheumatoid Vasculitis.

Intern Med. 2019;58(4):593-601

Authors: Yamasaki G, Okano M, Nakayama K, Jimbo N, Sendo S, Tamada N, Misaki K, Shinkura Y, Yanaka K, Tanaka H, Akashi K, Morinobu A, Yokozaki H, Emoto N, Hirata KI

Abstract
Rheumatoid vasculitis is a rare etiology for pulmonary hypertension (PH) in patients with connective tissue disease. We encountered a case of acute PH crisis in a case with rheumatoid vasculitis eight months after undergoing adalimumab reduction. Since no repetition of arthralgia occurred after the adalimumab reduction, we decided to not increase the dose of adalimumab. However, hemodynamic collapse thereafter developed and even though steroid pulse therapy was administered, the patient nevertheless died. The autopsy showed clusters of acute and chronic inflammation around the remodeled pulmonary arteries along with micro-thrombi in the vessel lumen. We should consider the possibility of critical worsening of PH as a phenotype of vasculitis related to immunosuppressive therapy reduction.

PMID: 30773522 [PubMed - indexed for MEDLINE]

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