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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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In vasculitis of small muscular arteries, activation of vessel-infiltrating CD8 T cells seems to be antigen-independent.

sam, 07/04/2018 - 02:23
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In vasculitis of small muscular arteries, activation of vessel-infiltrating CD8 T cells seems to be antigen-independent.

Virchows Arch. 2018 Feb;472(2):271-279

Authors: Kobayashi M, Ogawa E, Okuyama R, Kanno H

Abstract
The etiology of polyarteritis nodosa (PAN) and localized PAN is still unknown, although a T cell-mediated immune mechanism has been considered. CD8 T cells participate not only in the antigen-dependent adaptive immune system, but also in the antigen-independent innate immune system. Non-antigen-activated CD8 T cells express a unique phenotype: granzyme B (GrB) positive /CD25 negative /programmed death-1 (PD-1) negative. The aims of this study were to assess the participation of T cells, especially innate CD8 T cells, in the development of vasculitis. Twenty-eight consecutive cases of skin biopsy specimens with cutaneous vasculitis of small muscular arteries (CVSMA) were retrieved. The series comprises of 21 cases of cutaneous arteritis, three cases of PAN, and four cases of rheumatoid vasculitis. Cases of antineutrophil cytoplasmic antibody-associated vasculitis were excluded. The phenotypes of infiltrating lymphocytes in vasculitis lesions were evaluated by immunohistochemistry. In most cases of CVSMA, the number of CD8 T cells infiltrating the intima was higher than that of CD4 T cells, and significant numbers of GrB-positive cells, which represent activated CD8 T cells, were observed. However, GrB/CD25-double-positive cells, which correspond to antigen-activated T cells, were very few in a small number of cases. Cells positive for PD-1, which is also expressed on antigen-activated CD8 T cells, were not detected. We conclude that a T cell-mediated immune mechanism, involving cytotoxic CD8 T cells, may play a role in the development of CVSMA. Low expression of CD25 in activated CD8 T cells suggests that activation was antigen-independent.

PMID: 29128969 [PubMed - indexed for MEDLINE]

Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature.

mer, 04/04/2018 - 01:31
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Focus on the Involvement of the Nose and Paranasal Sinuses in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome): Nasal Cytology Reveals Infiltration of Eosinophils as a Very Common Feature.

Int Arch Allergy Immunol. 2018;175(1-2):61-69

Authors: Seccia V, Baldini C, Latorre M, Gelardi M, Dallan I, Cristofani-Mencacci L, Sellari-Franceschini S, Bartoli ML, Bacci E, Paggiaro P

Abstract
BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis that predominantly affects small- to medium-sized vessels. It is characterized by a wide spectrum of extrapulmonary symptoms, including sinonasal and paranasal sinus abnormalities. These are the most common features of this disease, constituting diagnostic criteria for EGPA. However, the actual clinical features, cellular mechanisms and impact on patients' quality of life (QoL) are still a matter of study.
METHODS: Thirty-nine EGPA patients underwent multidimensional rhinological evaluations, including rhinofibroscopy, nasal cytology, and QoL questionnaires. This was coupled with respiratory and rheumatological assessments.
RESULTS: Twenty-eight patients were diagnosed with chronic rhinosinusitis (CRS). Of these, 18 had nasal polyposis (NP). Chronic rhinitis was diagnosed in 10 patients. Of these, 3 had allergic rhinitis (AR) and seven had non-AR (NAR). Overall, only 1 patient (2.6%) was normal. Nasal cytology showed that hypereosinophilia was present in 17/28 patients with CRS, 4/7 patients with NAR and all patients with AR. SNOT-22 and SF-36 showed a severe impact of nasal symptoms on QoL. No differences in asthma control or rheumatological patterns for EGPA were observed among patients with or without NP.
CONCLUSIONS: Even when the rheumatological assessment scored EGPA "under control" according to the Birmingham Vasculitis Activity Score and Vasculitis Damage Index, sinonasal diseases and related nasal inflammatory processes were not controlled. Therefore, there is a need for clinical monitoring and targeted treatment to control the inflammatory processes and improve the QoL of EGPA patients.

PMID: 29393242 [PubMed - indexed for MEDLINE]

Association of killer cell immunoglobulin-like receptor (KIR) genes and their HLA ligands with susceptibility to Behçet's‎ disease.

mer, 04/04/2018 - 01:31
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Association of killer cell immunoglobulin-like receptor (KIR) genes and their HLA ligands with susceptibility to Behçet's‎ disease.

Scand J Rheumatol. 2018 Mar;47(2):155-163

Authors: Mohammad-Ebrahim H, Kamali-Sarvestani E, Mahmoudi M, Beigy M, Karami J, Ahmadzadeh N, Shahram F

Abstract
OBJECTIVES: Behçet's disease (BD) is a systemic inflammatory disorder with remissions and exacerbations. It is thought that defects in the natural killer (NK) cell repertoire may be involved in BD through killer cell immunoglobulin-like receptors (KIRs). This study aimed to evaluate KIR and HLA genes, their interactions in BD patients, and their associations with clinical manifestations.
METHOD: The presence or absence of KIR and HLA alleles and genotypes was analysed by polymerase chain reaction sequence-specific primer on genomic DNA of 397 BD patients and 300 healthy controls.
RESULTS: None of the KIR genes showed significant effects on BD susceptibility. HLA-C1Asn80 showed a protective effect against BD, whereas HLA-C2Lys80, HLA-B-Bw4Ile80, HLA-B5, and HLA-B51 were associated with a susceptibility risk for BD. In the combination of KIR and HLA genes, the frequencies of HLA genotypes no. 2, 3, 5, and 8, and inhibitory KIR no. 4 were significantly higher in patients than in controls. The frequencies of KIR genotype no. 3 and HLA genotypes no. 1, 4, 6, 7, and 9 were significantly lower in patients than in controls. There were many associations between KIR and HLA genes with clinical features of BD.
CONCLUSION: Differences in the frequency of HLA genes, KIR-HLA interactions, and genotypes between BD and healthy controls and their associations with clinical manifestations indicate that NK cells are involved in BD pathogenesis. The observed differences indicated an NK cell activity imbalance in BD patients, and suggest a role of the KIR-HLA repertoire in the development of BD.

PMID: 28862099 [PubMed - indexed for MEDLINE]

Supraglottic stenosis in a case of Behcet's disease.

mer, 04/04/2018 - 01:31
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Supraglottic stenosis in a case of Behcet's disease.

Scand J Rheumatol. 2018 Mar;47(2):164-165

Authors: Hajialilo M, Nasemieh M, Khabbazi A

PMID: 28726536 [PubMed - indexed for MEDLINE]

Interferon alpha-2a treatment for refractory Behcet uveitis in Korean patients.

jeu, 22/03/2018 - 16:45
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Interferon alpha-2a treatment for refractory Behcet uveitis in Korean patients.

BMC Ophthalmol. 2018 Feb 20;18(1):52

Authors: Lee JH, Lee CS, Lee SC

Abstract
BACKGROUND: To evaluate therapeutic outcomes of interferon alpha-2a (IFNα2a) treatment in patients with Behcet's disease who were refractory to immunosuppressive agents.
METHODS: This retrospective case series reviewed the medical records of 5 patients with refractory Behcet uveitis from January 2011 to February 2017. IFNα2a was administered at a dose of 3 million IU 3 times per week. Clinical response, relapse rate, and change of visual acuity were evaluated.
RESULTS: The mean age of patients was 39.60 ± 9.21 years, and the median treatment duration was 6 months. Four of the 5 patients (80%) presented with responses to IFNα2a without any uveitis attack during the treatment period. The mean number of uveitis attacks/year per patient during the treatment was 0.40 ± 0.89. The mean log of the Minimum Angle of Resolution visual acuity improved from 1.44 ± 0.38 at baseline to 1.02 ± 0.58 at the final follow up.
CONCLUSIONS: IFNα2a is an effective therapy for Behcet uveitis refractory to conventional immunosuppressants in Korean patients.

PMID: 29463220 [PubMed - indexed for MEDLINE]

Interstitial Keratitis in Cogan's Syndrome.

ven, 16/03/2018 - 12:13
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Interstitial Keratitis in Cogan's Syndrome.

N Engl J Med. 2018 Mar 01;378(9):852

Authors: Rodrigues-Barros S, Parreira S

PMID: 29490187 [PubMed - indexed for MEDLINE]

Polyps, grommets and eosinophilic granulomatosis with polyangiitis.

ven, 16/03/2018 - 12:13
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Polyps, grommets and eosinophilic granulomatosis with polyangiitis.

J Laryngol Otol. 2018 Mar;132(3):236-239

Authors: Kavanagh FG, Hasan W, Smyth DA, Fenton JE

Abstract
OBJECTIVE: To explore the link between nasal polyposis, refractory otitis media with effusion and eosinophilic granulomatosis with polyangiitis.
METHODS: A retrospective observational study was carried out of patients diagnosed with refractory otitis media with effusion necessitating grommet insertion and who had nasal polyps. Patients were evaluated to determine if they fulfilled the diagnostic criteria of eosinophilic granulomatosis with polyangiitis.
RESULTS: Sixteen patients (10 males and 6 females) were identified. The mean age of grommet insertion was 45.4 years. The mean number of grommets inserted per patient was 1.6. The mean number of nasal polypectomies was 1.7. All 16 patients had paranasal sinus abnormalities and otitis media with effusion, 14 had asthma, 9 had serological eosinophilia and 7 had extravascular eosinophilia. Nine patients met the diagnostic criteria for eosinophilic granulomatosis with polyangiitis.
CONCLUSION: The co-presence of nasal polyps and resistant otitis media with effusion should raise the possibility of eosinophilic granulomatosis with polyangiitis.

PMID: 29310745 [PubMed - indexed for MEDLINE]

Intravenous Thrombolysis for Stroke and Presumed Stroke in Human Immunodeficiency Virus-Infected Adults: A Retrospective, Multicenter US Study.

ven, 16/03/2018 - 12:13
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Intravenous Thrombolysis for Stroke and Presumed Stroke in Human Immunodeficiency Virus-Infected Adults: A Retrospective, Multicenter US Study.

Stroke. 2018 01;49(1):228-231

Authors: AbdelRazek MA, Gutierrez J, Mampre D, Cervantes-Arslanian A, Ormseth C, Haussen D, Thakur KT, Lyons JL, Smith BR, O'Connor O, Willey JZ, Mateen FJ

Abstract
BACKGROUND AND PURPOSE: Human immunodeficiency virus (HIV) infection has been shown to increase both ischemic and hemorrhagic stroke risks, but there are limited data on the safety and outcomes of intravenous thrombolysis with tPA (tissue-type plasminogen activator) for acute ischemic stroke in HIV-infected patients.
METHODS: A retrospective chart review of intravenous tPA-treated HIV patients who presented with acute stroke symptoms was performed in 7 large inner-city US academic centers (various search years between 2000 and 2017). We collected data on HIV, National Institutes of Health Stroke Scale score, ischemic stroke risk factors, opportunistic infections, intravenous drug abuse, neuroimaging findings, and modified Rankin Scale score at last follow-up.
RESULTS: We identified 33 HIV-infected patients treated with intravenous tPA (mean age, 51 years; 24 men), 10 of whom were stroke mimics. Sixteen of 33 (48%) patients had an HIV viral load less than the limit of detection while 10 of 33 (30%) had a CD4 count <200/mm3. The median National Institutes of Health Stroke Scale score at presentation was 9, and mean time from symptom onset to tPA was 144 minutes (median, 159). The median modified Rankin Scale score for the 33-patient cohort was 1 and for the 23-patient actual stroke cohort was 2, measured at a median of 90 days poststroke symptom onset. Two patients had nonfatal hemorrhagic transformation (6%; 95% confidence interval, 1%-20%), both in the actual stroke group. Two patients had varicella zoster virus vasculitis of the central nervous system, 1 had meningovascular syphilis, and 7 other patients were actively using intravenous drugs (3 cocaine, 1 heroin, and 3 unspecified), none of whom had hemorrhagic transformation.
CONCLUSIONS: Most HIV-infected patients treated with intravenous tPA for presumed and actual acute ischemic stroke had no complications, and we observed no fatalities. Stroke mimics were common, and thrombolysis seems safe in this group. We found no data to suggest an increased risk of intravenous tPA-related complications because of concomitant opportunistic infections or intravenous drug abuse.

PMID: 29273597 [PubMed - indexed for MEDLINE]

Innate immune responses following Kawasaki disease and toxic shock syndrome.

mar, 13/03/2018 - 10:18
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Innate immune responses following Kawasaki disease and toxic shock syndrome.

PLoS One. 2018;13(2):e0191830

Authors: Chen KYH, Messina N, Germano S, Bonnici R, Freyne B, Cheung M, Goldsmith G, Kollmann TR, Levin M, Burgner D, Curtis N

Abstract
The pathogenesis of Kawasaki disease (KD) remains unknown and there is accumulating evidence for the importance of the innate immune system in initiating and mediating the host inflammatory response. We compared innate immune responses in KD and toxic shock syndrome (TSS) participants more than two years after their acute illness with control participants to investigate differences in their immune phenotype. Toxic shock syndrome shares many clinical features with KD; by including both disease groups we endeavoured to explore changes in innate immune responses following acute inflammatory illnesses more broadly. We measured the in vitro production of interferon (IFN)-γ, tumour necrosis factor (TNF)-α, interleukin (IL)-1β, IL-6, IL-1 receptor antagonist (IL-1ra), and IL-10 following whole blood stimulation with toll-like receptor and inflammasome ligands in 52 KD, 20 TSS, and 53 control participants in a case-control study. Analyses were adjusted for age, sex, and unstimulated cytokine concentrations. Compared to controls, KD participants have reduced IL-1ra production in response to stimulation with double stranded RNA (geometric mean ratio (GMR) 0.37, 95% CI 0.15, 0.89, p = 0.03) and increased IL-6 production in response to incubation with Lyovec™ (GMR 5.48, 95% CI 1.77, 16.98, p = 0.004). Compared to controls, TSS participants have increased IFN-γ production in response to peptidoglycan (GMR 4.07, 95% CI 1.82, 9.11, p = 0.001), increased IL-1β production to lipopolysaccharide (GMR 1.64, 95% CI 1.13, 2.38, p = 0.01) and peptidoglycan (GMR 1.61, 95% CI 1.11, 2.33, p = 0.01), and increased IL-6 production to peptidoglycan (GMR 1.45, 95% CI 1.10, 1.92, p = 0.01). Years following the acute illness, individuals with previous KD or TSS exhibit a pro-inflammatory innate immune phenotype suggesting a possible underlying immunological susceptibility or innate immune memory.

PMID: 29447181 [PubMed - indexed for MEDLINE]

The clinical characteristics and the features of immunophenotype of peripheral lymphocytes of adult onset chronic active Epstein-Barr virus disease at a Tertiary Care Hospital in Beijing.

sam, 10/03/2018 - 08:07
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The clinical characteristics and the features of immunophenotype of peripheral lymphocytes of adult onset chronic active Epstein-Barr virus disease at a Tertiary Care Hospital in Beijing.

Medicine (Baltimore). 2018 Mar;97(9):e9854

Authors: Luo L, Wang H, Fan H, Xie J, Qiu Z, Li T

Abstract
Chronic active Epstein-Barr virus (CAEBV) infection is a rare disease with high mortality. Most of CAEBV patients have been reported from Japan and are pediatric cases.The goal was to describe the clinical characteristics and the immunophenotypic features of peripheral lymphocytes in adult onset CAEBV patients.We retrospectively reviewed and analyzed all adult onset CAEBV cases admitted to Peking Union Medical College Hospital (PUMCH) between 2012 and 2016. Demographic, clinical, laboratory data, and the immunophentyping data of peripheral lymphocytes were collected.There were 28 adult onset CAEBV patients. The median age was 45 (range, 20-81). Most of the patients presented with fever; splenomegaly; lymphadenopathy and hepatitis. Unlike pediatric cases reported, the manifestations of cardiovascular diseases in our patients were pulmonary arterial hypertension, decreased cardiac function and aorta vasculitis. Prevalence of interstitial pneumonitis in our patients were comparatively higher and prevalence of hypersensitivity to mosquito bites were comparatively lower than that reported by Japan. In this study, CAEBV patients had decreased B cell, NK cell, CD4 cell and CD8 cell counts. The prevalence of low level of B cells, NK cells, CD4 cells was relatively higher than reported ever.Chinese adult onset CAEBV patients have different clinical characteristics and are featured by an immunosuppression status as demonstrated by decreased B cell, NK cell, CD4 cell and CD8 cell.

PMID: 29489682 [PubMed - indexed for MEDLINE]

Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis.

dim, 04/03/2018 - 06:00
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Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis.

Am J Med Sci. 2018 Feb;355(2):195-200

Authors: Salim SA, Yousuf T, Patel A, Fülöp T, Agarwal M

Abstract
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation. She was treated with 6 months of intravenous cyclophosphamide, followed by 2 doses of intravenous rituximab (1g each), thereafter maintained on mycophenolate mofetil and glucocorticoid-based therapy. She experienced a full recovery of renal function after 12 months of dialysis dependence. Hypocomplementemic urticarial vasculitis syndrome with crescentic glomerulonephritis is a rare disease with only 5 other reported cases in literature. In our case, we document a delayed but excellent renal recovery during a 2-year follow-up.

PMID: 29406049 [PubMed - indexed for MEDLINE]

Rhabdomyolysis-Associated Acute Kidney Injury With Normal Creatine Phosphokinase.

dim, 04/03/2018 - 06:00
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Rhabdomyolysis-Associated Acute Kidney Injury With Normal Creatine Phosphokinase.

Am J Med Sci. 2018 Jan;355(1):84-87

Authors: Kamal F, Snook L, Saikumar JH

Abstract
Rhabdomyolysis is a syndrome characterized by the breakdown of skeletal muscle and leakage of intracellular myocyte contents, such as creatine phosphokinase (CPK) and myoglobin, into the interstitial space and plasma resulting in acute kidney injury (AKI). Elevated CPK of at least 5 times the upper limit of normal is an important diagnostic marker of Rhabdomyolysis. We present a case of rhabdomyolysis with severe AKI with a normal CPK at presentation. A 32-year-old man presented with acute respiratory failure and AKI after an overdose of recreational drugs. Urinalysis at presentation showed trace amounts of blood, identified as rare red blood cells under microscopy. CPK was 156 U/L at presentation. Workup for glomerulonephritis and vasculitis was negative. He was initiated on renal replacement therapy, and a kidney biopsy showed severe acute tubular injury with positive myoglobin casts. Supportive management and renal replacement therapy was provided, and renal function spontaneously improved after a few weeks. This is an uncommon clinical presentation of severe rhabdomyolysis complicated by AKI. This suggests that CPK alone may not be a sensitive marker for rhabdomyolysis-induced AKI in some cases.

PMID: 29289268 [PubMed - indexed for MEDLINE]

Endovascular Treatment of Thromboangiitis Obliterans (Buerger's Disease).

dim, 04/03/2018 - 06:00
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Endovascular Treatment of Thromboangiitis Obliterans (Buerger's Disease).

Vasc Endovascular Surg. 2018 Feb;52(2):124-130

Authors: Modaghegh MS, Hafezi S

Abstract
PURPOSE: When critical limb ischemia (CLI) occurs in patients with thromboangiitis obliterans (TAO) or Buerger's disease, smoking cessation alone may be insufficient to relieve rest pain and promote wound healing. Accordingly, adjunctive measures are warranted to restore adequate blood flow required for limb salvage. This study aimed to evaluate the feasibility and efficacy of percutaneous transluminal angioplasty (PTA) for the treatment of patients with TAO and CLI. In addition, a review of the literature on endovascular management of TAO is included.
METHODS: Between April 2012 and June 2017, all patients with TAO and CLI who underwent PTA were studied retrospectively. Patient demographics, presentation, procedural details, and clinical response were recorded. Patients were monitored at 1 week, 1, 2, 3, and 6 months after revascularization and at least every 6 months thereafter.
RESULTS: Thirteen patients with TAO and CLI, who presented with rest pain only (n = 1), ischemic ulcer (n = 4), or gangrene (n = 8) underwent endovascular interventions with primary and assisted primary technical success of 85% and 92%, respectively. A below-knee amputation was eventually done in the only patient with technical failure (limb salvage rate: 92%). Following the procedures, 11 patients had clinical response, one of whom also received intra-arterial vasodilator to achieve complete symptom relief. The other patient who failed PTA underwent a successful lumbar sympathectomy. In addition, all ulcers healed and eight minor amputations were performed due to already established gangrene. During follow-up (mean: 19.4 months), four patients needed reintervention. Patients who continued to smoke experienced more severe ischemia ( P = .017) and were more likely to require reintervention ( P = .009).
CONCLUSION: Percutaneous transluminal angioplasty can be considered as a technically feasible and potentially effective treatment for patients with TAO and CLI, as well as a last resort for limb salvage when other options have failed. However, reintervention may be required, especially in patients who continue smoking.

PMID: 29237360 [PubMed - indexed for MEDLINE]

Extra-vascular findings in patients undergoing magnetic resonance angiography of the abdomen, pelvis and lower extremities: A retrospective study of 352 patients.

dim, 04/03/2018 - 06:00
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Extra-vascular findings in patients undergoing magnetic resonance angiography of the abdomen, pelvis and lower extremities: A retrospective study of 352 patients.

Vascular. 2018 Feb;26(1):27-38

Authors: Sohns JM, Menke J, Bergau L, Weiss BG, Schmuck S, Weiberg D, Staab W, Derlin T, Dorenkamp M, Sohns C

Abstract
Background The aim of this study was to assess the prevalence and clinical significance of extra-vascular findings in patients undergoing magnetic resonance angiography of the abdomen, pelvis and lower extremities. Materials and methods Three hundred fifty-two patients underwent abdominal, pelvic and lower extremity 1.5 T magnetic resonance angiography. Clinically relevant vascular and extra-vascular findings were identified. Relevant vascular findings were classified as stenosis, occlusion, aneurysm, sclerosis, dissection or vasculitis. Relevant extra-vascular findings were categorized as 'safe' (Group A), intermediate - requiring additional investigation - (Group B) and malignant/endangering - requiring change of therapy (Group C). Results A total of 2152 clinically relevant vascular findings was identified (6.1/patient). The most frequent vascular finding was femoral artery stenosis (10.6%). Four hundred fifty-one extra-vascular findings were observed (1.3/patient) and classified into Group A (78%), Group B (19.5%) and Group C findings (2.4%). The most frequent malignant findings were lung cancer, lymphoma, osteosarcoma, hepatocellular carcinoma and renal cell carcinoma (7/352 patients). Conclusions Extravascular findings are frequently encountered in magnetic resonance angiography performed for vascular indications. Clinically relevant findings are seen in a substantial part of patients and should prompt further diagnostic work-up.

PMID: 28587578 [PubMed - indexed for MEDLINE]

Ulcerative colitis followed by the development of typical intestinal Behçet disease: A case report.

lun, 26/02/2018 - 01:15
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Ulcerative colitis followed by the development of typical intestinal Behçet disease: A case report.

Medicine (Baltimore). 2018 Feb;97(7):e9882

Authors: Zhu Z, Shu X, Long S, Jiang X, Lu N, Zhu X, Liao W

Abstract
RATIONALE: Intestinal Behçet disease (intestinal BD) and inflammatory bowel disease (IBD) share a lot of characteristics, including genetic background, clinical manifestations, and therapeutic strategies, especially the extraintestinal manifestations, such as oral ulcers, arthralgia, eye lesions, skin lesions, etc, but the coexistence of these 2 diseases are uncommon. Behçet disease with gastrointestinal involvement in ulcerative colitis (UC) patient has been reported in just 1 previous case report, but, which can not be diagnosed as definite intestinal BD based on Korean novel diagnositic criteria due to lacking the typical ileocecal ulcer.
PATIENT CONCERNS: We present a 23-year-old woman with ulcerative disease who developed typical intestinal BD, which is the first case report of patient with coexisting UC and typical intestinal BD.
DIAGNOSES: This patient was diagnosed as coexistence of intestinal BD and UC base on the clinical manifestations, extra intestinal manifestations and typical colonoscopic findings.
INTERVENTIONS: Steroid and methotrexate were administered.
OUTCOMES: This patient achieved clinical remission and mucosal healing.
LESSONS: Coexistence of intestinal BD and UC is uncommon, and the combination with steroid, methotrexate, and 5-aminosalicylic acids is an effective therapy.

PMID: 29443756 [PubMed - indexed for MEDLINE]

Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis developed from ANCA negative renal limited vasculitis: A case report.

jeu, 22/02/2018 - 22:22
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Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis developed from ANCA negative renal limited vasculitis: A case report.

Medicine (Baltimore). 2017 Dec;96(51):e9128

Authors: Li XL, Xu PC, Chen T, Yan TK, Jiang JQ, Jia JY, Wei L, Shang WY, Hu SY

Abstract
RATIONALE: The relationship between antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and ANCA-negative vasculitis has not been elucidated.
PATIENT CONCERNS: A 64-year-old female with edema and proteinuria was admitted. A kidney biopsy indicated focal proliferative nephritis with crescents in 25% of glomeruli. Serum ANCA was negative. Eighteen months later, systemic symptoms emerged and acute kidney injury occurred. Serum ANCA against myeloperoxidase (MPO) turned positive. Repeated kidney biopsy showed more severe lesion than last time. Immunoglobulin (Ig)G was purified from serum obtained before the first kidney biopsy. Weak ANCA which could not be detected in serum was found in IgG.
DIAGNOSES: MPO-ANCA-associated AAV developed from ANCA-negative renal-limited AAV.
INTERVENTIONS: The patient was treated with glucocorticoid.
OUTCOMES: The serum creatinine decreased to 2.17 mg/dL a week later. MPO-ANCA turned negative when re-examined 3 weeks later. No relapse has been observed during follow-up for 6 months.
LESSONS: This is the first reported case about the spontaneous transformation from ANCA-negative renal-limited AAV to ANCA-positive systemic vasculitis. There might be a slow process of epitope spreading in the pathogenesis of disease. Physicians should try their best to detect the ANCA in the diagnose and treatment of ANCA-negative AAV.

PMID: 29390440 [PubMed - indexed for MEDLINE]

ANCA positive relapsing polychondritis, Graves disease, and suspected moyamoya disease: A case report.

ven, 16/02/2018 - 19:53
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ANCA positive relapsing polychondritis, Graves disease, and suspected moyamoya disease: A case report.

Medicine (Baltimore). 2017 Dec;96(51):e9378

Authors: Xuan YY, Li TF, Zhang L, Liu SY

Abstract
RATIOINALE: Relapsing polychondritis (RP) is a rare and heterogeneous disease complex of unknown origin which basically affects cartilaginous structures, 40% of which accompanied by rheumatic, hematologic, and endocrine disease. Among them, vasculitis is the most common accompanying type and usually presented with positive antineutrophilic cytoplasmic antibody (ANCA). The presence of ANCA could be primary or drug-induced like propylthiouracil (PTU). Central involvement of RP is very rare, and there is almost no report of cerebral vasculopathy manifested as moyamoya.
PATIENT CONCERNS: A 26-year-old woman complained about recurrent fever, auricular chondritis, ocular inflammation, and arthritis. She had an 8-year drug intake of PTU for Graves disease. Myeloperoxidase antineutrophilc cytoplasmic antibodies (MPO-ANCA) were found positive. Magnetic resonance angiography (MRA) detected multiple intracranial vasculopathy which we highly suspected it as moyamoya disease.
DIAGNOSES: Relapsing polychondritis, Graves disease and suspected moyamoya disease were clinically diagnosed.
INTERVENTIONS AND OUTCOMES: In case of possible PTU-induced vasculitis and the aggravation of vasculopathy, PTU was replaced by Iodine-131 (I) therapy. Induction treatment included oral prednisone 30 mg daily and oral cyclophosphamide 100 mg daily. Symptoms rapidly relieved before discharge. Inflammation markers were normal and MPO-ANCA decreased in 3 weeks after admission. Prednisone was gradually tapered to 7.5 mg daily and at month 10 azathioprine was continued for maintenance.
LESSONS: RP can overlap with Graves disease and moyamoya disease; comprehensive tests should be performed when admission. When relapsing polychondritis is accompanied with Graves disease, especially when ANCA is positive, PTU should be avoided.

PMID: 29390537 [PubMed - indexed for MEDLINE]

Evaluation of takayasu arteritis with delayed contrast-enhanced MR imaging by a free-breathing 3D IR turbo FLASH.

ven, 16/02/2018 - 19:53
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Evaluation of takayasu arteritis with delayed contrast-enhanced MR imaging by a free-breathing 3D IR turbo FLASH.

Medicine (Baltimore). 2017 Dec;96(51):e9284

Authors: Liu M, Liu W, Li H, Shu X, Tao X, Zhai Z

Abstract
The primary aim of our case-control study was to observe delayed contrast-enhanced magnetic resonance imaging (DCE-MRI) in patients with Takayasu arteritis (TA) in comparison with magnetic resonance angiography (MRA). Twenty-seven patients including 15 with active TA and 12 with stable TA who underwent both aortic MRA and DCE-MRI were included. A total of 27 sex- and age-matched healthy volunteers were enrolled as the control group. MRA were obtained with T1WI-volume-interpolated breath-hold examination sequence or fast low-angle shot (FLASH) sequence. DCE-MRI was acquired with a free-breathing three-dimensional inversion recovery Turbo fast low-angle shot (3D IR Turbo FLASH). Neither stenosis nor delayed enhancement of arterial wall was shown in the control group. In patients with stable TA, arterial stenosis was observed on MRA. On DCE-MR, delayed enhancement of arterial walls could be observed in the active TA group but not in the stable TA group or the control group. Stenotic arteries on MRA were comparable in the active TA and stable TA (χ = 2.70, P = .259); however, delayed enhancement of arterial walls in the active-TA group were more than those in the stable group (χ = 27.00, P < .001). Our results suggest that DCE-MRI with the free-breathing 3D IR Turbo FLASH sequence could assess TA and delayed enhancement on DCE-MRI is one characteristics of the active TA.

PMID: 29390488 [PubMed - indexed for MEDLINE]

Infliximab associated with life-threatening lung infection in a patient with Behcet disease with intestinal and hematopoietic system involvement: A case report.

ven, 16/02/2018 - 19:53
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Infliximab associated with life-threatening lung infection in a patient with Behcet disease with intestinal and hematopoietic system involvement: A case report.

Medicine (Baltimore). 2017 Dec;96(50):e9202

Authors: Chen Y, Shen Y, Ma HF, Cai JF, Hua YQ, Zou J, Guan JL

Abstract
RATIONALE: Tumor necrosis factor (TNF-α) participates in the pathophysiology of Behcet's disease (BD) and myelodysplastic syndrome (MDS). Infliximab is recommaned for the most severe type of BD, however, there is little evidence for its effectiveness in BD associated MDS.
PATIENT CONCERNS: A 46-year-old female, initially diagnosed with intestinal BD and leukopenia was later diagnosed as MDS. Treatement with infliximab and other immunoregulators lead to life-threatening pneumonia.
DIAGNOSIS: Intestinal BD associated with MDS involving trisomy 8.
INTERVENTIONS: The patient initially treated with methylprednisolone, thalidomide, cyclosporine A, and infliximab, which lead to severe lung infection. Therefore, the patient was transferred to Intensive Care Unit for life supportive, anti-infection and immune improving therapy.
OUTCOMES: The patient survived from the lung infection. With combination of methylprednisolone, thalidomide and cyclosporine A, the patient recovered from her intestinal ulceration and MDS manifestations.
LESSONS: Infliximab treatment may not benefit a patient with BD associated with MDS but place the patient at risk of infection.

PMID: 29390339 [PubMed - indexed for MEDLINE]

The prevalence of Behçet's disease in the north of Jordan: a hospital-based epidemiological survey.

ven, 16/02/2018 - 19:53
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The prevalence of Behçet's disease in the north of Jordan: a hospital-based epidemiological survey.

Clin Exp Rheumatol. 2017 Nov-Dec;35 Suppl 108(6):51-54

Authors: Madanat WY, Alawneh KM, Smadi MM, Saadeh SS, Omari MM, Bani Hani AB, Yazici H

Abstract
OBJECTIVES: To estimate the prevalence of Behçet's disease (BD) in Jordan, with the additional aim of comparing this prevalence among hospital workers in other geographical areas.
METHODS: In the first stage of our survey, 2,569 employees from 6 hospitals in north Jordan were interviewed using a screening questionnaire to identify individuals with recurrent oral ulcers (ROU), a previous diagnosis of BD (PDBD) and/or any major symptom related to BD. In the second stage, all individuals with ROU or PDBD identified at stage 1, were examined by 2 rheumatologists for the presence/confirmation of BD according to the International Study Group (ISG) criteria. Pathergy test was performed according to recommendations.
RESULTS: ROU were present in 210 (8.2%) individuals. BD was confirmed in 10 employees with PDBD. Seven more BD patients were found. Mean age of 17 BD patients was 38.6±10.7 (range 26-65 y). M: F was 2.4:1. Pathergy test was positive in 8/17. A family history of ROU or BD was noted in 9 (52%) and 3 (25.0%), respectively, compared to 227 (8.9%) and 62 (2.6%) in the whole group, excluding the BD patients (p<0.001 and 0.008, respectively). The prevalence rate of BD in the north of Jordan was estimated as 66:10.000 (95% CI 34.8 to 97.5:10000).
CONCLUSIONS: The results of this first ever survey indicated that the prevalence of BD in the north of Jordan is among the highest in the world. This prevalence can now be compared to hospital workers in other geographical areas.

PMID: 29224587 [PubMed - indexed for MEDLINE]

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