You are here

Vascularites

Subscribe to flux Vascularites Vascularites
NCBI: db=pubmed; Term=vasculitis AND ((English[lang] OR French[lang]) AND adult[MeSH] AND "last 90 days"[PDat])
Mis à jour : il y a 4 heures 10 min

Case Report: Masquerading Large-vessel Giant Cell Arteritis.

il y a 4 heures 10 min
Related Articles

Case Report: Masquerading Large-vessel Giant Cell Arteritis.

Optom Vis Sci. 2017 Dec;94(12):1159-1164

Authors: Jacquier P, Wuarin R, Chizzolini C, Thumann G, Steffen H, Chronopoulos A

Abstract
SIGNIFICANCE: Large-vessel giant cell arteritis (GCA) can be a diagnostic dilemma for the eye care provider because it may not involve the typical cranial arteries. When any of its potential ocular complications are diagnosed, it is important to consider this unusual form of GCA.
PURPOSE: To report an unusual ophthalmic presentation of large-vessel GCA with sequential bilateral anterior ischemic optic neuropathy and branch retinal artery occlusion.
METHODS: A 65-year-old previously healthy woman experienced sequential bilateral anterior ischemic optic neuropathy with branch retinal artery occlusion in the absence of other signs and symptoms suggestive of cranial GCA.
RESULTS: Extensive workup, including temporal artery biopsy, failed to demonstrate vascular inflammation suggestive of GCA or vascular abnormalities, such as atheromatous plaques, but coincidentally revealed a breast tumor, which was excised. Positron emission tomography scan was performed revealing distinct hypermetabolism of the thoracic and abdominal aorta consistent with large-vessel GCA, and corticosteroid therapy was initiated.
CONCLUSIONS: Large-vessel GCA is an underdiagnosed and undertreated type of GCA that does not typically affect the cranial arteries but rather larger proximal aortic branches. When associated with ocular complications, it can be a puzzling diagnostic dilemma for the eye care provider.

PMID: 29120976 [PubMed - indexed for MEDLINE]

Strawberry Gingivitis in Granulomatosis with Polyangiitis.

ven, 08/12/2017 - 07:15
Related Articles

Strawberry Gingivitis in Granulomatosis with Polyangiitis.

N Engl J Med. 2017 Nov 23;377(21):2073

Authors: Ghiasi M

PMID: 29166230 [PubMed - indexed for MEDLINE]

Cardiovascular and type 2 diabetes morbidity and all-cause mortality among diverse chronic inflammatory disorders.

ven, 08/12/2017 - 07:15
Related Articles

Cardiovascular and type 2 diabetes morbidity and all-cause mortality among diverse chronic inflammatory disorders.

Heart. 2017 Dec;103(23):1867-1873

Authors: Dregan A, Chowienczyk P, Molokhia M

Abstract
OBJECTIVES: The present study aimed to assess the relationship between inflammatory disorders with cardiometabolic diseases and mortality within a community-based population.
METHODS: The UK Biobank data were used to conduct two investigations: a cross-sectional study to estimate cardiometabolic risk and a prospective cohort study to estimate mortality risk. Binary regression analyses were used to model the association between coronary heart disease, stroke, type 2 diabetes, venous thromboembolism and peripheral artery disease diagnoses with seven inflammatory disorders (eg, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), psoriasis, ankylosing spondylitis (AS), systemic vasculitis, Crohn's disease and ulcerative colitis (UC)). Cox proportional hazards was used to estimate all-cause and cardiovascular-related mortality.
RESULTS: About 4% (n=19, 082) of the study population (n=5 02 641) were diagnosed with a chronic inflammatory disorder. The most common inflammatory disorder was psoriasis (n=6286), and the least common was SLE (n=654). SLE showed the strongest association with multiple (relative risk (RR) 6.36, 95% CI 4.37 to 9.25) risk of cardiometabolic diseases, followed by the RA (RR 1.70, 95% CI 1.59 to 1.83), UC (RR 1.69, 95% CI 1.51 to 1.89), AS (RR 1.28, 95% CI 1.09 to 1.52), vasculitis (RR 1.64, 95% CI 1.42-1.90) and psoriasis (RR 1.25, 95% 1.16 to 1.35) disorders. The magnitude of the association was higher among participants prescribed non-steroidal anti-inflammatory drugs or corticosteroids drugs, with multiple cardiometabolic risk being greater within SLE (RR 12.35, 95% CI 7.18 to 21.24), followed by UC (RR 3.81, 95% CI 2.69 to 5.38), Crohn's disease (RR 3.07, 95% CI 1.85 to 5.11), RA (RR 3.06, 95% CI 2.44 to 3.85), psoriasis (RR 2.36, 95% CI 1.88 to 2.95), AS (RR 2.25, 95% CI 1.48 to 3.41) and vasculitis (RR 1.89, 95% CI 1.28 to 2.79). Similar pattern was observed with respect to the cumulative cardiometabolic risk.
CONCLUSION: Inflammatory disorders are associated with heightened risk of cardiometabolic events, which may vary by anti-inflammatory therapy and duration. All-cause mortality was also higher among specific inflammatory disorders compared with the absence of inflammatory disorders.

PMID: 28601812 [PubMed - indexed for MEDLINE]

Endovascular Repair as a Bridge to Open Repair of a Ruptured Descending Thoracic Aspergillus Aortitis.

ven, 08/12/2017 - 07:15
Related Articles

Endovascular Repair as a Bridge to Open Repair of a Ruptured Descending Thoracic Aspergillus Aortitis.

Ann Thorac Surg. 2017 Dec;104(6):e425-e428

Authors: Tresson P, Roussel A, Mordant P, Cerceau P, Castier Y, Pellenc Q

Abstract
Invasive aspergillosis rarely involves the thoracic aorta and is associated with a poor prognosis. A 56-year-old heart transplant recipient presented with invasive aspergillosis, primary Aspergillus aortitis, and a ruptured thoracic aorta pseudoaneurysm. Open surgical repair was not possible because of severe sepsis. Therefore, a sequential surgical strategy was planned, including emergency thoracic endovascular aortic repair, followed by antifungal treatment and definitive open repair with explantation of the endograft and placement of a cryopreserved arterial allograft under extracorporeal membrane oxygenator support. The infection did not reoccur during follow-up, and the patient remained alive and well 13 months after the operation.

PMID: 28499737 [PubMed - indexed for MEDLINE]

Retinal and Choroidal Thickness in Adult Patients with Familial Mediterranean Fever.

ven, 08/12/2017 - 07:15
Related Articles

Retinal and Choroidal Thickness in Adult Patients with Familial Mediterranean Fever.

Ophthalmic Epidemiol. 2017 Oct;24(5):346-351

Authors: Bicer T, Celikay O, Kosker M, Alp MY, Ozisler C, Yesilyurt A, Kucuk Bicer B, Gurdal C

Abstract
PURPOSE: We aimed to evaluate changes in retinal, choroidal, ganglion cell complex (GCC) and retinal nerve fiber layer (RNFL) thicknesses in genetically diagnosed adult patients with familial Mediterranean fever (FMF).
METHODS: A total of 50 eyes of 50 genetically diagnosed patients with FMF and 50 eyes of controls were analyzed. Patients were recruited from the Genetic Diagnostic Center of Dışkapı Yıldırım Beyazıt Research and Training Hospital, Turkey. Retinal and choroidal thicknesses were obtained using spectral-domain optical coherence tomography from choroid, retina, GCC, and RNFL.
RESULTS: Average baseline choroidal thickness was statistically significantly thinner in patients with FMF than controls at Ccenter (325.85 ± 30.8 µm and 338.97 ± 23.9 µm, respectively, p = 0.038), Cnasal500 (328.77 ± 31.6 µm and 349.00 ± 23.3 µm, respectively, p = 0.002), Cnasal1000 (324.97 ± 33.6 µm and 351.23 ± 23.8 µm respectively, p = 0.0001) and Cnasal1500 (324.75 ± 37.1 µm and 344.61 ± 27.3 µm, respectively, p = 0.008). However, there was no significant difference in temporal choroidal thickness (Ctemporal500, Ctemporal1000 and Ctemporal1500) in patients with FMF compared to controls (p > 0.05). There were no significant differences in retinal, GCC and RNFL thicknesses between the groups (p > 0.05).
CONCLUSION: We hypothesize that the chronic inflammation seen in FMF could be the reason for the reduction seen in choroidal thickness in adult patients with FMF. Retinal, GCC and RNFL thicknesses did not differ from controls.

PMID: 28319450 [PubMed - indexed for MEDLINE]

An Unusual Cause of Leg Pain.

mar, 05/12/2017 - 06:14
Related Articles

An Unusual Cause of Leg Pain.

N Engl J Med. 2017 Nov 16;377(20):e29

Authors: Kelly NP, Vaidya A, Gerhard-Herman M, Desai AS

PMID: 29141172 [PubMed - indexed for MEDLINE]

A case of fatal perimyocarditis due to a rare disease.

mar, 05/12/2017 - 06:14
Related Articles

A case of fatal perimyocarditis due to a rare disease.

Forensic Sci Med Pathol. 2017 Dec;13(4):454-458

Authors: Plenzig S, Heinbuch S, Held H, Verhoff MA, Lux C

Abstract
Although myocarditis is caused by viral infections in about 50% of cases in European countries, various other causative agents are known. We report the case of a 51-year-old man who died several months after being diagnosed with asthma by his general practitioner. This diagnosis had been confirmed by a pulmonologist approximately 6 weeks before the man's death. To rule out the possibility of medical malpractice the prosecuting authority ordered a forensic autopsy. At autopsy macroscopic indicators for perimyocarditis and pneumonia were found. Microbiological and histological examination of tissue samples confirmed a diagnosis of Churg-Strauss syndrome, also known as Eosinophilic Granulomatosis with Polyangiitis (EGPA). The cause of death was determined to be cardiac involvement in Churg-Strauss syndrome. The presence of this disease also accounted for the man's recent medical history. There were no findings to indicate that a medical error had been made. The reported case illustrates why accessory histological and microbiological examinations should always be performed when macroscopic findings at autopsy suggest myocarditis. Determining the etiology of myocarditis is a necessary step to prevent overlooking rare diseases with inflammatory myocardial involvement, especially in the clarification of alleged medical malpractice.

PMID: 29022232 [PubMed - indexed for MEDLINE]

A case report of Takayasu arteritis with aortic dissection as initial presentation.

sam, 02/12/2017 - 06:01
Related Articles

A case report of Takayasu arteritis with aortic dissection as initial presentation.

Medicine (Baltimore). 2017 Nov;96(45):e8610

Authors: Guo J, Zhang G, Tang D, Zhang J

Abstract
RATIONALE: The initial symptoms and signs of Takayasu arteritis vary due to the heterogeneity of affected vessels. Moreover, the vascular lesions are difficult to detect at initial presentation, making diagnosis even more challenging. Although cases of aortic dissection with arteritis history have been reported, Takayasu arteritis in men with aortic dissection as initial presentation is very rare.
PATIENT CONCERNS: A 37-year-old man presenting with persistent chest and back pain for 6 days was transferred to our hospital for further treatment. Left hand pulse was absent and right lower limb pulse was feeble. Blood pressure was 144/83 mmHg in the right arm but only 114/62 mmHg in the left arm.
DIAGNOSES: Computed tomography angiography revealed aortic dissection (DeBakey type III b) from the descending aorta to the distal abdominal aorta.
INTERVENTIONS: High-dose glucocorticoid therapy and immunosuppressive therapy have been used to control inflammatory reaction during acute period of Takayasu arteritis. Endovascular graft exclusion (EVGE) surgery was performed to cover the primary entry tear and re-expand true lumen during inactive stage.
OUTCOMES: His pain symptoms improved progressively and he was followed in our outpatient clinic after discharged from hospital, without recurrence.
LESSONS: Timely therapy (glucocorticoid and immunosuppressive) and corrective surgery (endovascular graft exclusion) for Takayasu arteritis with aortic dissection at the inactive stage is essential and beneficial.

PMID: 29137089 [PubMed - indexed for MEDLINE]

Recurrent Pulmonary Aneurysms: Hughes-Stovin Syndrome on the Spectrum of Behçet Disease.

sam, 02/12/2017 - 06:01
Related Articles

Recurrent Pulmonary Aneurysms: Hughes-Stovin Syndrome on the Spectrum of Behçet Disease.

Chest. 2017 Nov;152(5):e99-e103

Authors: Bennji SM, du Preez L, Griffith-Richards S, Smit DP, Rigby J, Koegelenberg CFN, Irusen EM, Allwood BW

Abstract
In this report, we describe a male patient who presented with recurrent life-threatening hemoptysis due to the sequential formation of multiple pulmonary aneurysms. Both pulmonary artery coil embolization and right lower lobectomy were performed, with limited success. The patient experienced extensive bilateral femoral DVT extending into the inferior vena cava, with massive hemoptysis, fulfilling the diagnosis of Hughes-Stovin syndrome. A final diagnosis of Behçet disease was made following extensive investigation, and the patient responded well to prednisone 20 mg orally and azathioprine 100 mg orally.

PMID: 29126538 [PubMed - indexed for MEDLINE]

Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Presenting With Branch Retinal Artery Occlusion.

sam, 02/12/2017 - 06:01
Related Articles

Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome Presenting With Branch Retinal Artery Occlusion.

Ophthalmic Surg Lasers Imaging Retina. 2017 Nov 01;48(11):948-951

Authors: Zacharia JA, Chin AT, Rebhun CB, Louzada RN, Adhi M, Cole ED, Moreira-Neto C, Waheed NK, Duker JS

Abstract
Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) is a rare syndrome affecting the retinal and optic disc vasculature. Diffuse retinal ischemia, macular edema, and neovascularization may lead to bilateral vision loss. The authors report a case of a 36-year-old woman presenting with branch retinal artery occlusion (BRAO) in her right eye who was subsequently diagnosed with IRVAN syndrome. She was treated with panretinal photocoagulation for peripheral retinal ischemia and pars plana vitrectomy for vitreous hemorrhage. She later developed a BRAO in her left eye. This case demonstrates that BRAO may be a presenting feature of IRVAN syndrome. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:948-951.].

PMID: 29121367 [PubMed - indexed for MEDLINE]

Neuropathy and a rash.

sam, 02/12/2017 - 06:01
Related Articles

Neuropathy and a rash.

BMJ. 2017 10 05;359:j4109

Authors: Xie F, Creamer D

PMID: 28982972 [PubMed - indexed for MEDLINE]

The Incidence of Ocular Tuberculosis in Australia Over the Past 10 Years (2006-2015).

sam, 02/12/2017 - 06:01
Related Articles

The Incidence of Ocular Tuberculosis in Australia Over the Past 10 Years (2006-2015).

Ophthalmic Epidemiol. 2017 Dec;24(6):406-412

Authors: Darian-Smith E, Lin ML, Lim LL, McCluskey P, Hall AJ

Abstract
PURPOSE: To determine the incidence and clinical phenotype of ocular tuberculosis in Australia based on the mandatory jurisdictional health notification records for TB.
METHODS: A whole population retrospective case series (Australia). Patients diagnosed with ocular tuberculosis were identified over the past 10 years (1 January 2006 to 31 December 2015) as recorded by individual Health Department jurisdictions per mandatory health notifications. The incidence rates were calculated based on the available Australian census data. Incidence rates were age and sex standardized.
RESULTS: A total of 162 cases of ocular tuberculosis were identified across Australia over a 10-year time period. Of these, 156 participants were overseas born. The 10-year Australian incidence of ocular tuberculosis was 0.77 per 100,000 people. While there has been a downward trend in overall TB annual incidence rates from 2010 to 2015, over the same period the annual incidence of ocular TB has increased compared to the 4 previous years. Descriptive clinical data regarding the ocular manifestations of TB was available in 73/157 patients. In these 73 patients the commonest manifestations of ocular TB were unspecified uveitis (50.1%), focal, multifocal or serpiginous choroiditis or chorioretinitis (12.3%) and retinal vasculitis (11.0%). Of patients with ocular TB, 4/162 (2.47%) had associated pulmonary TB and 8/162 (4.94%) had associated systemic (non-pulmonary) TB. Systemic anti-TB therapy was administered to 161 patients.
CONCLUSIONS: The annual Australian incidence of ocular tuberculosis was 0.077 per 100,000 people. Increasing notifications in the past 6 years may demonstrate increased awareness and changing diagnostic criteria of the disease in the Australian population.

PMID: 28901810 [PubMed - indexed for MEDLINE]

Behcet's disease with recurrent thoracic aortic aneurysm combined with femoral artery aneurysm: a case report and literature review.

sam, 02/12/2017 - 06:01
Related Articles

Behcet's disease with recurrent thoracic aortic aneurysm combined with femoral artery aneurysm: a case report and literature review.

J Cardiothorac Surg. 2017 Sep 06;12(1):79

Authors: Zhang SH, Zhang FX

Abstract
BACKGROUND: Aneurysm or pseudoaneurysm is the main vascular complication of Behcet's disease. Most hospitals adopt endovascular treatment.
CASE PRESENTATION: We report a case of Behcet's disease with recurrent thoracic aortic aneurysm combined with femoral artery aneurysm. The patient underwent two rounds of endovascular surgery, but developed new aneurysms immediately after surgery. Eventually, the patient died due to rupture of recurrent aneurysm.
CONCLUSIONS: For vasculo-Behcet's disease, we suggest performing the operation during the stable period. At the same time, glucocorticoids could be used with immunosuppressants preoperatively and postoperatively.

PMID: 28874203 [PubMed - indexed for MEDLINE]

Idiopathic Myointimal Hyperplasia of Mesenteric Veins: An Uncommon Cause of Ischemic Colitis With Distinct Mucosal Features.

sam, 02/12/2017 - 06:01
Related Articles

Idiopathic Myointimal Hyperplasia of Mesenteric Veins: An Uncommon Cause of Ischemic Colitis With Distinct Mucosal Features.

Am J Surg Pathol. 2017 Dec;41(12):1657-1665

Authors: Yantiss RK, Cui I, Panarelli NC, Jessurun J

Abstract
Idiopathic myointimal hyperplasia of mesenteric veins causes chronic ischemic mucosal injury with segmental strictures that mimic inflammatory bowel disease and nonocclusive ischemic colitis. It is characterized by myointimal proliferative changes that narrow the lumina of veins combined with ischemic injury and ulcers. Most cases reported to date have been diagnosed following surgical resection. The aim of this study was to determine whether mucosal changes of idiopathic myointimal hyperplasia of mesenteric veins are sufficiently sensitive and specific to allow its recognition in biopsy material. The study group consisted of 10 patients with idiopathic myointimal hyperplasia of mesenteric veins who underwent surgical resection of the affected colon, 7 of whom had available prior endoscopic biopsies. The control group included 10 patients each with radiation, nonocclusive ischemia, Crohn disease, diverticulitis, and mucosal amyloidosis, and 5 cases of small vessel (leukocytoclastic) vasculitis. Study patients were mostly older men with distal colorectal disease. All resection specimens showed mucosal ischemia with numerous thick-walled (arteriolized) capillaries and glassy subendothelial fibrin deposits; numerous hyalinized, eosinophilic thrombi were detected in 90% of colectomy specimens. Biopsies showed arteriolized capillaries (100%), subendothelial fibrin deposits (86%), fibrin thrombi (43%), and perivascular hyalinization (43%). Fibrin thrombi were observed in only one case each of ischemic colitis and small vessel vasculitis, and none of the other abovementioned features were seen in any of the controls. We conclude that arteriolized capillaries, subendothelial fibrin deposits, and perivascular hyalinization are frequent and specific features that can facilitate recognition of idiopathic myointimal hyperplasia of mesenteric veins in biopsy samples.

PMID: 28817406 [PubMed - indexed for MEDLINE]

Epidemiology and burden of systemic lupus erythematosus in a Southern European population: data from the community-based lupus registry of Crete, Greece.

sam, 02/12/2017 - 06:01
Related Articles

Epidemiology and burden of systemic lupus erythematosus in a Southern European population: data from the community-based lupus registry of Crete, Greece.

Ann Rheum Dis. 2017 Dec;76(12):1992-2000

Authors: Gergianaki I, Fanouriakis A, Repa A, Tzanakakis M, Adamichou C, Pompieri A, Spirou G, Bertsias A, Kabouraki E, Tzanakis I, Chatzi L, Sidiropoulos P, Boumpas DT, Bertsias GK

Abstract
OBJECTIVES: Several population-based studies on systemic lupus erythematosus (SLE) have been reported, yet community-based, individual-case validated, comprehensive reports are missing. We studied the SLE epidemiology and burden on the island of Crete during 1999-2013.
METHODS: Multisource case-finding included patients ≥15 years old. Cases were ascertained by the ACR 1997, SLICC 2012 criteria and rheumatologist diagnosis, and validated through synthesis of medical charts, administrative and patient-generated data.
RESULTS: Overall age-adjusted/sex-adjusted incidence was 7.4 (95% CI 6.8 to 7.9) per 100 000 persons/year, with stabilising trends in women but increasing in men, and average (±SD) age of diagnosis at 43 (±15) years. Adjusted and crude prevalence (December 2013) was 123.4 (113.9 to 132.9) and 143 (133 to 154)/105 (165/105 in urban vs 123/105 in rural regions, p<0.001), respectively. Age-adjusted/sex-adjusted nephritis incidence was 0.6 (0.4 to 0.8) with stable trends, whereas that of neuropsychiatric SLE was 0.5 (0.4 to 0.7) per 100 000 persons/year and increasing. Although half of prevalent cases had mild manifestations, 30.5% developed organ damage after 7.2 (±6.6) years of disease duration, with the neuropsychiatric domain most frequently afflicted, and 4.4% of patients with nephritis developed end-stage renal disease. The ACR 1997 and SLICC 2012 classification criteria showed high concordance (87%), yet physician-based diagnosis occurred earlier than criteria-based in about 20% of cases.
CONCLUSIONS: By the use of a comprehensive methodology, we describe the full spectrum of SLE from the community to tertiary care, with almost half of the cases having mild disease, yet with significant damage accrual. SLE is not rare, affects predominantly middle-aged women and is increasingly recognised in men. Neuropsychiatric disease is an emerging frontier in lupus prevention and care.

PMID: 28780511 [PubMed - indexed for MEDLINE]

Current Pathologic Scoring Systems for Metal-on-metal THA Revisions are not Reproducible.

sam, 02/12/2017 - 06:01
Related Articles

Current Pathologic Scoring Systems for Metal-on-metal THA Revisions are not Reproducible.

Clin Orthop Relat Res. 2017 Dec;475(12):3005-3011

Authors: Smeekes C, Cleven AHG, van der Wal BCH, Dubois SV, Rouse RW, Ongkiehong BF, Wolterbeek R, Nelissen RGHH

Abstract
BACKGROUND: The aseptic lymphocyte vasculitis-associated lesion (ALVAL) score and the modified Oxford ALVAL score are frequently used scoring methods to evaluate the morphologic features of periprosthetic tissues around metal-on-metal (MoM) hip implants. Except for the initial studies of these two morphology scoring methods, to our knowledge, no other studies have reported on intraclass correlation coefficient (ICC) values for interobserver reliability of these scoring methods.
QUESTIONS/PURPOSES: Are the ALVAL and Oxford ALVAL scores reproducible?
METHODS: The periprosthetic tissue of 37 revisions of 36 patients with failed MoM THAs were independently scored by three experienced pathologists using ALVAL and Oxford ALVAL scoring methods. All patients were included who underwent revision surgery in our hospital until January 2013, with a large-head MoM prosthesis and also met the criteria: blood serum cobalt levels, available MRI scan, and intraarticular cobalt levels. The population included 26 patients with pseudotumors diagnosed by two radiologists using the method described by Matthies et al. The ALVAL describes morphologic features of the synovial lining, tissue organization, and inflammatory cell infiltrate in periprosthetic tissues. The Oxford-ALVAL score uses a semiquantitative measure of the immune response which should be easier to score.
RESULTS: The ALVAL score showed an ICC of 0.38 (95% CI, 0.18-0.58) (fair) for the sum score and this improved up to 0.50 (95% CI, 0.31-0.68) (moderate) using the modified Oxford ALVAL score. The individual parameters of the ALVAL score showed an ICC for the scoring of inflammatory infiltrate of 0.37 (95% CI, 0.17-0.57), an ICC of 0.32 (95% CI, 0.12-0.53) for the scoring of tissue organization, and an ICC of 0.14 (95% CI, -0.04 to 0.34) for synovial lining.
CONCLUSIONS: Scoring morphologic features of MoM tissue is not reproducible using the ALVAL score or the Oxford ALVAL score. This may reflect heterogeneous morphologic features in tumor tissue and between different tumor tissue samples that cannot be reliably quantified by pathologists using the parameters of these two scoring methods. An alternative, simplified scoring system should be developed to improve the interrater agreement.
LEVEL OF EVIDENCE: Level III, diagnostic study.

PMID: 28688018 [PubMed - indexed for MEDLINE]

Rare esophageal ulcers related to Behçet disease: A case report.

ven, 10/11/2017 - 18:19
Related Articles

Rare esophageal ulcers related to Behçet disease: A case report.

Medicine (Baltimore). 2017 Nov;96(44):e8469

Authors: Jia N, Tang Y, Liu H, Li Y, Liu S, Liu L

Abstract
RATIONALE: The fundamental pathogenesis of Behçet disease (BD) is still unclear and controversial. Many cases of oral aphthous ulcers and genital ulcers related to BD are reported; nevertheless, idiopathic giant esophageal ulcers related to BD are rare. A rare case for esophageal ulcers related to BD is presented.
PATIENT CONCERNS: In China, BD is represented with esophageal involvement which is called esophageal BD (EBD).
DIAGNOSES: A 56-year-old man diagnosed to the Gastroenterology Department of Integrated Traditional Chinese and Western Medicine Hospital, for multiple discrete, elliptical esophageal ulcers related to BD.
INTERVENTIONS: The esophageal ulcers were treated with corticosteroid treatment for 12 weeks.
OUTCOME: The esophageal ulcers were cured.
LESSONS: Our report might give further strength to avoiding the erroneous diagnosis or missed diagnosis for EBD, which is different from esophageal carcinoma, esophageal tuberculosis and esophageal Crohns disease.

PMID: 29095300 [PubMed - indexed for MEDLINE]

Management of Kawasaki disease in adults.

ven, 10/11/2017 - 18:19
Related Articles

Management of Kawasaki disease in adults.

Heart. 2017 Nov;103(22):1760-1769

Authors: Denby KJ, Clark DE, Markham LW

Abstract
Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Since the vast majority of Kawasaki disease initially presents at <5 years of age, many adult cardiologists are unfamiliar with the pathophysiology of this disease. This vasculitis has a predilection for coronary arteries with a high complication rate across the lifespan for those with medium to large coronary artery aneurysms. An inflammatory cascade produces endothelial dysfunction and damage to the vascular wall, leading to aneurysmal dilatation. Later, pseudonormalisation of the vascular lumen occurs through vascular remodelling and layering thrombus, but this does not necessarily indicate resolution of disease or reduction of risk for future complications. There is a growing prevalence of Kawasaki disease, making it increasingly relevant for adult cardiologists as this population transitions into adulthood. As the 2017 American Heart Association (AHA) and 2014 Japanese Circulation Society (JCS) guidelines emphasise, Kawasaki disease requires rigorous follow-up with cardiac stress testing and non-invasive imaging to detect progressive stenosis, thrombosis and luminal occlusion that may lead to myocardial ischaemia and infarction. Due to differences in disease mechanisms, coronary disease due to Kawasaki disease should be managed with different pharmacological and non-pharmacological treatment algorithms than atherosclerotic coronary disease. This review addresses gaps in the current knowledge of the disease and its optimal treatment, differences in the AHA and JCS guidelines, targets for future research and obstacles to transition of care from adolescence into adulthood.

PMID: 28751537 [PubMed - indexed for MEDLINE]

A comparison of patients' and physicians' assessments of disease activity using the Swedish version of the Systemic Lupus Activity Questionnaire.

ven, 10/11/2017 - 18:19
Related Articles

A comparison of patients' and physicians' assessments of disease activity using the Swedish version of the Systemic Lupus Activity Questionnaire.

Scand J Rheumatol. 2017 Nov;46(6):474-483

Authors: Pettersson S, Svenungsson E, Gustafsson J, Möller S, Gunnarsson I, Welin Henriksson E

Abstract
OBJECTIVES: We compared patients' assessments of systemic lupus erythematosus (SLE) disease activity by a Swedish version of the Systemic Lupus Activity Questionnaire (SLAQ) with physicians' assessments by the Systemic Lupus Activity Measure (SLAM) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K). We also explored the performance of the SLAQ in patients with short (< 1 year) versus long (≥ 1 year) disease duration.
METHOD: Patients filled out the SLAQ before physicians' assessments. Correlations between SLAQ total, subscales (Symptom score, Flares, Patients global) and SLAM and SLEDAI-2K, as well as between the corresponding items in SLAQ and SLAM, were evaluated using Spearman's ρ. Comparisons between patients with different disease durations were performed with Mann-Whitney U or chi-squared tests.
RESULTS: We included 203 patients (79% women), with a median age of 45 years [interquartile range (IQR) 33-57 years] and disease duration of 5 years (IQR 0-14 years). Correlations between physicians' SLAM without laboratory items (SLAM-nolab) and patients' assessments were: SLAQ total, ρ = 0.685, Symptom score, ρ = 0.651, Flares, ρ = 0.547, and Patients global, ρ = 0.600. Of the symptom items, fatigue (ρ = 0.640), seizures (ρ = 0.635), and headache (ρ = 0.604) correlated most closely. Neurology/stroke syndrome, skin, and lymphadenopathy correlated less well (ρ < 0.24). Patients' and physicians' assessments were notably more discordant for patients with short disease durations.
CONCLUSION: We confirm that the SLAQ can be used to monitor disease activity. However, the discrepancy between patients' and physicians' assessments was greater for patients with short versus long disease duration. We encourage further use of the SLAQ, but would like to develop a shorter version which would be valuable in modern, partly web-based, clinical care.

PMID: 28293972 [PubMed - indexed for MEDLINE]

Risk of ocular manifestations in patients with giant cell arteritis: a nationwide study in Sweden.

ven, 10/11/2017 - 18:19
Related Articles

Risk of ocular manifestations in patients with giant cell arteritis: a nationwide study in Sweden.

Scand J Rheumatol. 2017 Nov;46(6):484-489

Authors: Ji J, Dimitrijevic I, Sundquist J, Sundquist K, Zöller B

Abstract
OBJECTIVE: No large-scale nationwide study has determined the risk of ocular manifestations in patients with giant cell arteritis (GCA). The aim was to study the incidence and risk factors of ocular manifestations in patients with GCA in Sweden.
METHOD: A national cohort was created by linking Swedish nationwide registers. GCA patients were identified from the Swedish Hospital Inpatient and Outpatient Registers between 2002 and 2010, and were followed until the development of ocular manifestations. Standardized incidence ratios (SIRs) and 95% confidence intervals (CIs) were calculated for ocular manifestations in patients with GCA compared to those without GCA.
RESULTS: We identified 3737 males and 8311 females with GCA. A total of 1618 individuals had subsequent ocular manifestations, representing 13.4% of the GCA patients. The overall SIR of ocular manifestations was 6.96 (95% CI 6.63-7.31). The risk for disorders of the optic nerve or visual tract was particularly high (SIR = 51.68, 95% CI 46.12-57.73). Men with GCA had a higher risk than women, and GCA patients without polymyalgia rheumatica (PMR) symptoms had a higher risk than those with PMR symptoms. Living outside big cities was negatively associated with ocular manifestations in GCA patients, whereas hypertension and diabetes were associated with an increased risk of ocular manifestations.
CONCLUSION: The overall risk of ocular manifestations was higher in GCA patients than in the general population, especially for men and for those without PMR symptoms.

PMID: 28276953 [PubMed - indexed for MEDLINE]

Pages