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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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Multiple Cranial Nerve Palsies in Giant Cell Arteritis.

dim, 17/12/2017 - 13:30
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Multiple Cranial Nerve Palsies in Giant Cell Arteritis.

J Neuroophthalmol. 2017 Dec;37(4):398-400

Authors: Ross M, Bursztyn L, Superstein R, Gans M

Abstract
Giant cell arteritis (GCA) is a systemic vasculitis of medium and large arteries often with ophthalmic involvement, including ischemic optic neuropathy, retinal artery occlusion, and ocular motor cranial nerve palsies. This last complication occurs in 2%-15% of patients, but typically involves only 1 cranial nerve. We present 2 patients with biopsy-proven GCA associated with multiple cranial nerve palsies.

PMID: 28538036 [PubMed - indexed for MEDLINE]

Relative Frequencies of Arteritic and Nonarteritic Anterior Ischemic Optic Neuropathy in an Arab Population.

dim, 17/12/2017 - 13:30
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Relative Frequencies of Arteritic and Nonarteritic Anterior Ischemic Optic Neuropathy in an Arab Population.

J Neuroophthalmol. 2017 Dec;37(4):382-385

Authors: Gruener AM, Chang JR, Bosley TM, Al-Sadah ZM, Kum C, McCulley TJ

Abstract
BACKGROUND: To evaluate the relative frequencies of arteritic and nonarteritic anterior ischemic optic neuropathy (AION) in an Arab population and to compare and contrast these findings with known epidemiological data from Caucasian populations.
METHODS: A retrospective review of the medical records of all patients diagnosed with AION at the King Khaled Eye Specialist Hospital (KKESH) in Riyadh, Saudi Arabia, between 1997 and 2012.
RESULTS: Of 171 patients with AION, 4 had biopsy-proven giant-cell arteritis (GCA). The relative frequencies of arteritic anterior ischemic optic neuropathy (AAION) and nonarteritic anterior ischemic optic neuropathy (NAION) in this Arab cohort were 2.3% and 97.7%, respectively.
CONCLUSIONS: The relative frequencies of arteritic anterior ischemic optic neuropathy and nonarteritic anterior ischemic optic neuropathy differ between Arab and North American clinic-based populations, with giant-cell arteritis-related ischemia being much less frequent in Saudi Arabia.

PMID: 28099197 [PubMed - indexed for MEDLINE]

Aortic Aneurysm in Takayasu Arteritis.

jeu, 14/12/2017 - 11:25
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Aortic Aneurysm in Takayasu Arteritis.

Am J Med Sci. 2017 Dec;354(6):539-547

Authors: Yang KQ, Meng X, Zhang Y, Fan P, Wang LP, Zhang HM, Wu HY, Jiang XJ, Cai J, Zhou XL, Hui RT, Zheng DY, Liu LS

Abstract
BACKGROUND: Aortic aneurysm (AA) is a severe complication of Takayasu arteritis (TA). This study aimed to evaluate the prevalence, clinical and imaging features, management and long-term outcomes of AA in patients with TA.
MATERIALS AND METHODS: A retrospective study was performed of TA patients with AA admitted to Fuwai Hospital from 1996-2015. Baseline clinical data and follow-up data of TA patients with AA were collected and analyzed.
RESULTS: Thirty-nine (4.2%) of 934 patients with TA were identified with AA that was related to vasculitis. The mean age at disease onset was 31 ± 10 years, with a female-to-male ratio of 1.79:1. The ascending aorta was the most common site of the aneurysmal lesion (18, 33.3%), and the most frequent manifestations associated with AA were chest tightness (12, 30.8%) and shortness of breath (12, 30.8%), which were usually concomitant with aortic valve insufficiency. Involvement of multiple sites in AA was found in 8 patients (20.5%), and multiple AAs were found in 5 patients (12.8%). No significant difference was observed in clinical and imaging findings between sexes. Of 25 patients (64.1%) with a median 72-month follow-up, 1 patient suffered from heart failure owing to perivalvular leakage, and 1 patient died, possibly related to severe complications of the operation.
CONCLUSIONS: The prevalence of AA is relatively low in Chinese patients with TA. AA seems to develop more frequently in male patients with TA. Management should consider location and size of AA, complexity of vessel lesions and disease status. Long-term follow-up is indispensable.

PMID: 29208249 [PubMed - indexed for MEDLINE]

Internal tissue references for 18Fluorodeoxyglucose vascular inflammation imaging: Implications for cardiovascular risk stratification and clinical trials.

jeu, 14/12/2017 - 11:25
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Internal tissue references for 18Fluorodeoxyglucose vascular inflammation imaging: Implications for cardiovascular risk stratification and clinical trials.

PLoS One. 2017;12(11):e0187995

Authors: Ahlman MA, Vigneault DM, Sandfort V, Maass-Moreno R, Dave J, Sadek A, Mallek MB, Selwaness MAF, Herscovitch P, Mehta NN, Bluemke DA

Abstract
INTRODUCTION: 18Fluorodeoxyglucose (FDG) positron emission tomography (PET) uptake in the artery wall correlates with active inflammation. However, in part due to the low spatial resolution of PET, variation in the apparent arterial wall signal may be influenced by variation in blood FDG activity that cannot be fully corrected for using typical normalization strategies. The purpose of this study was to evaluate the ability of the current common methods to normalize for blood activity and to investigate alternative methods for more accurate quantification of vascular inflammation.
MATERIALS AND METHODS: The relationship between maximum FDG aorta wall activity and mean blood activity was evaluated in 37 prospectively enrolled subjects aged 55 years or more, treated for hyperlipidemia. Target maximum aorta standardized uptake value (SUV) and mean background reference tissue activity (blood, spleen, liver) were recorded. Target-to-background ratios (TBR) and arterial maximum activity minus blood activity were calculated. Multivariable regression was conducted, predicting uptake values based on variation in background reference and target tissue FDG uptake; adjusting for gender, age, lean body mass (LBM), blood glucose, blood pool activity, and glomerular filtration rate (GFR), where appropriate.
RESULTS: Blood pool activity was positively associated with maximum artery wall SUV (β = 5.61, P<0.0001) as well as mean liver (β = 6.23, P<0.0001) and spleen SUV (β = 5.20, P<0.0001). Artery wall activity divided by blood activity (TBRBlood) or subtraction of blood activity did not remove the statistically significant relationship to blood activity. Blood pool activity was not related to TBRliver and TBRspleen (β = -0.36, P = NS and β = -0.58, P = NS, respectively).
CONCLUSIONS: In otherwise healthy individuals treated for hyperlipidemia, blood FDG activity is associated with artery wall activity. However, variation in blood activity may mask artery wall signal reflective of inflammation, which requires normalization. Blood-based TBR and subtraction do not sufficiently adjust for blood activity. Warranting further investigation, background reference tissues with cellular uptake such as the liver and spleen may better adjust for variation in blood activity to improve assessment of vascular activity.

PMID: 29131857 [PubMed - indexed for MEDLINE]

Middle-Aged Male With Right Neck Pain.

jeu, 14/12/2017 - 11:25
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Middle-Aged Male With Right Neck Pain.

Ann Emerg Med. 2017 Oct;70(4):463-494

Authors: He Z, Zhang G, Chen G, He X

PMID: 28946974 [PubMed - indexed for MEDLINE]

The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study.

jeu, 14/12/2017 - 11:25
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The Epidemiology of Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis in Olmsted County, Minnesota: A Twenty-Year US Population-Based Study.

Arthritis Rheumatol. 2017 Dec;69(12):2338-2350

Authors: Berti A, Cornec D, Crowson CS, Specks U, Matteson EL

Abstract
OBJECTIVE: To estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), in a US-based adult population.
METHODS: All medical records of patients with a diagnosis of, or suspicion of having, AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. AAV incidence rates were age- and sex-adjusted to the 2010 US white population. Age- and sex-adjusted prevalence of AAV was calculated on January 1, 2015. Survival rates observed in the study cohort were compared with expected rates in the Minnesota population.
RESULTS: Of the 58 incident cases of AAV in Olmsted County during the study period, 23 (40%) were cases of GPA, 28 (48%) were cases of MPA, and 7 (12%) were cases of EGPA. Overall, 28 (48%) of the patients with AAV were women and 57 (98%) were white. The mean ± SD age at diagnosis was 61.1 ± 16.5 years. Thirty-four patients (61%) had myeloperoxidase (MPO)-ANCAs, and 17 (30%) were positive for proteinase 3 (PR3)-ANCAs; 5 (9%) were ANCA-negative. The annual incidence of AAV was 3.3 per 100,000 population (95% confidence interval [95% CI] 2.4-4.1). The incidence rates of GPA, MPA, and EGPA were 1.3 (95% CI 0.8-1.8), 1.6 (95% CI 1.0-2.2), and 0.4 (95% CI 0.1-0.6), respectively. The overall prevalence of AAV was 42.1 per 100,000 (95% CI 29.6-54.6). The mortality rate among AAV patients overall, and among patients with EGPA, those with MPA, and those with MPO-ANCAs, was increased in comparison to the Minnesota general population (each P < 0.05), whereas mortality rates among patients with GPA, those with PR3-ANCAs, and ANCA-negative patients did not differ from that in the general population.
CONCLUSION: The annual incidence of AAV in Olmsted County, Minnesota over the 20 years of the study was 3.3 per 100,000, with a prevalence of 42.1 per 100,000, which is substantially higher than the rates reported in other areas worldwide. The incidence of GPA was similar to that of MPA. Patients with MPA and those with EGPA, but not patients with GPA, experienced higher rates of mortality than that in the Minnesota general population. MPO-ANCAs were a marker of poor survival in this population of patients with AAV.

PMID: 28881446 [PubMed - indexed for MEDLINE]

Herpes Zoster as a Risk Factor for Incident Giant Cell Arteritis.

jeu, 14/12/2017 - 11:25
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Herpes Zoster as a Risk Factor for Incident Giant Cell Arteritis.

Arthritis Rheumatol. 2017 Dec;69(12):2351-2358

Authors: England BR, Mikuls TR, Xie F, Yang S, Chen L, Curtis JR

Abstract
OBJECTIVE: Histopathologic studies have implicated herpes zoster (HZ) as a causative organism of giant cell arteritis (GCA). The purpose of this study was to assess the epidemiologic association of HZ events with incident GCA.
METHODS: We performed a retrospective cohort study in 2 large independent US administrative data sets: Medicare 5% and Truven Health Analytics MarketScan. Eligible subjects had 12 months of continuous coverage, were >50 years old, and had no history of GCA or polymyalgia rheumatica. HZ events (complicated and uncomplicated) and GCA were identified by the presence of International Classification of Diseases, Ninth Revision, Clinical Modification codes from physician visit or hospital discharge records. Antiviral therapies and vaccinations were identified from prescription claims and drug codes. Risk of incident GCA was calculated using multivariable Cox proportional hazards regression.
RESULTS: Among 16,686,345 subjects, a total of 5,942 GCA cases occurred, with 3.1% (MarketScan) and 6.0% (Medicare) having preceding HZ events. Unadjusted GCA incidence rates were highest in the groups with complicated and uncomplicated HZ. After multivariable adjustment, complicated HZ was associated with an increased risk of GCA (hazard ratio [HR] 1.99 [95% confidence interval (95% CI) 1.32-3.02] in the Medicare cohort and 2.16 [95% CI 1.46-3.18] in the MarketScan cohort), as was uncomplicated HZ (HR 1.42 [95% CI 1.02-1.99] and HR 1.45 [95% CI 1.05-2.01] in the respective cohorts). Vaccination and antiviral treatment were not consistently associated with GCA risk, although antiviral treatment was marginally associated with a decreased risk of GCA in the Medicare cohort (HR 0.67 [95% CI 0.46-0.99]).
CONCLUSION: HZ is associated with an increased risk of GCA. The infrequency of HZ in GCA patients suggests that it is only one potential trigger for GCA. Antivirals and vaccination did not consistently mitigate this risk.

PMID: 28853238 [PubMed - indexed for MEDLINE]

Stroke and methamphetamine use in young adults: a review.

jeu, 14/12/2017 - 11:25
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Stroke and methamphetamine use in young adults: a review.

J Neurol Neurosurg Psychiatry. 2017 Dec;88(12):1079-1091

Authors: Lappin JM, Darke S, Farrell M

Abstract
BACKGROUND: Methamphetamine use and stroke are significant public health problems. Strokes among people aged below 45 years are much less common than in older age groups but have significant mortality and morbidity. Methamphetamine is a putative cause of strokes among younger people.
METHODS: A review of methamphetamine-related strokes was conducted. Bibliographic databases were searched until February 2017 for articles related to methamphetamine and stroke. Both haemorrhagic and ischaemic strokes were considered.
RESULTS: Of 370 articles screened, 77 were selected for inclusion. There were 81 haemorrhagic and 17 ischaemic strokes reported in case reports and series. Both types were approximately twice as common in males. Route of administration associated with haemorrhagic stroke was typically oral or injecting, but for ischaemic stroke inhalation was most common. Haemorrhagic stroke was associated with vascular abnormalities in a third of cases. One quarter of individuals completely recovered, and a third died following haemorrhagic stroke. One-fifth completely recovered, and one-fifth died following ischaemic stroke.
CONCLUSIONS: There is a preponderance of haemorrhagic strokes associated with methamphetamine use in young people, and methamphetamine-related stroke is associated with poor clinical outcomes. Mechanisms of methamphetamine-associated stroke include hypertension, vasculitis, direct vascular toxicity and vasospasm. In a period of rising worldwide methamphetamine use, the incidence of methamphetamine-related stroke will increase, with a consequent increase in the burden of disease contributed by such events.

PMID: 28835475 [PubMed - indexed for MEDLINE]

Brief Report: Drug-Free Long-Term Remission in Severe Behçet's Disease Following Withdrawal of Successful Anti-Tumor Necrosis Factor Treatment.

jeu, 14/12/2017 - 11:25
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Brief Report: Drug-Free Long-Term Remission in Severe Behçet's Disease Following Withdrawal of Successful Anti-Tumor Necrosis Factor Treatment.

Arthritis Rheumatol. 2017 Dec;69(12):2380-2385

Authors: Sfikakis PP, Arida A, Panopoulos S, Fragiadaki K, Pentazos G, Laskari K, Tektonidou M, Markomichelakis N

Abstract
OBJECTIVE: To test the hypothesis that remission of Behçet's disease (BD) in patients with severe vital organ involvement is maintained after withdrawal of successful anti-tumor necrosis factor (anti-TNF) treatment.
METHODS: This single-center, retrospective, longitudinal outcomes study focused on consecutive patients with disease refractory to treatment with conventional immunosuppressant agents who responded to add-on long-term anti-TNF treatment that was subsequently discontinued. The end point was the proportion of patients remaining in complete remission for at least 3 years after withdrawal of anti-TNF treatment.
RESULTS: In our BD cohort comprising 87 patients, 29 were eligible for analysis. All of these patients had disease that was refractory to conventional immunosuppressive therapy and had received successful anti-TNF treatment for a median of 2 years (interquartile range [IQR] 1.1-2.0) before treatment discontinuation. Of these patients, 12 (41%) achieved the study end point. The remaining 17 patients experienced a relapse within 1 year (IQR 0.6-1.5) after discontinuation. Re-treatment with anti-TNF was safe and effective in 14 (82%) of 17 patients; so far, 4 of these patients also achieved the study end point. Overall, 16 patients have remained in complete remission (median 6.5 years [IQR 5.5-8]). Ten of these patients are in drug-free remission (treated with anti-TNF agents, mainly for sight-threatening disease), and 6 are in azathioprine-maintained remission (treated with anti-TNF agents for ocular, intestinal, or central nervous system involvement). Notably, patients in drug-free remission were significantly younger and had a significantly shorter duration of BD when anti-TNF treatment was initiated compared to patients receiving azathioprine maintenance treatment.
CONCLUSION: Drug-free, long-term remission after withdrawal of successful anti-TNF treatment is feasible in patients with severe BD. Because an anti-TNF agent-induced "cure" cannot be differentiated from spontaneous remission by natural history, prospective studies should examine whether anti-TNF agents should be used as first-line treatment for the induction of remission in every patient with vital organ involvement.

PMID: 28834393 [PubMed - indexed for MEDLINE]

Case Report: Masquerading Large-vessel Giant Cell Arteritis.

lun, 11/12/2017 - 07:46
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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Neuropathy and a rash.

BMJ. 2017 10 05;359:j4109

Authors: Xie F, Creamer D

PMID: 28982972 [PubMed - indexed for MEDLINE]

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