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Infliximab as an alternative therapy for refractory adult onset Kawasaki disease: A case report.

Vascularites - sam, 13/10/2018 - 17:57
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Infliximab as an alternative therapy for refractory adult onset Kawasaki disease: A case report.

Medicine (Baltimore). 2018 Oct;97(40):e12720

Authors: Kawaguchi T, Rikitake Y, Tsuruda T, Kawata C, Rikitake M, Iwao K, Aizawa A, Kariya Y, Matsuda M, Miyauchi S, Umekita K, Takajo I, Okayama A

Abstract
RATIONALE: Kawasaki disease (KD) is an acute febrile illness predominantly affecting children less than 5 years of age and characterized by systemic inflammation in all medium-sized arteries. Adult-onset KD (AKD) is rare with only 105 case reports published. Recently, the efficacy of infliximab (IFX) for patients with refractory KD has been demonstrated.
PATIENT CONCERNS: A previously healthy 24-year-old man was admitted because of a persistent fever, and elevated serum level of AST, ALT, LDH, and CRP.
DIAGNOSIS: The patients met the diagnostic criteria for KD based on the findings of persistent fever, polymorphous exanthema, unilateral cervical lymphadenopathy, non-purulent palpebral conjunctivitis and membranous desquamation. Echocardiogram revealed the dilatation at the proximal sites of the right coronary artery (7.9 mm) and left anterior descending artery (5 mm). The patient was treated with high-dose intravenous immunoglobulin (1 g/kg/day for 2 days) and ASA (100 mg daily). However, his fever and arthralgia persisted.
INTERVENTIONS: He was administered single 5 mg/kg doses of IFX.
OUTCOMES: He became afebrile the next day and his arthralgia improved.
LESSONS: We report the first case of administration of IFX in a patient with AKD refractory to intravenous immunoglobulin (IVIG), and successful reduction of systemic inflammation. However, the effectiveness of IFX in the regression of coronary artery aneurysm remains to be determined.

PMID: 30290679 [PubMed - indexed for MEDLINE]

Extensive Gastrointestinal Manifestations as the Main Relapsing Disease in Granulomatosis with Polyangiitis.

Vascularites - mer, 10/10/2018 - 17:00
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Extensive Gastrointestinal Manifestations as the Main Relapsing Disease in Granulomatosis with Polyangiitis.

Dig Dis Sci. 2018 10;63(10):2810-2812

Authors: Vergara-Fernández O, Chablé-Montero F, Ruiz N, Mitre-Reyes D, Cerda-Contreras E, Hernández A, Marroquín-Fabián E, Flores-Suárez LF

PMID: 29862483 [PubMed - indexed for MEDLINE]

Choroidal Thickness Indicates Subclinical Ocular and Systemic Inflammation in Eyes with Behçet Disease without Active Inflammation.

Vascularites - jeu, 04/10/2018 - 11:56
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Choroidal Thickness Indicates Subclinical Ocular and Systemic Inflammation in Eyes with Behçet Disease without Active Inflammation.

Korean J Ophthalmol. 2018 08;32(4):290-295

Authors: Chung YR, Cho EH, Jang S, Lee SY, Lee ES, Lee K

Abstract
PURPOSE: To investigate whether subfoveal choroidal thickness, measured using enhanced depth imaging optical coherence tomography (EDI-OCT), is an indicator of subclinical ocular or systemic inflammation in eyes with Behçet disease (BD) without active ocular inflammation.
METHODS: A retrospective analysis was used to examine clinical features of non-uveitic patients with BD (NUBD group), patients with a previous history of Behçet uveitis in an inactive state (IUBD group), and healthy controls were evaluated from October 2014 to September 2015. Subfoveal choroidal thickness was measured using EDI-OCT.
RESULTS: The NUBD group included 46 eyes in 24 patients; the IUBD group included 16 eyes in 11 patients; and the control group included 35 eyes in 23 individuals. The mean subfoveal choroidal thicknesses differed significantly among these groups. Choroidal thickness was significantly greater in the NUBD (310.5 ± 81.0 μm) than in the IUBD (263.1 ± 56.6 μm, p = 0.013) and control (256.9 ± 67.9 μm, p = 0.002) groups. The disease activity score was significantly higher in the NUBD than in the IUBD group (p < 0.001), while the use of cyclosporine was significantly associated with choroidal thickness in eyes with NUBD (p = 0.039).
CONCLUSIONS: Subfoveal choroidal thickness, as measured by EDI-OCT, may be a clinical indicator of subclinical ocular inflammation and systemic inflammation in BD patients without active ocular inflammation.

PMID: 30091307 [PubMed - indexed for MEDLINE]

Asymmetrical Dimethylarginine Levels in Hepatitis B Virus-Positive Patients.

Vascularites - lun, 01/10/2018 - 10:55
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Asymmetrical Dimethylarginine Levels in Hepatitis B Virus-Positive Patients.

Ann Lab Med. 2018 Sep;38(5):446-449

Authors: Karakecili F, Cikman A, Aydin M, Gulhan B

Abstract
BACKGROUND: High asymmetrical dimethylarginine (ADMA) levels have been associated with endothelial dysfunction and contribute to the development of several diseases. However, data on the relationship between hepatitis B virus (HBV) and ADMA are limited. The aim of our study was to explore the relationship between ADMA and HBV by comparing the ADMA levels in patients with chronic active hepatitis B (CHB), inactive HBV carriers (carriers), and healthy volunteers (controls).
METHODS: The participants were divided into three groups: 90 patients with CHB, 90 HBV carriers, and 90 controls. Serum ADMA levels were quantified using an ELISA kit (Cusabio, Wuhan, China). The data were analyzed using an ANOVA or the Kruskal-Wallis test as appropriate, with P<0.05 considered significant.
RESULTS: Serum ADMA levels were significantly higher in patients with CHB (228.35±91.10 ng/mL) than in HBV carriers (207.80±75.80 ng/mL) and controls (207.61±89.10 ng/mL) (P=0.049). The clinical scores of the patients were positively correlated with ADMA levels.
CONCLUSIONS: The elevated serum ADMA levels in patients with CHB confirm that HBV plays a role in vasculitis. Further investigation of the mechanisms contributing to the high levels of ADMA in CHB may contribute toward development of new treatment modalities.

PMID: 29797815 [PubMed - indexed for MEDLINE]

Reduction of Nicardipine-Related Phlebitis in Patients with Acute Stroke by Diluting Its Concentration.

Vascularites - ven, 28/09/2018 - 08:59
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Reduction of Nicardipine-Related Phlebitis in Patients with Acute Stroke by Diluting Its Concentration.

J Stroke Cerebrovasc Dis. 2018 Jul;27(7):1783-1788

Authors: Kawada K, Ohta T, Tanaka K, Miyamoto N

Abstract
BACKGROUND: Nicardipine is frequently used in the treatment of hypertension for patients with acute stroke; however, its dosing is complicated by a high risk of phlebitis. In the present study, we examined whether restricting nicardipine concentration under a specific value could reduce the incidence of nicardipine-related phlebitis in patients with acute stroke.
METHODS: Intravenous nicardipine-related phlebitis was retrospectively analyzed. From July 2015, a simple proposition was made to dilute maximum intravenous nicardipine concentration to lower than 130 µg/mL. The maximum intravenous nicardipine concentration and the incidence of phlebitis were compared between patients treated from July 2014 to June 2015 (preproposition group) and patients treated from July 2015 to June 2016 (postproposition group).
RESULTS: A total of 300 patients (preproposition group, 138; postproposition group, 162) were included. The postproposition group demonstrated significantly lower maximum intravenous nicardipine concentration (in µg/mL, 76.9, 47.6-104.5 versus 130.4, 69.8-230.8; P < .001) and incidence of phlebitis (9.9%, 16/162 vs. 30%, 42/138; P < .001) than the preproposition group. Multivariable logistic regression analysis revealed that the maximum intravenous nicardipine concentration lower than 130 µg/mL (odds ratio [OR] .15; 95% confidence interval [CI] .06-.35; P < .001) and National Institutes of Health Stroke Scale on admission (OR .95; 95% CI .91-.99; P = .007) were the statistically significant independent factors for phlebitis, which indicated the usefulness of the proposition to dilute maximum intravenous nicardipine concentration to lower than 130 µg/mL.
CONCLUSIONS: The simple and appropriate proposition about nicardipine administration lowered maximum nicardipine concentration and reduced the incidence of nicardipine-related phlebitis in patients with acute stroke.

PMID: 29519742 [PubMed - indexed for MEDLINE]

Antineutrophil cytoplasmic antibodies in Chinese patients with tuberculosis.

Vascularites - sam, 22/09/2018 - 06:14
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Antineutrophil cytoplasmic antibodies in Chinese patients with tuberculosis.

Rev Soc Bras Med Trop. 2018 Jul-Aug;51(4):475-478

Authors: Huan G, Yang G, Xiao-Yu Q, Jiancheng X, Yan-Qing S

Abstract
INTRODUCTION: Based on reports, infection with Mycobacterium tuberculosis is believed to induce the development of antibodies that are considered to be biological indicators for the diagnosis of some other diseases. However, conflicting results have been published regarding the presence of antineutrophil cytoplasmic antibodies (ANCAs) in patients with tuberculosis. We aim to study the seroprevalence of ANCA in a population of Chinese patients with tuberculosis, which may lead to the misdiagnosis of vasculitic disorders.
METHODS: The study was conducted from January 2016 to May 2017 to evaluate the presence of ANCA in 103 Chinese patients using indirect immunofluorescent assay. An enzyme-linked immunosorbent assay was performed for anti-myeloperoxidase (MPO) and anti-proteinase 3 (PR3) detection.
RESULTS: Perinuclear ANCA (p-ANCA) was detected in 4.8% (5/103) of patients, whereas cytoplasmic ANCA (c-ANCA) was not detected; 1.9% (2/103) of patients with tuberculosis was positive for anti-MPO antibodies, and none had anti-PR3 antibodies. Both anti-MPO-positive patients were diagnosed with ANCA-associated vasculitides.
CONCLUSIONS: ANCA positivity may be more related to vasculitis and immunological disorders than to a M. tuberculosis infection. Therefore, to improve diagnostic accuracy, patients with M. tuberculosis who are ANCA positive should be investigated for concurrent diseases, including the effects of drugs. Therefore, even in tuberculosis epidemic area, ANCA seropositivity, detected by ELISA, is still more suggestive of ANCA-associated vasculitides.

PMID: 30133630 [PubMed - indexed for MEDLINE]

Socio-economic inequalities in occurrence and health care costs in rheumatic and musculoskeletal diseases: results from a Spanish population-based study including 1.9 million persons.

Vascularites - sam, 22/09/2018 - 06:14
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Socio-economic inequalities in occurrence and health care costs in rheumatic and musculoskeletal diseases: results from a Spanish population-based study including 1.9 million persons.

Clin Exp Rheumatol. 2018 Jul-Aug;36(4):589-594

Authors: Putrik P, Ramiro S, Orueta JF, Keszei A, Alonso Moran E, Nuño Solinis R, Boonen A

Abstract
OBJECTIVES: To explore and compare the impact of socio-economic deprivation on the occurrence of the major rheumatic and musculoskeletal diseases (RMDs) and health care costs.
METHODS: Data on diagnoses, socio-demographics and health care costs of the entire adult population of the Basque Country (Spain) was used. Area deprivation index included five categories (1 to 5 (most deprived)). Cost categories included primary and specialist care, emergency room, hospitalisations, and drug prescriptions. Twenty-nine RMDs were grouped into seven groups: Rheumatoid Arthritis, Spondyloarthritis, Crystal Arthropathies, Osteoarthritis, Soft Tissue Diseases, Connective Tissue Diseases, and Vasculitis. The relations between the deprivation and the occurrence of RMD and costs were explored in regression models adjusted for relevant confounders.
RESULTS: Data from 1,923,156 adults were analysed. Mean age was 49.9 (SD18.4) years, 49% were males. Soft tissue diseases were the most prevalent RMD (5.5%, n=105,656), followed by osteoarthritis (2.2%, n=41,924). Socio-economic deprivation was associated with higher likelihood to have any of the 29 RMDs. The strongest socio-economic gradient was seen for the soft tissue diseases (OR 1.82 [95%CI 1.78;1.85], most vs. least deprived), followed by osteoarthritis (OR 1.59 [1.54;1.64]). Deprivation was also associated with higher costs across the majority of the conditions however patterns were more blurred, and inverse relationship was observed for connective tissue diseases, gout, hip osteoarthritis and undifferentiated (poly)arthritis.
CONCLUSIONS: Socio-economic deprivation is associated with increased occurrence of all RMDs, and in most cases more deprived patients incur higher health care costs.

PMID: 29465359 [PubMed - indexed for MEDLINE]

Hypoalbuminaemia in antineutrophil cytoplasmic antibody-associated vasculitis: incidence and significance.

Vascularites - sam, 22/09/2018 - 06:14
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Hypoalbuminaemia in antineutrophil cytoplasmic antibody-associated vasculitis: incidence and significance.

Clin Exp Rheumatol. 2018 Jul-Aug;36(4):603-611

Authors: Xu PC, Tong ZY, Chen T, Gao S, Hu SY, Yang XW, Yan TK, Lin S

Abstract
OBJECTIVES: Hypoalbuminaemia has been proved to be a biomarker of poor prognosis in many diseases. The objective of this study was to investigate the significance of hypoalbuminaemia in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
METHODS: Data of 117 AAV patients were analysed retrospectively. The relationship between hypoalbuminaemia and disease severity were studied. The influence of albumin on the pathogenetic role of ANCA was investigated in vitro.
RESULTS: Among all patients, 52 had light hypoalbuminaemia (30g/L<=albumin<35g/L) and 40 had nephrotic hypoalbuminaemia (albumin <30g/L). Patients with hypoalbuminaemia had higher inflammation levels and more severe kidney injury than patients without hypoalbuminaemia, but no significant difference of the urinary protein levels were found between patients with nephrotic and light hypoalbuminaemia. Multivariate analysis showed serum albumin correlated with age (r=-0.566, p=0.018), C-reactive protein (r=-0.521, p=0.032) and haemoglobin (r=0.512, p=0.036). Patients with nephrotic hypoalbuminaemia had higher incidence of infection, end stage renal disease and all cause mortality during treatment than patients with light hypoalbuminaemia or normal serum albumin. In vitro study indicated albumin could inhibit the binding between ANCA and neutrophils in a concentration dependent manner. Albumin also inhibited the ANCA-induced respiratory burst and neutrophil extracellular traps formation.
CONCLUSIONS: Serum albumin have an inhibitory effect on the binding between ANCA and its antigen. The incidence of hypoalbuminaemia in AAV with kidney involvement is high but is not caused by heavy proteinuria. Hypoalbuminaemia is correlated with the high inflammation level and poor prognosis of AAV. Therapy targeting hypoalbuminaemia might benefit patients with AAV.

PMID: 29352844 [PubMed - indexed for MEDLINE]

Ulcerative colitis in an adult patient mimicking Henoch-Schönlein purpura: A case report.

Vascularites - mer, 19/09/2018 - 03:30
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Ulcerative colitis in an adult patient mimicking Henoch-Schönlein purpura: A case report.

Medicine (Baltimore). 2018 Aug;97(35):e12036

Authors: Lu B, Niu LL, Xu XG, Yao SL, Tan XY

Abstract
RATIONALE: Ulcerative colitis (UC) is one of the chronic inflammatory diseases of the intestinal tract. UC being misdiagnosed as Henoch-Schönlein purpura (HSP) in the elderly has seldom been reported about.
PATIENT CONCERNS: A 64-year-old man was admitted to the hospital with petechiae and palpable purpura in lower limbs and abdominal pain for about 1 month.
DIAGNOSES: Colonoscopy demonstrated severe inflammation in the colon, mucosal congestion, and edema, and multiple hemorrhages and ulcerations, with purulent adhesions. A histopathologic examination of the colon biopsies revealed extensive infiltration of immune cells and mucosal ulcerations in the intestine. UC was diagnosed.
INTERVENTIONS: The patient was treated with prednisone (1.0 mg/kg/d) with progressive dose reduction.
OUTCOMES: The skin lesions were healed within 4 weeks, and his abdominal pain was alleviated remarkably. He is currently under follow-up.
LESSONS: As the treatment used for patients with HSP was not effective, it was advised that UC should be taken into consideration.

PMID: 30170414 [PubMed - indexed for MEDLINE]

Rheumatoid Arthritis-Associated Orbital Vasculitis.

Vascularites - mer, 19/09/2018 - 03:30
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Rheumatoid Arthritis-Associated Orbital Vasculitis.

Ophthalmic Plast Reconstr Surg. 2018 Sep/Oct;34(5):e164-e166

Authors: Weber AC, Shetlar DJ, Yen MT

Abstract
A patient with a history of rheumatoid arthritis presented with bilateral orbital lesions while on abatacept. Biopsy of the lesions yielded pathologic assessment showing vasculitis with a mixed inflammatory infiltrate notable for numerous eosinophils. Despite initial improvement on cyclophosphamide therapy, she developed progression of the orbital lesions and lesions elsewhere on her body. All serologic studies were normal except for increased levels of rheumatoid factor and eosinophilia. The patient underwent a second biopsy, which was consistent with her initial pathology. The patient was started on high-dose mycophenolate mofetil treatment with a good response. After 1 year of treatment, the patient's lesions have nearly completely resolved. This patient illustrates the diagnostic and therapeutic challenges encountered when treating orbital vasculitis. Furthermore, this case shows the importance of not categorizing vasculitis with unclear etiology under the umbrella diagnosis of atypical granulomatosis with polyangiitis. This patient did not exhibit the serologic or pathologic characteristics of granulomatosis with polyangiitis, and she did not respond to treatment as would be expected for granulomatosis with polyangiitis. Maintaining an open approach to diagnosis and treatment was crucial in obtaining a favorable outcome for this patient.

PMID: 30119075 [PubMed - indexed for MEDLINE]

Comparative effectiveness of 18F-FDG PET-CT and contrast-enhanced CT in the diagnosis of suspected large-vessel vasculitis.

Vascularites - dim, 16/09/2018 - 03:07
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Comparative effectiveness of 18F-FDG PET-CT and contrast-enhanced CT in the diagnosis of suspected large-vessel vasculitis.

Br J Radiol. 2018 Sep;91(1089):20180247

Authors: Vaidyanathan S, Chattopadhyay A, Mackie SL, Scarsbrook AF

Abstract
OBJECTIVE: Large-vessel vasculitis (LVV) is a serious illness with potentially life-threatening consequences. (18Fluorine) fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) has emerged as a valuable diagnostic tool in suspected LVV, combining the strengths of functional and structural imaging. This study aimed to compare the accuracy of FDG PET-CT and contrast-enhanced CT (CECT) in the evaluation of patients with LVV.
METHODS: A retrospective database review for LVV patients undergoing CECT and PET-CT between 2011 to 2016 yielded demographics, scan interval and vasculitis type. Qualitative and quantitative PET-CT analyses included aorta:liver FDG uptake, bespoke FDG uptake distribution scores and vascular maximum standardised uptake values (SUVmax). Quantitative CECT data were assessed for wall thickness and mural-lumen ratio. Receiver operating characteristics (ROC) curves were constructed to evaluate comparative diagnostic accuracy and a correlational analysis was conducted between SUVmax and wall thickness.
RESULTS: 36 adults (17 LVV, 19 controls) with a mean age (range) 63 (38-89) years, of which 17 (47%) were males were included. Time interval between CT and PET was mean [standard deviation (SD)] 1.9 (1.2) months. Both SUVmax and wall thickness demonstrated a significant difference between LVV and controls, with a mean difference [95%confidence interval (CI)] for SUVmax 1.6 (1.1, 2.0) and wall thickness 1.25 (0.68, 1.83) mm, respectively. These two parameters were significantly correlated (p < 0.0001, R = 0.62). The area under the curve (AUC) (95% CI) for SUVmax was 0.95 (0.88-1.00), and for mural thickening was 0.83 (0.66-0.99).
CONCLUSION: FDG PET-CT demonstrated excellent accuracy whilst CECT mural thickening showed good accuracy in the diagnosis of LVV. Both parameters showed a highly significant correlation. In hospitals without access to FDG PET-CT or in patients unsuitable for PET-CT (e.g. uncontrolled diabetes) CECT offers a viable alternative for the assessment of LVV. Advances in knowledge: FDG PET-CT is a highly accurate test for the diagnosis of LVV. Aorta:liver SUVmax ratio is the most specific parameter for LVV. In hospitals without PET-CT or in unsuitable patients e.g. diabetics, CECT is a viable alternative.

PMID: 29927635 [PubMed - indexed for MEDLINE]

Chronic Abdominal Pain in a 47-Year-Old Woman.

Vascularites - dim, 16/09/2018 - 03:07
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Chronic Abdominal Pain in a 47-Year-Old Woman.

Gastroenterology. 2018 Sep;155(3):627-628

Authors: Anderson B, Sweetser S

PMID: 29913135 [PubMed - indexed for MEDLINE]

An Unusual Cause of Skin Rash in Crohn's Disease.

Vascularites - dim, 16/09/2018 - 03:07
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An Unusual Cause of Skin Rash in Crohn's Disease.

Gastroenterology. 2018 Sep;155(3):618-620

Authors: Chebli JMF, de Oliveira Moreira B, da Rocha Ribeiro TC

PMID: 29409941 [PubMed - indexed for MEDLINE]

Senile Lemierre syndrome complicated with descending necrotizing mediastinitis: A case report.

Vascularites - lun, 10/09/2018 - 00:55
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Senile Lemierre syndrome complicated with descending necrotizing mediastinitis: A case report.

Medicine (Baltimore). 2018 Aug;97(35):e11903

Authors: Yang X, Yang YF, Zhu ZC, Xu TS, Cheng YN, Sun ZY

Abstract
RATIONALE: Senile patients with LS complicated with DNM are rarely seen in clinical practice, and extensive cervical incision and drainage plus administration of effective antibiotics are the basis for treatment. Currently, the treatment controversy mainly has focused on whether mediastinal incision and drainage is necessary for patients with type I DNM, and whether anticoagulation therapy is required for jugular venous emboli and distant metastatic emboli induced by LS.
PATIENT CONCERNS: A female, 76 years old, developed pain of tonsil on right side 5 days ago, and felt that the pain aggravated complicated with dysphagia and swelling pain of neck on both sides since then.
DIAGNOSES: She was diagnosed with LS complicated with type I DNM.
INTERVENTIONS: Tazobactam and Piperacillin 4.5 q8h and Ornidazole 100 ml q6h ivgtt were administered empirically,and secondary extensive cervical incision and drainage was performed under general anesthesia, after which low molecular weight heparin 4250 U q12h SC was administered. G test was performed 3 days later, which showed (1,3)-β-D-glucan >1000 pg/ml. Bridging anticoagulation therapy, low molecular weight heparin 4250 U q12h SC, and Warfarin 2.5 mg qd po were given one week later. Low molecular weight heparin SC was discontinued and only Warfarin po was administered after treatment of bridging therapy for 3 days.
OUTCOMES: CT of head and neck was reexamined on post-admission d24 and revealed that neck infection was improved on both sides, jugular vein distension on right side was restored to normal, abscess and pneumatosis of superior mediastinum were improved, distension of pulmonary artery on both sides was normalized, WBC was 9.94×109/L, neutrophil count was 4.43×109/L, CRP level was 9.8mg/L, D-D level was 0.81mg/L, PCT level was 0.800ng/mL and G test suggested (1,3)-β-D-glucan pf 27.1 pg/mL.
LESSONS: Concomitant use of anticoagulants on the basis of repeated cervical incision and drainage + administration of effective antibiotics can obtain excellent therapeutic efficacy in the treatment of patient with LS complicated with type I DNM.

PMID: 30170383 [PubMed - indexed for MEDLINE]

Secondary Coats' response to retinal vasculitis managed with bevacizumab.

Vascularites - ven, 07/09/2018 - 00:41
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Secondary Coats' response to retinal vasculitis managed with bevacizumab.

Indian J Ophthalmol. 2018 09;66(9):1355-1357

Authors: Chaudhary P, Varshney A, Videkar C

Abstract
We report a case of 22-year-old young male who presented with a 3-month history of gradual and painless decrease of vision in his right eye (RE). On ophthalmological examination, best-corrected visual acuity in his RE was counting finger close to the face. Left eye ophthalmic examination was unremarkable. RE indirect ophthalmoscopy revealed multiple telangiectatic vessels, aneurysmal dilations, extensive yellow (lipid) exudation over the posterior pole along with perivascular exudation, and multiple sclerosed vessels in peripheral retina. Fluorescein angiography also revealed progressive leakage from telangiectasias, multiple aneurysmal outpouchings, extensive capillary dropouts, and vascular communicating channels in all quadrants. Spectral-domain-optical coherence tomography of macula demonstrated marked intraretinal fluid. Based on the above findings, a diagnosis of healed retinal vasculitis with Coats'-like response was made. The patient received 4-weekly two intravitreal bevacizumab injections which resulted in stabilization of the retinal findings and improvement in visual acuity.

PMID: 30127171 [PubMed - indexed for MEDLINE]

C-Reactive Protein to Serum Albumin Ratio Is an Independent Predictor of All-Cause Mortality in Patients with ANCA-Associated Vasculitis.

Vascularites - lun, 03/09/2018 - 23:22
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C-Reactive Protein to Serum Albumin Ratio Is an Independent Predictor of All-Cause Mortality in Patients with ANCA-Associated Vasculitis.

Yonsei Med J. 2018 Sep;59(7):865-871

Authors: Moon JS, Ahn SS, Park YB, Lee SK, Lee SW

Abstract
PURPOSE: We investigated whether C-reactive protein (CRP) to serum albumin ratio (CAR) could be an independent predictor of all-cause mortality in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
MATERIALS AND METHODS: We retrospectively reviewed the medical records of 170 patients with AAV. We collected clinical and laboratory data. We also examined AAV-related and traditional risk factors of all-cause mortality. To assess the hazard ratios of variables, we performed univariable and multivariable Cox hazard model analyses.
RESULTS: The mean age was 55.0 years and 53 patients (31.2%) were male among 170 patients with AAV (88 microscopic polyangiitis, 43 granulomatosis with polyangiitis, and 39 eosinophilic granulomatosis with polyangiitis). ANCA was detected in 129 patients (75.9%). The initial mean CRP and serum albumin were 41.1 (mg/L) and 3.6 (g/dL), and the mean CAR at diagnosis was 14.8. The most common risk factor of mortality was hypertension (42.4%), followed by chronic kidney disease ≥stage 3 (25.9%). Fourteen patients (8.2%) died during the mean follow-up of 56.7 months. In both multivariable Cox hazard model analyses, CAR at diagnosis was identified as an independent predictor of all-cause of mortality comparable to diabetes mellitus (DM). Moreover, patients with CAR ≥10.35 and having DM exhibited a higher frequency of all-cause mortality than those without.
CONCLUSION: CAR at diagnosis can be an independent predictor of all-cause mortality, comparable to DM, the conventional risk factor of mortality.

PMID: 30091320 [PubMed - indexed for MEDLINE]

Phlebitis-related peripheral venous catheterization and the associated risk factors.

Vascularites - lun, 03/09/2018 - 23:22
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Phlebitis-related peripheral venous catheterization and the associated risk factors.

Niger J Clin Pract. 2018 Jul;21(7):827-831

Authors: Atay S, Sen S, Cukurlu D

Abstract
Background and Aim: Development of phlebitis is a painful and common complication in the application of peripheral intravenous catheter (PIC). This is a prospective observational study performed to identify development rate of phlebitis in application of PIC and the factors that affect the development of phlebitis.
Materials and Methods: The study universe comprises of catheters applied on inpatients in the internal diseases clinic of a state hospital, and the sample comprises of catheters eligible to be included in the study. Five hundred and thirty-two PICs applied on a total of 317 patients were reviewed. The patient identification form, information form for peripheral venous catheter and treatment, and visual infusion phlebitis (VIP) assessment scale were used to collect data.
Results: 31.8% had phlebitis and a large number of them (79.2%) were Level I phlebitis. There was a significant relationship between having a chronic disease, duration of catheterization and type of fluid used and the development rate of phlebitis.
Conclusion: The phlebitis in individuals receiving intravenous (IV) treatment was higher than the rate defined by both the centers for disease control and prevention and IV nurses society. It may be recommended to assess phlebitis by VIP assessment scale and to take preventive measures specifically for development of phlebitis.

PMID: 29984711 [PubMed - indexed for MEDLINE]

Clinical features of different orbital manifestations of granulomatosis with polyangiitis.

Vascularites - lun, 03/09/2018 - 23:22
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Clinical features of different orbital manifestations of granulomatosis with polyangiitis.

Graefes Arch Clin Exp Ophthalmol. 2018 Sep;256(9):1751-1756

Authors: Ismailova DS, Abramova JV, Novikov PI, Grusha YO

Abstract
PURPOSE: The aim of this study was to distinguish different forms of orbital involvement in granulomatosis with polyangiitis (GPA) and to compare their clinical course and outcomes.
METHODS: Two hundred twenty-six consecutive patients with GPA were retrospectively studied. All patients fulfilled the classification criteria of American College of Rheumatology; diagnosis of localized GPA was established according to Chapel Hill Consensus. Of those, 74 patients with orbital disease were enrolled into further investigation of different forms of orbital disease.
RESULTS: Overall ocular involvement was diagnosed in 50% (113/226) of patients; in 5.3% (12/226), inflammatory eye disease resulted in permanent visual loss. Most common ophthalmic manifestations were orbital masses, episcleritis/conjunctivitis, and scleritis (74/226, 32/226, and 12/226, respectively). Seventy-four patients with orbital involvement were divided into three groups: those with orbital mass without primary lacrimal gland involvement (1st group, 45 patients), those with lacrimal gland involvement (2nd group, 26 patients), and those with extraocular myositis (3rd group, 3 patients). Symptoms such as orbital pain, decreased vision, double vision, red eye, scleritis, orbital wall destruction, primary gaze strabismus, and ocular motility restriction were significantly more common in the 1st group. Peripheral ulcerative keratitis (PUK) was present in the 1st group only. Patients of the 1st group suffered from visual loss, double vision, primary gaze strabismus, and ocular motility restriction significantly more often. Eleven of them developed visual loss; 5 of them underwent enucleation, whereas in the group of patients with dacryoadenitis, only 1 patient had BCVA less than 0.3.
CONCLUSIONS: Three types of orbital disease in GPA were proposed: orbital mass without primary lacrimal gland involvement, dacryoadenitis, and extraocular myositis. Patients with orbital mass without lacrimal gland involvement are characterized with a higher rate of systemic disease, severe clinical course not uncommonly associated with other ophthalmic manifestations (necrotizing scleritis, PUK, orbital walls destruction), relatively unfavorable outcome with high level of morbidity (optic nerve atrophy, anophthalmos, strabismus), and higher rate of recurrences. Dacryoadenitis in GPA showed to be not severe manifestation with favorable prognosis.

PMID: 29846785 [PubMed - indexed for MEDLINE]

Erythema elevatum diutinum.

Vascularites - sam, 25/08/2018 - 18:08
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Erythema elevatum diutinum.

An Bras Dermatol. 2018 Jul-Aug;93(4):614-615

Authors: Mançano VS, Dinato SLME, Almeida JRP, Romiti N

PMID: 30066783 [PubMed - indexed for MEDLINE]

Long-Term Outcomes of Patients With HCV-Associated Cryoglobulinemic Vasculitis After Virologic Cure.

Vascularites - jeu, 16/08/2018 - 15:15
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Long-Term Outcomes of Patients With HCV-Associated Cryoglobulinemic Vasculitis After Virologic Cure.

Gastroenterology. 2018 08;155(2):311-315.e6

Authors: Bonacci M, Lens S, Mariño Z, Londoño MC, Rodriguez-Tajes S, Sánchez-Tapias JM, Ramos-Casals M, Hernández-Rodríguez J, Forns X

Abstract
Patients with hepatitis C virus-associated cryoglobulinemic vasculitis (HCV-CV) have high rates of clinical remission after treatment with direct-acting antivirals (DAAs), but circulating cryoglobulins persist, and vascular disorders reappear in some patients shortly after DAA treatment ends. We performed a prospective study to assess the long-term clinical and immune system effects of HCV eradication with DAAs in 46 patients with HCV-CV and 42 asymptomatic patients with circulating cryoglobulins. A median of 24 months after DAA treatment (range, 17-41 months), 66% of patients with HCV-CV and 70% of asymptomatic patients with circulating cryoglobulins had an immunologic response, with comparable reductions in cryocrit from 2.6% to 0% (P < .05). However, 20% of patients still had positive test results for cryoglobulins after DAA therapy. Among patients with HCV-CV, 42 (91%) had a clinical response, in that their Birmingham Vasculitis Activity Score (version 3) decreased from 7 to 0 (P < .01). Nevertheless, within 2 years after a sustained viral response to DAA therapy, 5 patients with HCV-CV (11%, 4 with cirrhosis) had relapses of vasculitis that included severe organ damage and death.

PMID: 29705529 [PubMed - indexed for MEDLINE]

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