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HIV-associated vasculitis. Part II: histologic and angiographic diagnostic reconfirmation after an uncontrolled HIV infection and fatal outcome.

Vascularites - mer, 19/06/2019 - 10:01
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HIV-associated vasculitis. Part II: histologic and angiographic diagnostic reconfirmation after an uncontrolled HIV infection and fatal outcome.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):151-152

Authors: Laurent C, Marçal AL, Prieto-González S, Balagué O, Morales X, Darnell A, Ripoll E, Cid MC, Hernández-Rodríguez J

PMID: 31074723 [PubMed - indexed for MEDLINE]

Comparison of Radiological and Histological Findings of Lung Parenchyma in Patients with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Vascularites - mer, 19/06/2019 - 10:01
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Comparison of Radiological and Histological Findings of Lung Parenchyma in Patients with Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Yonsei Med J. 2019 May;60(5):454-460

Authors: Park HJ, Jung SM, Song JJ, Park YB, Song JS, Lee SW

Abstract
PURPOSE: The present study investigated chest computed tomography (CT) patterns and lung histological features, as well as the consistency between radiological and histological features among patients with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic GPA (EGPA).
MATERIALS AND METHODS: The medical records of 74 antineutrophil cytoplasmic antibody-associated vasculitis (AAV) patients with radiological lung parenchymal lesions were reviewed along with the histological results for 28 of them. Chest CT patterns were divided according 12 items mostly suggested by radiologists and histological features were divided according to necrotising granuloma, necrotising vasculitis, eosinophilic infiltration, and hemosiderin laden macrophages as defined by a pathologist.
RESULTS: The mean age was 57.1 years (22 men). The most common clinical manifestation other than lung manifestation was renal manifestation (62.2%), and the most common chest CT pattern was lung involvement of vasculitis (35.1%). In MPA patients, the major histological features were hemosiderin-laden macrophages in the alveolar space and vasculitis. In GPA patients, the major histological features were necrotizing vasculitis and necrotizing granuloma, while in EGPA patients, the major histological feature was only necrotising vasculitis. The consistency rate in GPA patients was the highest (100%), followed by that in MPA patients (66.7%) and EGPA patients (50.0%).
CONCLUSION: When lung involvement of AAV is suspected on chest CT, lung biopsy should be recommended for the proper classification of AAV, due to the discordance rate between radiological and histological findings in MPA and EGPA patients, but not GPA patients.

PMID: 31016907 [PubMed - indexed for MEDLINE]

Erythrocytes restrict microvesicle-induced production of reactive oxygen species by polymorphonuclear leukocytes.

Vascularites - mer, 19/06/2019 - 10:01
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Erythrocytes restrict microvesicle-induced production of reactive oxygen species by polymorphonuclear leukocytes.

APMIS. 2019 Jul;127(7):538-542

Authors: Winberg LK, Rasmussen NS, Nielsen CH, Jacobsen S

Abstract
Microvesicles (MVs) are extracellular vesicles released by several cell types upon activation or apoptosis. MVs have the potential to activate complement, which has been suggested to mediate their clearance. However, it is not clear how complement-opsonized MVs are prevented from activating circulating polymorphonuclear leukocytes (PMNs) with release of reactive oxygen species (ROS) and potential damage of endothelium and other bystander cells as consequence. We hypothesized that binding of opsonized MVs to erythrocytes (Es) attenuates MV-induced PMN activation. To test this, normal PMNs were exposed to MVs in the presence and absence of Es from allogenic healthy donors. As analyzed by flow cytometry, the presence of Es restricted the PMN binding of MVs by about 85% (p = 0.002) and mediated a 60-70% inhibition of the PMN production of the ROS H2 O2 , induced by MVs, when lipopolysaccharide was used as a primer (p = 0.002). The competitive binding of MVs to Es was partly dependent on complement, since EDTA inhibited MV binding to Es by 75%. These data suggest that Es, through competitive binding, may restrict MV-induced activation of circulating PMNs and thereby serve a role as a regulator of PMN activation.

PMID: 31009117 [PubMed - indexed for MEDLINE]

G-CSF-induced aortitis: Two cases and review of the literature.

Vascularites - mer, 19/06/2019 - 10:01
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G-CSF-induced aortitis: Two cases and review of the literature.

Autoimmun Rev. 2019 Jun;18(6):615-620

Authors: Parodis I, Dani L, Notarnicola A, Martenhed G, Fernström P, Matikas A, Wiklander OPB

Abstract
BACKGROUND: Febrile neutropenia is generally recognised as a complication of myelosuppressive chemotherapy. Recombinant human granulocyte colony stimulating factor (G-CSF) is commonly used as a primary or secondary prophylaxis to reduce the degree and duration of neutropenia in patients at risk of developing chemotherapy-induced neutropenic fever and infectious complications. G-CSF is known to decrease mortality and increase the possibility of maintaining adequate chemotherapy dose intensity and density, which is essential in curable malignancies. Common side effects are generally mild. However, potentially fatal adverse events have also been reported.
CASE PRESENTATION: Herein, we summarise previously reported and report two new independent cases of G-CSF-induced aortitis, both in patients treated with chemotherapy for breast cancer. The two cases, identified only a few months apart, share several common characteristics including type of cancer, gender, age, chemotherapy, G-CSF treatment regimen, and time span from G-CSF initiation to aortitis manifestation. The two cases were both diagnosed by CT scan and successfully treated with corticosteroids along with discontinuation of G-CSF.
CONCLUSION: This case report highlights that although aortitis is a rare adverse event of G-CSF treatment, it should be considered in cases of unexplained fever and/or clinical and laboratory findings that do not respond to antibiotics.

PMID: 30959218 [PubMed - indexed for MEDLINE]

Giant Cell Arteritis with Generalized Granuloma Annulare.

Vascularites - mer, 19/06/2019 - 10:01
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Giant Cell Arteritis with Generalized Granuloma Annulare.

Intern Med. 2019 Apr 15;58(8):1173-1177

Authors: Torisu Y, Horai Y, Michitsuji T, Kawahara C, Mori T, Iwanaga N, Izumi Y, Kawakami A

Abstract
We report the case of an 80-year-old man with generalized granuloma annulare (GGA) who subsequently developed giant cell arteritis (GCA). Steroid treatment was effective for both diseases in this case. Although cases of concomitant GGA and GCA have rarely been reported, previous studies suggest that common histological characteristics underlie the two diseases. It is therefore necessary to recognize that GGA can be complicated by GCA, particularly when typical symptoms, such as headache and visual disturbance, are present.

PMID: 30568109 [PubMed - indexed for MEDLINE]

Abdominal adipose tissue predicts major cardiovascular events in systemic necrotising vasculitides.

Vascularites - dim, 16/06/2019 - 07:23
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Abdominal adipose tissue predicts major cardiovascular events in systemic necrotising vasculitides.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):130-136

Authors: Briot K, Dunogué B, Henriquez S, Etcheto A, Kolta S, Régent A, Cohen P, Berezne A, Le Jeunne C, Mouthon L, Roux C, Guillevin L, Terrier B, French Vasculitis Study Group (FVSG)

Abstract
OBJECTIVES: Cardiovascular (CV) events are highly prevalent in systemic necrotising vasculitides (SNV). Visceral/subcutaneous adipose tissue (VAT/SAT) ratio has been shown to be associated with CV events in various diseases. We aimed to assess the relevance of abdominal adipose tissue measurement to predict major CV events (MCVEs) in SNV.
METHODS: Patients with SNV were successively included in a longitudinal study assessing MCVEs and other sequelae. Dual x-ray absorptiometry was performed to evaluate abdominal adipose tissue. Patients were prospectively followed for MCVEs, defined as myocardial infarction, unstable angina, stroke, arterial revascularisation and/or hospitalisation for or death from CV causes.
RESULTS: One hundred and twenty consecutive SNV patients were included and analysed (54 males, mean age 53±18 years). High CV risk was found in 28 (23.3%) patients. In univariate analysis, age, male gender, VDI, VAT/SAT ratio and serum troponin level were significantly associated with high CV risk, whereas age and VAT/SAT ratio remained independently associated with high CV risk. Variables associated with high tertile of VAT/SAT ratio included age and metabolic risk factors. After median follow-up of 42 months, 19 (16%) patients experienced MCVEs. Hazard ratios for incident MCVEs compared with 1st tertile of VAT/SAT ratio were 7.22 (1.02-51.3; p=0.048) and 9.90 (3.15-31.2; p=0.0002) in the 2nd and 3rd tertile, respectively.
CONCLUSIONS: Abdominal visceral adipose tissue is a reliable surrogate marker of CV risk and predicts incident MCVEs in SNV patients. Abdominal adipose tissue should be probably evaluated routinely in these patients to assess CV risk.

PMID: 31162033 [PubMed - indexed for MEDLINE]

Cytokine expression in temporal arteries: comparative analysis between patients with biopsy-positive giant cell arteritis, biopsy-negative giant cell arteritis and biopsy-negative without arteritis.

Vascularites - dim, 16/06/2019 - 07:23
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Cytokine expression in temporal arteries: comparative analysis between patients with biopsy-positive giant cell arteritis, biopsy-negative giant cell arteritis and biopsy-negative without arteritis.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):122-129

Authors: Grossman C, Yassin N, Avivi C, Bornstein G, Ben-Zvi I, Barshack I

Abstract
OBJECTIVES: To investigate whether expression of pro-inflammatory cytokines in the temporal artery may aid in differentiating biopsy-negative giant cell arteritis (GCA) patients from those with a negative biopsy without arteritis.
METHODS: We investigated cytokine expression in temporal artery biopsy (TAB) of 54 consecutive patients: 17 with biopsy-positive GCA, 17 with biopsy-negative GCA, and 20 biopsy-negative without arteritis. We compared the expression rate of the following cytokines among these 3 groups of patients: interleukin-6 (IL-6), osteopontin (OPN), COX-2, and TNF-α.
RESULTS: IL-6 was expressed in 13 (76%) patients with biopsy-positive GCA, 0 patients in biopsy-negative GCA, and 1(5%) patient with biopsy-negative without arteritis (p<0.05). OPN was expressed in 17 (100%) patients with biopsy-positive GCA, 2 (12%) patients with biopsy-negative GCA, and 0 patients with biopsy-negative without arteritis (p<0.05). Cox-2 was expressed in 16 (94%) patients with biopsy-positive GCA, 0 patients with biopsy-negative GCA, and 3 (15%) patients with biopsy-negative without arteritis (p<0.05). TNF- α was expressed in 17 (100%) patients with biopsy-positive GCA, 14 (82%) patients with biopsy-negative GCA, and 8 (40%) patients with biopsy-negative without arteritis (p<0.05).
CONCLUSIONS: IL-6, COX-2 and OPN are significantly more expressed in the presence of a positive TAB compared to a negative TAB. TNF-α is significantly more expressed in GCA patients compared to non-GCA patients. Thus, TNF-α expression may suggest a diagnosis of GCA despite a negative TAB. Further larger studies are needed to confirm these findings.

PMID: 31162032 [PubMed - indexed for MEDLINE]

Cost-effectiveness of rituximab versus azathioprine for maintenance treatment in antineutrophil cytoplasmic antibody-associated vasculitis.

Vascularites - dim, 16/06/2019 - 07:23
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Cost-effectiveness of rituximab versus azathioprine for maintenance treatment in antineutrophil cytoplasmic antibody-associated vasculitis.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):137-143

Authors: Montante A, Le Bras A, Pagnoux C, Perrodeau E, Ravaud P, Terrier B, Guillevin L, Durand-Zaleski I, French Vasculitis Study Group

Abstract
OBJECTIVES: Rituximab was proven superior to azathioprine for maintenance treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The high cost of rituximab might, however, limit its routine use. This study determined the cost-effectiveness of intravenous rituximab (5 x 500 mg until month 18), versus oral azathioprine (2 mg/kg per day, gradually decreased between month 12 and 22), for maintenance treatment of patients with granulomatosis with polyangiitis, microscopic polyangiitis, or renal-limited vasculitis, aged 18-75.
METHODS: We performed a single-trial based economic evaluation. MAINRITSAN was a 28-month multicentre, prospective, randomised, controlled open-label trial. We estimated the cost of healthcare resources and quality of life using prospectively collected data. Healthcare costs were estimated from the perspective of the French Social Health Insurance's perspective, using 2016 tariffs for reimbursement. Utilities were derived from Short Form 36 scores. We estimated total average cost, incremental cost per incremental relapse averted and per quality-adjusted life-year (QALY) gained. Sensitivity analyses were performed to assess uncertainty over relapses, severe adverse events, discount rate, utility weights, time horizon and the cost of rituximab. Costs drivers were tested using a generalised linear model.
RESULTS: Total average costs were €13,387 (€11,605-€15,646) and €10,217 (€7,567-12,949) in the rituximab and azathioprine groups respectively. The incremental cost-effectiveness ratio (ICER) was €12,824 per relapse averted and the incremental cost-utility ratio (ICUR) €37,782 per QALY gained. Besides the unit cost of rituximab, the major cost drivers were relapses and severe adverse events.
CONCLUSIONS: Maintenance treatment by rituximab could be cost-effective for preventing relapses in patients with AAV.

PMID: 31162031 [PubMed - indexed for MEDLINE]

The different clinical patterns of giant cell arteritis.

Vascularites - dim, 16/06/2019 - 07:23
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The different clinical patterns of giant cell arteritis.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):57-60

Authors: de Boysson H, Liozon E, Ly KH, Dumont A, Delmas C, Aouba A

Abstract
OBJECTIVES: To estimate the frequency of different clinical patterns in giant-cell arteritis (GCA) at onset.
METHODS: All GCA patients consecutively followed-up in two referral centers for GCA with a biopsy-proven diagnosis and/or large-vessel vasculitis (LVV) demonstrated on imaging were analysed.
RESULTS: We analysed the initial clinical presentation of 693 patients with a median age of 75 [48-94] years and including 486 (70%) women. We identified four different clinical patterns: isolated cranial GCA (in 80%), symptomatic LVV with or without associated cranial signs (9%), isolated fever or inflammatory response (9%), and isolated polymyalgia rheumatica with vasculitis (2%). A silent LVV was found in 110 (45%) out of the 247 patients without large-vessel symptoms who underwent imaging at GCA diagnosis. Symptomatic LVV patients were more frequently GC-dependent compared to other patterns (p=0.03) and showed the longest treatment duration (median: 37 [15-212] months versus <30 months for other clinical phenotypes; p=0.001).
CONCLUSIONS: This study suggests that 80% of GCA patients display a typical presentation, whereas the other 20% showed rarer presentations. Patients with symptomatic LVV required longer treatment duration.

PMID: 31162029 [PubMed - indexed for MEDLINE]

Ocular ischaemic complications in giant cell arteritis: CHADS2-score predicts risk of permanent visual impairment.

Vascularites - dim, 16/06/2019 - 07:23
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Ocular ischaemic complications in giant cell arteritis: CHADS2-score predicts risk of permanent visual impairment.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):61-64

Authors: Czihal M, Tschaidse J, Bernau C, Lottspeich C, Köhler A, Dechant C, Schulze-Koops H, Hoffmann U, Mackert MJ, Thurau S

Abstract
OBJECTIVES: To identify independent risk factors for permanent visual loss (PVL) in patients with giant cell arteritis (GCA), with a special focus on sonographic findings of the temporal, carotid and subclavian/axillary arteries, and on established scoring systems of ischaemia risk assessment.
METHODS: Consecutive patients with a diagnosis of GCA between 2002 and 2013 were retrospectively identified from a prospectively maintained database. Data on clinical characteristics including ophthalmological findings, laboratory values, and sonographic findings of the temporal, carotid an axillary arteries were extracted. CHADS2- and CHA2DS2-VASc-score were calculated. Clinical, laboratory and sonographic characteristics of patients with and without PVL were compared. Multiple logistic regression models were calculated to identify variables independently associated with PVL.
RESULTS: One-hundred-fifty-two patients were included in the analysis. PVL occurred in 30.2% of patients, with anterior ischaemic optic neuropathy as predominant underlying cause (91.3%). The frequency of PVL was strongly dependent on the age at diagnosis, with a significant increase after the age of 70 years. In multivariate analysis, axillary artery vasculitis with an odds ratio (OR) of 0.3 and constitutional symptoms with an OR of 0.1 were negatively associated with PVL. A CHADS2-score of 1 (OR 10.7) or ≥2 (OR 25) was associated with a significantly increased risk of PVL.
CONCLUSIONS: The risk of PVL secondary to GCA increases with age but is lower in patients presenting with constitutional symptoms and/or exhibiting axillary artery involvement. The CHADS2-score may help to discriminate patients with low vs. high risk of PVL.

PMID: 31162028 [PubMed - indexed for MEDLINE]

Endoscopic and microscopic findings of gastrointestinal tract in Henoch-Schönlein purpura: Single institute experience with review of literature.

Vascularites - dim, 16/06/2019 - 07:23
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Endoscopic and microscopic findings of gastrointestinal tract in Henoch-Schönlein purpura: Single institute experience with review of literature.

Medicine (Baltimore). 2019 May;98(20):e15643

Authors: Han Y, Jin SY, Kim DW, Jeen YM, Kim YH, Choi IH

Abstract
Asia has the highest incidence of Henoch-Schönlein purpura (HSP). Although 50% to 75% of patients with HSP manifest gastrointestinal (GI) symptoms, endoscopic, and pathologic findings of HSP have been rarely reviewed in Asia.Patients diagnosed with HSP who had undergone endoscopic biopsy from GI tract (GIT) in Soonchunhyang University Seoul Hospital from 2000 to 2018 were evaluated and 25 cases with 44 biopsies from upper GI tract (U-GIT) or lower GI tract (L-GIT) were enrolled. Their clinical and endoscopic findings and histologic findings of endoscopic biopsy were reviewed.Of the 25 patients, 15 were males and 10 were females. There were 6 children and 19 adults. The most common GI symptom was abdominal pain (20/25), followed by loose stool or diarrhea (9/25). Biopsied sites included 19 from U-GIT (9 stomach and 10 duodenum) and 25 from L-GIT (7 terminal ileum, 1 cecum, 4 ascending, 1 transverse, 2 descending, 7 sigmoid, and 3 rectum). Erythema/petechia was the most common endoscopic finding in U-GIT, while erosion/ulceration was the most common one in L-GIT. In U-GIT, extravasted red blood cell (RBC) (14/19) was the most common histologic finding, while leukocytoclastic vasculitis (LCV)/capillarities were identified in 7 specimens, including 5 duodenum samples. In endoscopic investigations of L-GIT, erosion/ulceration (9/14) was predominantly identified. The most common histologic finding was also extravasted RBC (22/25), while LCV/capillarities were noted in 10 specimens, including 5 specimens from terminal ileum.The HSP commonly involves GIT. Histologic findings of our cases were not significantly different from results of previous studies in Western countries. However, endoscopic and pathologic characteristics of HSP have been rarely reviewed in Asia. Herein, we share experience of endoscopic biopsy of GIT in patients with HSP.

PMID: 31096484 [PubMed - indexed for MEDLINE]

Phospholipase A2 receptor-associated membranous nephropathy in a patient with IgG4-related disease: A case report.

Vascularites - dim, 16/06/2019 - 07:23
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Phospholipase A2 receptor-associated membranous nephropathy in a patient with IgG4-related disease: A case report.

Medicine (Baltimore). 2019 May;98(20):e15616

Authors: Muhsin SA, Masia R, Smith RN, Wallace ZS, Perugino CA, Stone JH, Niles JL, Cortazar FB

Abstract
RATIONALE: IgG4-related disease (IgG4-RD) is a multiorgan disease of unestablished prevalence that is characterized histopathologically by a dense lymphoplasmacytic infiltrate enriched with IgG4-expressing plasma cells and associated with storiform fibrosis. Tubulointerstitial nephritis (TIN) is the most common renal manifestation of IgG4-RD, but membranous nephropathy (MN) has also been described and often occurs in the context of concurrent TIN. Patients with IgG4-related MN have been characteristically negative for autoantibodies to the phospholipase A2 receptor (PLA2R).
PATIENT CONCERNS: A 45-year-old man presented with abdominal pain and lower extremity edema.
DIAGNOSIS: Histopathological evaluation of pancreas and liver biopsies established a diagnosis of IgG4-RD. Renal biopsy confirmed a diagnosis of PLA2R-associated MN without evidence of concurrent TIN.
INTERVENTIONS: The patient was treated with rituximab, a short course of low-dose, oral cyclophosphamide, and a rapid glucocorticoid taper.
OUTCOMES: The patient achieved remission of MN after 8 months of therapy and maintained remission of IgG4-RD.
LESSONS: PLA2R-associated MN may be a rare manifestation of IgG4-RD. Systematic evaluation of larger cohorts of IgG4-RD patients for the presence of PLA2R autoantibodies and the investigation of PLA2R-associated MN cohorts for evidence of IgG4-RD would facilitate the understanding of the nature of the relationship between these observations.

PMID: 31096469 [PubMed - indexed for MEDLINE]

Association between increased arterial stiffness measured by brachial-ankle pulse wave velocity and cardiovascular events in patients with Takayasu's arteritis.

Vascularites - dim, 16/06/2019 - 07:23
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Association between increased arterial stiffness measured by brachial-ankle pulse wave velocity and cardiovascular events in patients with Takayasu's arteritis.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):65-71

Authors: He Y, Cheng N, Dang A, Lv N

Abstract
OBJECTIVES: This study aims to investigate the association between arterial stiffness measured by brachial-ankle pulse wave velocity (baPWV) and cardiovascular events (CVEs) in patients with Takayasu's arteritis (TAK).
METHODS: A total of 240 TAK patients, who underwent baPWV measurement, were included in the study. The primary outcome was CVEs, which was defined as presently or previously diagnosed with myocardial infarction, unstable angina, congestive heart failure, aortic aneurysm/dissection, cerebral infarction/transient ischaemic attack (TIA), or cerebral haemorrhage.
RESULTS: A total of 74 (30.8%) patients with CVEs were included in the present cohort. Compared with the patients without CVEs, those with CVEs had a higher prevalence of hyperlipidaemia (HL), smoking history, active stage, angiographic type V, renal dysfunction (RDF), higher baPWV and high sensitive C-reactive protein (hs-CRP) level (all, p<0.05). The multivariate logistic regression analysis showed that HL (OR: 2.465, 95%CI: 1.308-4.648, p=0.005), smoking history (OR: 4.764, 95%CI: 1.623-13.985, p=0.004), baPWV (OR: 1.132, 95%CI: 1.063-1.204, p<0.001), and hs-CRP (OR: 1.111, 95%CI: 1.040-1.188, p=0.002) were independently associated with the presence of CVEs. The multiple linear regression analysis revealed that age (β=0.100, p=0.002), mean blood pressure (β=0.071, p<0.001), angiographic type V (β=3.681, p<0.001) and RDF (β=1.800, p=0.048) were independently correlated with baPWV.
CONCLUSIONS: Increased baPWV was independently associated with CVEs in patients with TAK. Age, angiographic type V, mean blood pressure and RDF were the strongest determinants for baPWV in TAK. BaPWV may be a potential maker to predict CVEs in patients with TAK.

PMID: 31074717 [PubMed - indexed for MEDLINE]

Interleukin-6 expression in inflamed and non-inflamed temporal arteries from patients with giant cell arteritis.

Vascularites - dim, 16/06/2019 - 07:23
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Interleukin-6 expression in inflamed and non-inflamed temporal arteries from patients with giant cell arteritis.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):98-103

Authors: Pipitone N, Muratore F, Tamagnini I, Cavazza A, Cimino L, Boiardi L, Restuccia G, Croci S, Bonacini M, Salvarani C

Abstract
OBJECTIVES: To evaluate whether interleukin-6 expression in the temporal arteries could be a more sensitive marker of active inflammation compared to the presence of an inflammatory infiltrate.
METHODS: Sixty-three formalin-fixed, paraffin-embedded temporal artery biopsies performed between 2009 and 2012 from 32 patients with biopsy-proven giant cell arteritis, 8 patients with a negative biopsy but with a final diagnosis of giant cell arteritis, and 23 controls (patients with an initial clinical suspicion of giant cell arteritis in whom an alternative diagnosis subsequently was made) were examined. Biopsy specimens showing a transmural inflammatory infiltrate were considered positive for giant cell arteritis. Immunochemistry was performed to detect interleukin-6 in the temporal artery specimens. Slides of temporal artery biopsies were independently assessed by five readers. Interleukin-6 expression was graded as 0 (absent), 1 (mild), 2 (moderate) and 3 (marked). We considered anti-IL-6 staining positive if staining was of grade 2 or 3.
RESULTS: Temporal artery biopsies specimens from patients with biopsy-proven giant cell arteritis, biopsy-negative giant cell arteritis and controls were positive for anti-interleukin-6 staining in 59%, 13% and 48% of cases, respectively.
CONCLUSIONS: Interleukin-6 expression does not increase the sensitivity of temporal artery biopsy in patients with giant cell arteritis who have morphologically uninflamed arteries.

PMID: 31025922 [PubMed - indexed for MEDLINE]

Study on the association of serum pentraxin-3 and lysosomal-associated membrane protein-2 levels with disease activity in Chinese Takayasu's arteritis patients.

Vascularites - dim, 16/06/2019 - 07:23
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Study on the association of serum pentraxin-3 and lysosomal-associated membrane protein-2 levels with disease activity in Chinese Takayasu's arteritis patients.

Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):109-115

Authors: Chen Z, Hu C, Sun F, Li J, Yang Y, Tian X, Zeng X

Abstract
OBJECTIVES: To evaluate the association between disease activity and serum levels of pentraxin-3 (PTX-3) and lysosomal-associated membrane protein-2 (LAMP-2) as well as the acute reactants in Chinese Takayasu's arteritis (TAK) patients.
METHODS: The serum PTX-3 and LAMP-2 levels were tested in 98 TAK patients and 40 age- and gender-matched healthy controls. The disease activity of these TAK patients was assessed according to the National Institute of Health (NIH) and Abatacept in giant cell arteritis and Takayasu's arteritis (AGATA) criteria, respectively.
RESULTS: Among the 98 TAK patients, 45 and 52 patients had active disease according to the NIH criteria and AGATA criteria respectively. The total agreement rate between these two criteria was 90.82% (κ=0.817, p<0.001). Both serum PTX-3 and LAMP-2 levels were elevated in TAK patients compared with those in healthy controls (PTX- 3: 0.32±0.03 ng/ml vs. 0.18±0.02 ng/ ml, p=0.001; LAMP-2: 4.40±0.14 ng/ ml vs. 3.30±0.20 ng/ml, p<0.001). TAK patients with active disease had higher serum PTX-3 levels compared with those who had inactive disease (NIH criteria: 0.42±0.06 ng/ml vs. 0.25±0.02 ng/ml, p=0.004; AGATA criteria: 0.38±0.05 ng/ml vs. 0.27±0.02 ng/ml, p=0.049). However, serum LAMP-2 levels did not differ between patients with active and inactive disease according to both NIH and AGATA criteria. A cutoff value of PTX-3 with 0.30 ng/ml maximised the ability of disease activity assessment with a sensitivity/specificity of 57.10%/73.10% and 47.90%/71.10% according to the NIH and AGATA criteria, respectively.
CONCLUSIONS: Serum PTX-3 and LAMP- 2 levels are elevated in Chinese TAK patients. However, serum PTX-3 but not LAMP-2 level is associated with active disease.

PMID: 30943132 [PubMed - indexed for MEDLINE]

Rapidly Progressive Intracranial Vasculopathy in Graft Versus Host Disease.

Vascularites - dim, 16/06/2019 - 07:23
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Rapidly Progressive Intracranial Vasculopathy in Graft Versus Host Disease.

J Stroke Cerebrovasc Dis. 2019 Jun;28(6):e73-e74

Authors: Bowen T, Silver SA, Sila C

Abstract
After allogenic hematopoietic stem cell transplantation, cerebrovascular complications are uncommon, occurring in approximately 2%, and typically due to coagulopathy or infection. Graft versus host disease has been rarely reported to affect the central nervous system but these cases typically describe leukoencephalopathy, encephalitis, or perivascular infiltrates or vasculitis with subcortical ischemia or hemorrhage. We report a previously undescribed noninflammatory vasculopathy causing multifocal intracranial arterial occlusions and cerebral infarctions in a man following allogenic hematopoietic stem cell transplantation for chronic lymphocytic leukemia, which we propose to be a central nervous system manifestation of graft versus host disease.

PMID: 30878370 [PubMed - indexed for MEDLINE]

Celiac Artery Vasculitis.

Vascularites - dim, 16/06/2019 - 07:23
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Celiac Artery Vasculitis.

Ann Vasc Surg. 2019 Apr;56:354.e17-354.e19

Authors: Chait J, Pavalonis A, Rajaee S, Hingorani A, Ascher E

Abstract
Isolated celiac artery vasculitis is an uncommon disease rarely reported in the Western literature. In this case report, we describe a 52-year-old Chinese male presenting with abdominal pain who was diagnosed with nonspecific celiac artery vasculitis and was successfully treated with a short course of oral corticosteroids.

PMID: 30500651 [PubMed - indexed for MEDLINE]

An Unusual Case of Bilateral Upper Extremity Ischemia Caused by Forearm Vessel Fibromuscular Dysplasia.

Vascularites - dim, 16/06/2019 - 07:23
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An Unusual Case of Bilateral Upper Extremity Ischemia Caused by Forearm Vessel Fibromuscular Dysplasia.

Ann Vasc Surg. 2019 Apr;56:353.e7-353.e11

Authors: Antonescu I, Knowles M, Wirtz E, Pascarella L

Abstract
Fibromuscular dysplasia (FMD) is a nonatherosclerotic disease that generally affects medium-sized arteries. The distribution typically involves the renal, extracranial carotid/vertebral, and iliac arteries. FMD in other vascular beds is rare. We herein present the case of a 47-year-old female with rapid-onset bilateral digital ischemia. Initial differential diagnosis included vasospastic disorders and vasculitis. An upper extremity arteriogram was suggestive of ulnar and radial FMD. Percutaneous intervention was not successful, and the patient was managed conservatively with symptomatic improvement. This case highlights the important diagnostic and therapeutic considerations in patients with less common etiologies of upper extremity ischemia.

PMID: 30500650 [PubMed - indexed for MEDLINE]

Clinical outcomes of extended endovascular recanalization of 16 consecutive Buerger's disease patients.

Vascularites - dim, 16/06/2019 - 07:23
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Clinical outcomes of extended endovascular recanalization of 16 consecutive Buerger's disease patients.

Vascular. 2019 Jun;27(3):233-241

Authors: Kacmaz F, Kaya A, Keskin M, Keceoglu S, Algin IH, Yilmazkaya B, Ilkay E

Abstract
OBJECTIVE: Buerger's disease is one of the worst diseases causing peripheral artery occlusions (especially lower extremity) with increased morbidity and mortality. Endovascular treatment of the diseased arteries gains preference over bypass surgery nowadays. Here, we aimed to present the clinical outcomes of 16 consecutive Buerger's disease patients underwent extended endovascular recanalization which is a new technique to restore direct blood flow to at least one foot artery, with the performance of angioplasty for each tibial and foot artery obstructions.
METHODS: A total of 16 consecutive patients with confirmed diagnosis of Buerger's disease that percutaneously treated in our center between February 2014 and March 2018 were included in the study. The mean age of the patients was 44.25 ± 4.28 ranging from 36 to 50 years. After physical examination and complementary diagnostic tests, performance of extended angioplasty for occluded arteries was intended to restore direct blood flow to at least one of the blow-the-knee arteries.
RESULTS: A successful extended endovascular treatment was performed in 20 of 22 limbs, achieving a technical success of 91%. All patients were successfully discharged without any complication. Mean follow-up duration was 21.43 ± 7.08 months. Reintervention was performed in one patient and minor amputation was needed in one of the failed limbs. Limb salvage rate was 100%. A significant difference was observed based on Rutherford classification, ankle brachial index, direct blood flow to foot, presence of ulcer and rest pain when compared before and after the intervention.
CONCLUSION: We showed successful extended endovascular recanalization of Buerger's disease patients with a high technical success rate and sustained clinical improvement. Extended endovascular recanalization could be a therapeutic option in Buerger's disease patients, since they are not good candidates for surgery.

PMID: 30305010 [PubMed - indexed for MEDLINE]

Differences in clinical manifestations and prognosis of Chinese giant cell arteritis patients with or without polymyalgia rheumatica.

Vascularites - dim, 16/06/2019 - 07:23
Related Articles

Differences in clinical manifestations and prognosis of Chinese giant cell arteritis patients with or without polymyalgia rheumatica.

Ir J Med Sci. 2019 May;188(2):713-720

Authors: Zhang Y, Wang D, Chu X, Zhang W, Zeng X

Abstract
BACKGROUND: The symptoms of giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) frequently overlap in the elderly. Whether there are differences in clinical features or prognosis between GCA patients with or without PMR remains unknown.
AIMS: To identify differences in clinical manifestation and prognosis between Chinese GCA patients with or without PMR.
METHODS: A retrospective study of patients diagnosed with GCA in Peking Union Medical College Hospital (PUMCH) during the last 20 years was conducted. Clinical data was collected and analyzed accordingly, and follow-up was performed.
RESULTS: A total of 50 patients had PMR, while 41 patients did not, with no significant differences in age, gender, and disease course between the two groups. GCA patients with PMR presented with higher risks of family history of malignancy (p = 0.048). Patients without PMR had higher proportion of hearing loss (p = 0.006), ANCA positive (p = 0.024), and abnormal imaging findings illustrating the involvement of arteries under aortic arch (p = 0.018). Before treatment, total lymphocyte counts in patients without PMR were lower than those with PMR, and monocyte counts in both groups were higher than normal. Acute phase reactants in patients without PMR were higher than the other group. No significant differences were found in prognosis during follow-up.
CONCLUSIONS: GCA patients with or without PMR have different clinical characteristics. Patients with PMR present myalgia or arthralgia more frequently, while those without PMR have higher inflammatory markers, lower lymphocyte counts, and wider involvement of arteries under aortic arch.

PMID: 30251177 [PubMed - indexed for MEDLINE]

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