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Ulnar Artery Occlusion and severity markers of vasculopathy in Systemic sclerosis: a multicenter cross-sectional study.

Actualités En Médecine Interne - dim, 16/12/2018 - 12:14

Ulnar Artery Occlusion and severity markers of vasculopathy in Systemic sclerosis: a multicenter cross-sectional study.

Arthritis Rheumatol. 2018 Dec 15;:

Authors: Lescoat A, Yelnik CM, Coiffier G, Wargny M, Lamotte C, Cazalets C, Belhomme N, Ballerie A, Hatron PY, Launay D, Perdriger A, Sobanski V, Hachulla E, Jégo P

Abstract
OBJECTIVE: to evaluate the association of ulnar artery occlusion (UAO) assessed by Doppler Ultrasonography (dUS) with the severity markers of systemic sclerosis (SSc) METHODS: 204 unselected patients fulfilling 2013 ACR/EULAR classification criteria for SSc were included in this cross-sectional multicenter study. All patients benefit from bilateral hand dUS evaluating the presence of UAO and clinical/paraclinical visceral evaluation according to current guidelines. Univariable and multivariable ordinal regression models were conducted, grading the severity of UAO as "no UAO", "only one UAO" and "UAO on both hands" and assessing its association with clinical features of SSc.
RESULTS: UAO was found in 76 patients (37.3%) and was bilateral in 49 (24.0%). UAO as an ordinal event was significantly associated with disease duration, history of fingertip ulcers, telangiectasia, higher Rodnan skin score, worse DLCO values, higher tricuspid jet velocity, late capillaroscopic pattern and positivity for anti-centromere antibodies (ACA) (univariable analysis). In the adjusted multivariable ordinal model, UAO was less frequent in women (OR=0.35 [0.15-0.83]; p=0.017) and in patients with steroids (OR=0.24 [0.09-0.62]; p=0.0034). Significant association with UAO was persistent in multivariable analyses for history of fingertip ulcers (OR=2.55 [1.24-5.21]; p=0.011), higher Rodnan skin score (OR=1.65 [1.06-2.56]; p=0.025), lower DLCO values (OR=0.85 [0.78-0.94]; p=0.0015) and positivity of ACA (OR=2.89 [1.36-6.11]; p=0.0056).
CONCLUSION: UAO may represent a relevant severity marker of the vasculopathy in SSc. Its predictive value for the onset of severe vascular manifestations such as pulmonary arterial hypertension and its association with mortality remain to be determined in longitudinal studies. This article is protected by copyright. All rights reserved.

PMID: 30552835 [PubMed - as supplied by publisher]

Molecular mimicry, genetic homology, and gene sharing proteomic "molecular fingerprints" using an EBV (Epstein-Barr virus)-derived microarray as a potential diagnostic method in autoimmune disease.

Actualités En Médecine Interne - dim, 16/12/2018 - 12:14
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Molecular mimicry, genetic homology, and gene sharing proteomic "molecular fingerprints" using an EBV (Epstein-Barr virus)-derived microarray as a potential diagnostic method in autoimmune disease.

Immunol Res. 2018 Dec 15;:

Authors: Dreyfus DH, Farina A, Farina GA

Abstract
EBV (Epstein-Barr Virus) and other human DNA viruses are associated with autoimmune syndromes in epidemiologic studies. In this work, immunoglobulin G response to EBV-encoded proteins which share regions with human immune response proteins from the human host including ZEBRA (BZLF-1 encoded protein), BALF-2 recombinase expressed primarily during the viral lytic replication cycle, and EBNA-1 (Epstein-Barr Virus Nuclear Antigen) expressed during the viral latency cycle respectively were characterized using a laser-printed micro-array ( PEPperprint.com ). IgG response to conserved "A/T hooks" in EBV-encoded proteins such as EBNA-1 and the BALF-2 recombinase related to host DNA-binding proteins including RAG-1 recombinase and histones, and EBV-encoded virokines such as the IL-10 homologue BCRF-1 suggest further directions for clinical research. The author suggests that proteomic "molecular fingerprints" of the immune response to viral proteins shared with human immune response genes are potentially useful in early diagnosis and monitoring of autoantibody production and response to therapy in EBV-related autoimmune syndromes.

PMID: 30552620 [PubMed - as supplied by publisher]

Specific features of immune complexes in patients with sarcoidosis and pulmonary tuberculosis.

Actualités En Médecine Interne - dim, 16/12/2018 - 12:14
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Specific features of immune complexes in patients with sarcoidosis and pulmonary tuberculosis.

Immunol Res. 2018 Dec 14;:

Authors: Starshinova A, Zinchenko Y, Filatov M, Denisova N, Istomina E, Landa S, Burdakov V, Churilov L, Sapozhnikova N, Pavlova M, Stepanenko T, Mayevskaya V, Yablonskiy P

Abstract
Clinical and radiological features of tuberculosis and sarcoidosis are quite overlapping, and therefore, a diagnostic dilemma often persists. There are no commonly accepted criteria for the diagnosis of sarcoidosis due to the lack of data on the etiology of the disease. The exclusion of tuberculosis in every patient with suspected sarcoidosis is a mandatory stage of diagnosis, especially in countries with a high burden of tuberculosis. A prospective study was conducted with two groups of patients: group I (n = 50)-patients with pulmonary sarcoidosis established according to standard criteria; group II (n = 28)-patients with pulmonary tuberculosis with bacterial excretion. The control group (n = 24) was presented by healthy subjects. The examination complex included x-ray, bacteriological, immunological (Mantoux test with 2 TE, TB.SPOT test), and histological methods. All patients and healthy subjects were assessed for immune complexes with the use of the dynamic light scattering (DLS) method and adding of "healthy lung tissue extract" antigens and specific tuberculosis antigens ESAT-6 and SFP-10 in vitro. Significant differences were found in determining specific immune complexes in patients with pulmonary sarcoidosis and pulmonary tuberculosis. Registration of specific immune complex formation with "healthy lung tissue extract" in 100% cases may indicate the autoimmune nature of sarcoidosis. The absence of the immune complex formation in response to ESAT-6/SFP-10 antigens can be used for the differential diagnosis of two diseases. The diagnostic significance of the DLS method was 100% for sarcoidosis and 92.2% for tuberculosis. The data obtained in the study allows not only understanding the etiology of sarcoidosis, but also obtaining new criteria for the differential diagnosis of tuberculosis and pulmonary sarcoidosis.

PMID: 30552618 [PubMed - as supplied by publisher]

Off-label use of tocilizumab to treat non-juvenile idiopathic arthritis in pediatric rheumatic patients: a literature review.

Actualités En Médecine Interne - sam, 15/12/2018 - 13:57
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Off-label use of tocilizumab to treat non-juvenile idiopathic arthritis in pediatric rheumatic patients: a literature review.

Pediatr Rheumatol Online J. 2018 Dec 14;16(1):79

Authors: Jung JY, Kim MY, Suh CH, Kim HA

Abstract
Tocilizumab, an anti-interleukin-6 (IL-6) agent, is indicated as a treatment for several autoimmune or inflammatory diseases, including rheumatoid arthritis and juvenile idiopathic arthritis (JIA). IL-6 plays roles in both immune system dysregulation and inflammation, and thus efforts to extend the utility of tocilizumab in patients with autoinflammatory conditions are ongoing. Here, we survey the literature on the off-label use of tocilizumab in patients with juvenile-onset rheumatic diseases including juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis (DM), vasculitis, juvenile scleroderma, and other autoinflammatory diseases. There is no real evidence that tocilizumab is useful for patients with SLE and juvenile DM, but several cases of childhood Takayasu arteritis have experienced promising outcomes. In juvenile-onset scleroderma, for which no therapy that can halt disease progression is available, tocilizumab may stop progression and the associated functional impairment. Tocilizumab prevents systemic inflammation in patients with Kawasaki's disease, but may develop coronary aneurysms. Tocilizumab has been used to treat several pediatric autoinflammatory diseases, including JIA-associated uveitis and Castleman's disease. Further work in larger populations is necessary to confirm the effects of tocilizumab in patients with pediatric rheumatic diseases.

PMID: 30547812 [PubMed - in process]

Anti-MDA5 antibody-positive hypomyopathic dermatomyositis complicated with pneumomediastinum.

Actualités En Médecine Interne - sam, 15/12/2018 - 13:57
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Anti-MDA5 antibody-positive hypomyopathic dermatomyositis complicated with pneumomediastinum.

Fukushima J Med Sci. 2018;64(2):89-94

Authors: Yashiro M, Asano T, Sato S, Kobayashi H, Watanabe H, Miyata M, Migita K

Abstract
Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive interstitial lung disease (RP-ILD) resulting in high mortality. Here we report a 51-year-old Japanese woman with anti-MDA5 antibody-positive hypomyopathic dermatomyositis (DM) who developed RP-ILD. She developed respiratory failure and pneumomediastinum, however her RP-ILD responded favorably to the combined immunosuppressive treatments consisting of steroids, intravenous cyclophosphamide and tacrolimus. She was complicated with severe infections, which were successfully managed by combined modality therapy including artificial ventilation and antibiotics in addition to immunosuppressive treatments in parallel to the decline of anti-MDA5 antibody titer (>150 Index to 75 Index). She was discharged after 6 months of treatment without any respiratory sequelae. Hypomyopathic DM patients with high titers of anti-MDA5 antibody should be treated with aggressive immunosuppressive therapies and closely monitored to prevent various infections.

PMID: 30158335 [PubMed - indexed for MEDLINE]

Brain 18F-FDG PET Metabolic Abnormalities in Macrophagic Myofasciitis: Are They Stable?

Actualités En Médecine Interne - sam, 15/12/2018 - 13:57
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Brain 18F-FDG PET Metabolic Abnormalities in Macrophagic Myofasciitis: Are They Stable?

J Nucl Med. 2017 09;58(9):1532-1533

Authors: Blanc-Durand P, Van Der Gucht A, Sebaiti MA, Abulizi M, Authier FJ, Itti E

PMID: 28302762 [PubMed - indexed for MEDLINE]

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease; +18 new citations

Actualités En Médecine Interne - ven, 14/12/2018 - 22:02

18 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:

((((((thrombophlebitis) OR takayasu) OR temporal arteritis) OR sarcoidosis) OR myositis) OR scleroderma) OR sjogren's disease

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Primary diffuse large B-cell lymphoma as a chest-wall mass: A case report.

Vascularites - ven, 14/12/2018 - 06:28
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Primary diffuse large B-cell lymphoma as a chest-wall mass: A case report.

Medicine (Baltimore). 2018 Nov;97(47):e13291

Authors: Zhang Q, Ju Y, Qu T, Wang T, Liu X

Abstract
RATIONALE: Primary diffuse large B-cell lymphoma of the chest wall is extremely rare. A majority of the pleural lymphomas develop in patients with chronic tuberculous pyothorax. The underlying mechanism might be attributed to the sustained stimulation of chronic inflammation. Surgery followed by adjuvant chemotherapy can improve the outcome in some patients with lymphoma localized only in the chest wall. Thus, an early diagnosis of pyothorax-associated lymphoma is essential as it is a malignant, life-threatening condition.
PATIENT CONCERNS: A 79-year-old male complained of left-side chest pain for more than 2 months, which was not alleviated with nitrates and aspirin. The patient presented an intermittent low fever, anorexia, and marasmus, accompanied by tuberculosis 40 years ago and chronic left-side pyothorax. Also, ANCA (antineutrophil cytoplasmic autoantibody)-associated vasculitis occurred for >3years.
DIAGNOSIS: Computed tomography scan showed a solid mass in the left lateral chest wall. The patient underwent ultrasonic-guided biopsy of the lesion. A diagnosis of primary diffuse large B-cell lymphoma of the chest wall was established after histological examination.
INTERVENTION: Due to advanced age and poor physical condition, the patient received CHOP chemotherapy at a reduced dose.
OUTCOMES: The patient died 5 days after the first cycle of chemotherapy with severe dyspnea and high fever.
LESSONS: The chronic inflammation stimulation might result in the development of lymphoma in the chest wall of patients with long-term pyothorax, vasculitis, or other autoimmune diseases associated with malignancies. The fever, chest pain, or other nonspecific clinical symptoms in these patients should be under intensive focus as it might indicate the development of malignant lymphoma. Thus, histological examination in these patients is essential for accurate early diagnosis.

PMID: 30461640 [PubMed - indexed for MEDLINE]

Severe ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review.

Vascularites - ven, 14/12/2018 - 06:28
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Severe ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review.

BMC Ophthalmol. 2018 Nov 16;18(1):299

Authors: Zhang X, Xing B, You H, Wu H, Zhong Y, Ma J

Abstract
BACKGROUND: Granulomatosis with polyangiitis (GPA), a necrotizing granulomatous disease, very rarely involves the central nervous system (CNS), particularly the pituitary. Delayed treatment may cause permanent bilateral blindness. We report an isolated case of pituitary GPA that manifested as a progressive bilateral temporal visual field (VF) defect and was diagnosed via pituitary biopsy. Additionally, we review ocular, chiasmal and cranial nerve involvement in pituitary GPA.
CASE PRESENTATION: A 20-year-old Chinese man was referred for repeated fever, sudden headache, diplopia with a bilateral best-corrected visual acuity (BCVA) of 10/20, ptosis in both eyes and restricted abduction on the right side. VF tests showed bitemporal hemianopsia. Laboratory tests revealed hypothyroidism and were negative for autoimmune markers. Enhanced magnetic resonance imaging (MRI) showed pituitary enlargement. The diagnosis was lymphocytic pituitaritis. After intravenous (IV) dexamethasone treatment, full recovery occurred within 2 months. Two years later, the patient was readmitted for headache recurrence. With oral prednisone, the visual acuity in his right eye rapidly decreased to hand motion (HM) within one month. Enhanced MRI showed pituitary enlargement and a new, invasive suprasellar CNS lesion. All infection- and autoimmune-related tests were negative. The visual acuity in his right and left eye decreased to no light perception (NLP) after 6 days and 2 weeks, respectively. The biopsy results suggested GPA. After IV methylprednisolone treatment, complete remission of the symptoms occurred and was confirmed by MRI. The 15-month follow-up showed no signs of recurrence.
CONCLUSION: GPA typically affects the respiratory tract, lungs and kidneys. To date, 50 cases with pituitary involvement have been reported. Chiasmal and cranial nerve involvement leading to visual acuity impairment are common. We found 2 cases with severe visual loss resembling our case and discuss certain similarities.

PMID: 30445952 [PubMed - indexed for MEDLINE]

An unusual occurrence of stromal keratitis in dengue fever.

Vascularites - ven, 14/12/2018 - 06:28
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An unusual occurrence of stromal keratitis in dengue fever.

Indian J Ophthalmol. 2018 Nov;66(11):1631-1633

Authors: Bawankar P, Lahane T, Parekh R, Lahane S, Lahane S, Pathak P, Sonawane S

Abstract
Dengue is a mosquito-borne infection endemic in the tropical and subtropical regions of the world. Classic dengue fever is a self-limiting, influenza-like illness transmitted by Aedes aegypti mosquito. Ophthalmic manifestations though rare can involve both the anterior and posterior segments and are usually associated with the thrombocytopenic state. However, ophthalmic complications such as anterior uveitis and vasculitis suggest immune-mediated pathogenesis. Herein, we report a rare case of stromal keratitis and an unusual occurrence of simultaneous bilateral blindness following dengue fever in a young girl.

PMID: 30355887 [PubMed - indexed for MEDLINE]

Purpura fulminans with Lemierre's syndrome caused by Gemella bergeri and Eikenella corrodens: a case report.

Vascularites - ven, 14/12/2018 - 06:28
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Purpura fulminans with Lemierre's syndrome caused by Gemella bergeri and Eikenella corrodens: a case report.

BMC Infect Dis. 2018 Oct 19;18(1):523

Authors: Yamagishi T, Hikone M, Sugiyama K, Tanabe T, Wada Y, Furugaito M, Arai Y, Uzawa Y, Mizushima R, Kamada K, Itakura Y, Iguchi S, Yoshida A, Kikuchi K, Hamabe Y

Abstract
BACKGROUND: Gemella bergeri is one of the nine species of the genus Gemella and is relatively difficult to identify. We herein describe the first case of septic shock due to a Gemella bergeri coinfection with Eikenella corrodens.
CASE PRESENTATION: A 44-year-old Asian man with a medical history of IgG4-related ophthalmic disease who was prescribed corticosteroids (prednisolone) presented to our hospital with dyspnea. On arrival, he was in shock, and a purpuric eruption was noted on both legs. Contrast enhanced computed tomography showed fluid retention at the right maxillary sinus, left lung ground glass opacity, and bilateral lung irregular opacities without cavitation. Owing to suspected septic shock, fluid resuscitation and a high dose of vasopressors were started. In addition, meropenem, clindamycin, and vancomycin were administered. Repeat computed tomography confirmed left internal jugular and vertebral vein thrombosis. Following this, the patient was diagnosed with Lemierre's syndrome. Furthermore, he went into shock again on day 6 of hospitalization. Additional soft tissue infections were suspected; therefore, bilateral below the knee amputations were performed for source control. Cultures of the exudates from skin lesions and histopathological samples did not identify any pathogens, and histopathological findings showed arterial thrombosis; therefore it was concluded that the second time shock was associated with purpura fulminans. Following this, his general status improved. He was transferred to another hospital for rehabilitation. The blood culture isolates were identified as Gemella bergeri and Eikenella corrodens. Gemella bergeri was identified by matrix-assisted laser desorption ionization-time of flight mass spectrometry and confirmed by 16S rRNA gene sequencing later. The primary focus of the infection was thought to be in the right maxillary sinus, because the resolution of the fluid retention was confirmed by repeat computed tomography.
CONCLUSIONS: Gemella bergeri can be the causative pathogen of septic shock. If this pathogen cannot be identified manually or through commercial phenotypic methods, 16S rRNA gene sequencing should be considered.

PMID: 30340466 [PubMed - indexed for MEDLINE]

Ischemic stroke as a complication of cryptococcal meningitis and immune reconstitution inflammatory syndrome: a case report.

Vascularites - ven, 14/12/2018 - 06:28
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Ischemic stroke as a complication of cryptococcal meningitis and immune reconstitution inflammatory syndrome: a case report.

BMC Infect Dis. 2018 Oct 16;18(1):520

Authors: Ellis JP, Kalata N, Joekes EC, Kampondeni S, Benjamin LA, Harrison TS, Lalloo DG, Heyderman RS

Abstract
BACKGROUND: Cryptococcal meningitis remains the leading cause of adult meningitis in Sub-Saharan Africa. Immune Reconstitution Inflammatory Syndrome (IRIS) following anti-retroviral therapy (ART) initiation is an important complication. Here we report the first documented case of a IRIS reaction presenting as an ischemic stroke.
CASE PRESENTATION: A 38 year old newly diagnosed HIV-infected, ART naive Malawian male presented to a tertiary referral hospital in Blantyre, Malawi with a 2 week history of headache. A diagnosis of cryptococcal meningitis was made and the patient was started on 1200 mg fluconazole once daily and flucytosine 25 mg/kg four times daily as part of the Advancing Cryptococcal Treatment for Africa (ACTA) clinical trial. There was an initial clinical and microbiological response to anti-fungal treatment and anti-retroviral therapy was started at week 4. The patient re-presented 16 days later with recurrence of headache, fever, and a sudden onset of left sided weakness in the context of rapid immune reconstitution; peripheral CD4 count had increased from a baseline of 29 cells/μl to 198 cells/μl. Recurrence of cryptococcal meningitis was excluded through CSF examination and fungal culture. Magnetic Resonance Imaging (MRI) of the brain demonstrated multi-focal DWI (diffusion weighted imaging) positive lesions consistent with an ischemic stroke. Given the temporal relationship to ART initiation, these MRI findings in the context of sterile CSF with raised CSF protein and a rapid immune reconstitution, following an earlier favorable response to treatment is most consistent with a paradoxical Immune Reconstitution Inflammatory Syndrome.
CONCLUSIONS: Stroke is an increasing cause of morbidity and mortality amongst HIV infected persons. Ischemic stroke is a recognized complication of cryptococcal meningitis in the acute phase and is thought to be mediated by an infectious vasculitis. This is the first time an ischemic stroke has been described as part of a paradoxical IRIS reaction. This report adds to the spectrum of clinical IRIS presentations recognized and highlights to clinicians the potential complications encountered at ART initiation in severely immunocompromised patients.

PMID: 30326861 [PubMed - indexed for MEDLINE]

Severe Phlebitis Accompanying Vascular Rejection Type Acute T Cell-Mediated Rejection in Renal Transplantation.

Vascularites - ven, 14/12/2018 - 06:28
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Severe Phlebitis Accompanying Vascular Rejection Type Acute T Cell-Mediated Rejection in Renal Transplantation.

Transplant Proc. 2018 Oct;50(8):2545-2547

Authors: Yamanaka K, Oka K, Abe T, Nakazawa S, Kato T, Imamura R, Kishikawa H, Ichimaru N, Nishimura K, Kyakuno M, Takahara S, Nonomura N

Abstract
PURPOSE: Renal transplant patients with vascular rejection type acute T cell-mediated rejection (ATCMR) grade II have a poor prognosis. Vascular lesions in those cases are thought to randomly occur, thus we searched for a novel pathological marker related to vascular rejection in kidney transplantation.
METHODS: We determined pathological characteristics in 14 ATCMR grade II patients treated during an acute phase from 2004 to 2013. We then examined whether those findings appeared in transplant kidney biopsy specimens, except for cases of vascular rejection, in patients examined from 2010 to 2014.
RESULTS: In 9 of the 14 ATCMR grade II patients, phlebitis was accompanied by inflammatory cells that formed polypoid projections in the venous lumen and partial disappearance of vascular endothelium. Further investigation showed those inflammatory cells to be T cells and macrophages. Histological findings revealed coexisting phlebitis in 2 of 13 patients with ATCMR grade I, 3 of 24 with borderline changes, and none with normal findings. Phlebitis occurred at a significantly greater rate than the other findings in cases of vascular rejection (P < .05). However, there was no significant difference in regard to graft survival between patients with and without phlebitis (P = .1829).
CONCLUSION: Our results suggest severe phlebitis as a novel finding associated with the pathology of vascular rejection in patients with a renal allograft.

PMID: 30316395 [PubMed - indexed for MEDLINE]

Postoperative Hemorrhagic Occlusive Retinal Vasculitis with Intracameral Vancomycin.

Vascularites - ven, 14/12/2018 - 06:28
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Postoperative Hemorrhagic Occlusive Retinal Vasculitis with Intracameral Vancomycin.

Korean J Ophthalmol. 2018 Oct;32(5):430-431

Authors: Lee JY, Lee EK, Lee HJ, Jeong J, Lee SY, Kim JY

PMID: 30311469 [PubMed - indexed for MEDLINE]

Cerebral granulomatosis as a manifestation of Crohn's disease.

Vascularites - ven, 14/12/2018 - 06:28
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Cerebral granulomatosis as a manifestation of Crohn's disease.

BMC Neurol. 2018 Oct 03;18(1):161

Authors: Whittaker K, Guggenberger K, Venhoff N, Doostkam S, Schaefer HE, Fritsch B

Abstract
BACKGROUND: Crohn's disease (CD) is associated with a variety of extra-intestinal manifestations. Most commonly these involve the eye, skin, joints, coagulation system and liver. Cerebral manifestations of CD have been reported to a far lesser extent. The extensive detrimental impact of neurological symptoms on a patient's quality of life makes an early diagnosis and treatment particularly important. In previous case-reports, diagnosis of cerebral manifestations in CD often relied upon magnetic resonance imaging (MRI) and computed tomography (CT) alone. To our knowledge, only one case-report has documented a histologically confirmed case of cerebral lesions associated with CD so far.
CASE PRESENTATION: A 39-year-old right-handed woman with a history of CD was referred to our hospital with etiologically unexplained Gadolinium (Gd)-enhancing cortical lesions, triggering epileptic seizures. A CT-scan of the thorax and bronchoalveolar lavage found no signs of sarcoidosis. Lumbar punctures and laboratory testing found no underlying infection or coincidental autoimmune disorders and MRI-scans showed progression of lesion load. Consequently, the patient underwent stereotactic biopsy of a cortical lesion. Histological examination revealed a mixed lympho-histiocytic and tuberculoid granulomatous inflammation surrounding small vessels and no signs for infection. After exclusion of other granulomatous diseases and the typical histological findings we diagnosed a cerebral granulomatosis as a manifestation of CD. The patient was initially started on azathioprine, which had to be switched to corticosteroids and methotrexate because of an azathioprine related pancreatitis. The patient has not suffered any further epileptic seizures to date.
CONCLUSION: Cerebral manifestation of CD is a possibly underreported entity that may respond well to immunosuppressive treatment. In contrast to earlier reports of cerebral manifestations in CD, our patient showed no coincident gastrointestinal symptoms indicating an activity of CD during the progression of cortical lesion load, suggesting that similar to other extra-intestinal manifestations in CD, the activity of gastrointestinal symptoms does not necessarily reflect the activity of CD associated cerebral vasculitis. Therefore, diagnosis and therapy of cerebral manifestation may be delayed when focusing on gastrointestinal symptoms alone.

PMID: 30285676 [PubMed - indexed for MEDLINE]

The influence of oral health and psycho-social well-being on clinical outcomes in Behçet's disease.

Vascularites - ven, 14/12/2018 - 06:28
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The influence of oral health and psycho-social well-being on clinical outcomes in Behçet's disease.

Rheumatol Int. 2018 Oct;38(10):1873-1883

Authors: Senusi A, Higgins S, Fortune F

Abstract
This study was designed to investigate the association of oral ulceration and oral health factors, together with psycho-social well-being in Behçet's disease (BD), and to clarify the importance of psycho-social support of patients in the overall management of BD. The study comprised of a cohort of 146 BD patients (mean age ± SD = 39.65 ± 13.20) and 20 recurrent aphthous stomatitis (RAS) patients (mean age ± SD = 42.32 ± 11.32). Oral ulcer severity score (OUSS), Behçet's disease current activities form (BDCAF), hospital anxiety and depression scale (HADS), and the work and social adjustment scale (WSAS) were investigated. Oral health risk factors were also included. The analysis of variance, regression, and factor analysis were used to scrutinise the data. Almost 73% of patients were at high caries risk in BD and RAS groups. Thirty-nine percent of BD and forty percent of RAS had a score of BPE3 (probing depth 3.5-5.5 mm). Regression analysis revealed that OUSS and WSAS had a positive impact to increase the BDCAF score in BD patients (β = 0.395, P = 0.001; β = 0.240, P = 0.019), respectively. Dental health, periodontal health, anxiety, depression, and WSAS variables had strong loadings by factor analysis based on gender and at the time of present and absent of oral ulceration. The main oral ulcer characteristics that had significant influences on the total of oral health quality of life by 68.6% were: size, duration, ulcer-free period, and pain. The results highlighted the significant influence of oral ulceration, patients' oral health, diet, and psycho-social well-being as multi-factorial causes on increasing disease activity in BD patients.

PMID: 30151720 [PubMed - indexed for MEDLINE]

Intracranial Giant Mycotic Aneurysm without Endocarditis and Vasculitis: Report of Rare Entity and Review of Literature.

Vascularites - ven, 14/12/2018 - 06:28
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Intracranial Giant Mycotic Aneurysm without Endocarditis and Vasculitis: Report of Rare Entity and Review of Literature.

World Neurosurg. 2018 Nov;119:353-357

Authors: Boissonneau S, Graillon T, Meyer M, Brunel H, Fuentes S, Dufour H

Abstract
BACKGROUND: Infectious aneurysm is a rare complication in intracranial aneurysm. Moreover, giant aneurysm is a rare entity in intracranial aneurysm. In the great majority of infectious intracranial aneurysms, vasculitis and/or endocarditis is associated.
CASE DESCRIPTION: Here, we report the case of an 83-year-old man who developed a giant infectious intracranial aneurysm on the anterior communicating artery. This patient had never shown any intracranial bleeding. A surgery was performed, and purulent liquid and aneurysm wall were removed during procedure. The bacteriologic analyses reported Campylobacter fetus.
CONCLUSIONS: This bacterium seems to be more and more frequent in North America and Europe, according to the current literature. Moreover, cases of mycotic extracranial aneurysm were reported. Here we report the first case of intracranial giant infectious aneurysm in an adult patient, without any endocarditis or vasculitis. This new entity could be the cause of a bacterial graft on an asymptomatic giant preexisting intracranial aneurysm or a consequence of campylobacteriosis.

PMID: 30144607 [PubMed - indexed for MEDLINE]

Neutropenia related to an azathioprine metabolic disorder induced by an inosine triphosphate pyrophosphohydrolase (ITPA) gene mutation in a patient with PR3-ANCA-positive microscopic polyangiitis
.

Vascularites - ven, 14/12/2018 - 06:28
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Neutropenia related to an azathioprine metabolic disorder induced by an inosine triphosphate pyrophosphohydrolase (ITPA) gene mutation in a patient with PR3-ANCA-positive microscopic polyangiitis
.

Clin Nephrol. 2018 Nov;90(5):363-369

Authors: Honda K, Kobayashi A, Niikura T, Hasegawa T, Saito Z, Ito S, Sasaki T, Komine K, Ishizuka S, Motoi Y, Kubota T, Yamamoto H, Yokoo T

Abstract
A 68-year-old Japanese man was monitored for chronic kidney disease (CKD), with unknown primary disease starting in 2014. His serum creatinine (sCr) was stable at ~ 2.5 mg/dL for ~ 2 years. Two weeks before admission, he had bloody sputum, and sCr increased to 4.63 mg/dL. Soon after admission, the patient developed a high fever with pigment spots on the legs. A kidney biopsy was performed. The kidney specimens showed necrotizing and crescentic glomerulonephritis without granuloma formation. An additional blood-sampling test revealed high titers of PR3-ANCA, and we diagnosed PR3-ANCA-positive microscopic polyangiitis (MPA). Treatment with intravenous steroid pulse therapy and intermittent pulse intravenous cyclophosphamide therapy was started for remission induction. With these treatments, sCr improved to ~ 3.0 mg/dL. Azathioprine (AZA) was added for remission-maintenance therapy. Three days later, the dose of AZA was increased from 50 to 100 mg/day, and the number of neutrophils decreased to 30/μL. After withdrawal of AZA, neutrophil levels gradually recovered. We suspected that an abnormal metabolism of AZA was responsible for the neutropenia. Therefore, we analyzed three AZA metabolism-associated genes for mutations: thiopurine S-methyltransferase (TPMT), inosine triphosphate pyrophosphohydrolase (ITPA), and nucleoside diphosphate linked moiety X-type motif 15 (NUDT15), and we identified ITPA 94C>A mutation. This was a rare case of PR3-positive MPA with AZA-induced severe neutropenia that was possibly due to an ITPA gene mutation. This case suggests that ITPA gene mutation is related to the adverse reactions of AZA in Japanese patients. We have to pay attention to severe neutropenia when we use AZA, especially in Asian patients with CKD.
.

PMID: 30106365 [PubMed - indexed for MEDLINE]

A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts.

Vascularites - ven, 14/12/2018 - 06:28
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A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts.

Rheumatol Int. 2018 Oct;38(10):1833-1840

Authors: Karadag O, Erden A, Bilginer Y, Gopaluni S, Sari A, Armagan B, Ertenli I, Ozen S, Jayne D

Abstract
There is a need for better definition of polyarteritis nodosa (PAN) subphenotypes and the influence of ethnicity and geography. This study is aimed to study the demographic and clinical features of PAN cohorts from the UK and Turkey (TR) and to compare and contrast disease characteristics. A retrospective survey of databases from two vasculitis centres between 1990 and 2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. All paediatric-onset adult patients met the Ankara 2008 (EULAR/PReS endorsed) criteria for childhood PAN. Those with typical angiographic and/or histopathologic findings consistent with PAN were included. 93 (M/F: 51/42) patients (UK: 47, TR: 46) were included. Three were HBV-related, 20 (21.5%) had paediatric onset and 16 (16.5%), cutaneous PAN. TR patients had younger age of disease onset 44 (28.5-59.0) vs. 24.5 (11.8-40.5), p = 0.002. Twelve (26%) of TR patients had monogenic disease (Familial Mediterranean Fever association (n = 7), deficiency of adenosine deaminase 2, DADA2, (n = 5). No difference was found in phenotype between paediatric and adult onset patients except for frequency of cutaneous lesions (p = 0.002). During a median 67.5 (32-126) months follow-up, 13 patients died (12.7% in UK vs. 15.2% in Turkish cohorts). No difference was found between two cohorts in relation to relapse rate, death and vasculitis damage index. This study defined a diagnosis of PAN according to the EMEA algorithm. The TR group had a younger age of disease onset and more cases of monogenic disease; however, disease extent, relapse rate, damage index and death rates were similar between groups.

PMID: 30099592 [PubMed - indexed for MEDLINE]

Surgical Treatment of Symptomatic Aortic Aneurysm in a Patient with Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis: Case Report and Review of the Literature.

Vascularites - ven, 14/12/2018 - 06:28
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Surgical Treatment of Symptomatic Aortic Aneurysm in a Patient with Anti-neutrophil Cytoplasmic Antibody-associated Vasculitis: Case Report and Review of the Literature.

Ann Vasc Surg. 2018 Nov;53:270.e17-270.e21

Authors: Veraldi GF, Mezzetto L, Scorsone L, Sacco M, Eccher A, Idolazzi L

Abstract
Vasculitis is an heterogeneous group of syndromes, which shares inflammation of blood vessel wall as the main feature. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels (i.e., capillaries, venules, arterioles, and small arteries), associated with ANCAs specific for myeloperoxidase or proteinase 3. Clinical manifestations may be heterogeneous but an involvement of lungs and kidneys frequently occurs. AAV of large vessels is a very rare condition whose standard therapy is medical approach. Surgical revascularization has been described in selected patients after medical failure or in emergent settings. We report the case of a patient affected by symptomatic infrarenal aortic aneurysm related to AAV, who underwent in-situ reconstruction by means of cryopreserved homograft.

PMID: 30092440 [PubMed - indexed for MEDLINE]

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