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NCBI: db=pubmed; Term=vasculitis AND ((English[lang] OR French[lang]) AND adult[MeSH] AND "last 90 days"[PDat])
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Peripheral levels of brain-derived neurotrophic factor and S100B in neuropsychiatric systemic lupus erythematous.

ven, 04/01/2019 - 13:04
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Peripheral levels of brain-derived neurotrophic factor and S100B in neuropsychiatric systemic lupus erythematous.

Lupus. 2018 Nov;27(13):2041-2049

Authors: Noris-García E, Arce S, Nardin P, Lanigan ME, Acuña V, Gutierrez F, Robinson-Agramonte MA, Gonçalves CA

Abstract
BACKGROUND: The aim of this study was to investigate serum S100B and brain-derived neurotrophic factor (BDNF) in systemic lupus erythematous (SLE) patients, with and without neuropsychiatric (NP) manifestation activity.
METHODS: We assessed 47 SLE patients and 20 selected healthy individuals. Disease activity was assessed according to the SLE disease activity index (SLEDAI). Serum BDNF and S100B were measured by enzyme-linked immunosorbent assay.
RESULTS: Serum S100B protein was significantly higher in SLE patients. BDNF levels were significantly decreased in active SLE, when compared with inactive SLE, but not when compared with controls. S100B was clearly higher in the NPSLE group, when compared with the non-NPSLE or control groups. Receiver operating characteristic analysis of S100B revealed an area under the curve of 0.706 that discriminated NPSLE patients with peripheral polyneuropathy.
CONCLUSIONS: Our findings reinforce the use of serum S100B as a biomarker in SLE, particularly for NPSLE. Moreover, we found a strong association between serum S100B and peripheral neuropathy, indicating a specific utility for this biomarker in SLE that warrants clinical investigation.

PMID: 30376438 [PubMed - indexed for MEDLINE]

Skin signs in juvenile- and adult-onset systemic lupus erythematosus: clues to different systemic involvement.

ven, 04/01/2019 - 13:04
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Skin signs in juvenile- and adult-onset systemic lupus erythematosus: clues to different systemic involvement.

Lupus. 2018 Nov;27(13):2069-2075

Authors: Chottawornsak N, Rodsaward P, Suwannachote S, Rachayon M, Rattananupong T, Deekajorndech T, Asawanonda P, Chiewchengchol D, Rerknimitr P

Abstract
OBJECTIVE: We aim to explore the differences of skin signs between juvenile- and adult-onset systemic lupus erythematosus and to identify their associations to the development of systemic involvement.
METHODS: A retrospective chart review of 377 systemic lupus erythematosus patients was performed.
RESULTS: In total, 171 patients with juvenile systemic lupus erythematosus and 206 with adult systemic lupus erythematosus were studied. All patients were of Southeast Asian descent. The mean duration of follow up was 8.18 ± 6.19 and 9.36 ± 7.68 years for juvenile systemic lupus erythematosus and adult systemic lupus erythematosus, respectively. At diagnosis, most patients presented with acute cutaneous lupus erythematosus, whereas chronic cutaneous lupus erythematosus was twice as common in adult systemic lupus erythematosus ( p < 0.001). The mean Systemic Lupus Erythematosus Disease Activity Index of juvenile systemic lupus erythematosus was significantly higher than that of adult systemic lupus erythematosus (14.29 ± 7.13 vs 11.27 ± 6.53). Multivariate analysis revealed the following associations in juvenile systemic lupus erythematosus: acute cutaneous lupus erythematosus and non-scarring alopecia with increased risk of arthralgia, mucosal ulcers with leukopenia, cutaneous vasculitis with seizure, and finding of granular casts. On the contrary, the associations for adult systemic lupus erythematosus were oral ulcers with arthralgia and cutaneous vasculitis with myositis.
CONCLUSIONS: Cutaneous signs in systemic lupus erythematosus may signal prognostic implication. Interestingly, despite similar cutaneous lesions in systemic lupus erythematosus, different ages of onset are associated with different systemic involvement.

PMID: 30336755 [PubMed - indexed for MEDLINE]

The blood-brain barrier, TWEAK, and neuropsychiatric involvement in human systemic lupus erythematosus and primary Sjögren's syndrome.

ven, 04/01/2019 - 13:04
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The blood-brain barrier, TWEAK, and neuropsychiatric involvement in human systemic lupus erythematosus and primary Sjögren's syndrome.

Lupus. 2018 Nov;27(13):2101-2111

Authors: Lauvsnes MB, Tjensvoll AB, Maroni SS, Kvivik I, Grimstad T, Greve OJ, Harboe E, Gøransson LG, Putterman C, Omdal R

Abstract
OBJECTIVE: A prevailing hypothesis for neuropsychiatric involvement in systemic lupus erythematosus (SLE) and primary Sjögren's syndrome is that brain reactive autoantibodies enter the brain through a disrupted blood-brain barrier. Our aim was to investigate whether TNF-like weak inducer of apoptosis (TWEAK) plays a role in cerebral involvement in human SLE and primary Sjögren's syndrome, and whether an impaired blood-brain barrier is a prerequisite for neuropsychiatric manifestations.
METHODS: TWEAK was measured in the cerebrospinal fluid and serum and compared with markers of blood-brain barrier permeability (Q-albumin and MRI contrast-enhanced lesions) and S100B, an astrocyte activation marker in 50 SLE and 52 primary Sjögren's syndrome patients. Furthermore, we estimated the general intrathecal B-cell activation (IgG index), measured anti-NR2 antibodies in cerebrospinal fluid, and explored whether these variables were associated with neuropsychiatric manifestations.
RESULTS: No associations were found between TWEAK in the cerebrospinal fluid or serum and neuropsychiatric manifestations in SLE nor in primary Sjögren's syndrome patients. Furthermore, no associations were found between neuropsychiatric manifestations and indicators of blood-brain barrier integrity or astroglial activity. Anti-NR2 antibodies were associated with impaired visuospatial processing (odds ratio 4.9, P = 0.03) and motor functioning (odds ratio 6.0, P = 0.006).
CONCLUSION: No clinical neuropsychiatric manifestations could be attributed to impaired integrity of the blood-brain barrier, or to TWEAK levels in cerebrospinal fluid or serum in either patient group. The TWEAK concentration was considerably higher in the cerebrospinal fluid than in blood, which indicates intrathecal production. We hypothesize that increased TWEAK and S100B result from immunological stress caused by brain-reactive antibodies produced by brain residing immune cells.

PMID: 30282561 [PubMed - indexed for MEDLINE]

Occult Giant Cell Arteritis Concurrent With Pancreatic Carcinoma Revealed by 18F-FDG PET/CT.

mar, 01/01/2019 - 12:55
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Occult Giant Cell Arteritis Concurrent With Pancreatic Carcinoma Revealed by 18F-FDG PET/CT.

Clin Nucl Med. 2018 Dec;43(12):941-942

Authors: Fu Z, Chen X, Yang X, Li Q

Abstract
An F-FDG PET/CT was performed on a 74-year-old woman with pancreatic carcinoma and liver metastases. Occult giant cell arteritis, involving the aorta, truncus brachiocephalicus, subclavian arteries, axillaries, external carotid arteries, vertebral arteries, and superficial temporal arteries, was accidentally revealed.

PMID: 30300198 [PubMed - indexed for MEDLINE]

Arterial involvement in Erdheim-Chester disease: A retrospective cohort study.

sam, 29/12/2018 - 11:34
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Arterial involvement in Erdheim-Chester disease: A retrospective cohort study.

Medicine (Baltimore). 2018 Dec;97(49):e13452

Authors: Villatoro-Villar M, Bold MS, Warrington KJ, Crowson CS, Goyal G, Shah M, Go RS, Koster MJ

Abstract
Erdheim-Chester disease (ECD) is a rare histiocytosis of the "L" (Langerhans) group with multisystem involvement that can affect the large and medium-sized arteries mimicking vasculitis. Aortic involvement is common but the frequency and outcome of aortic branch vessel abnormalities are less well described.Patients with ECD were retrospectively identified. Images containing information of arterial involvement within 6 months of diagnosis were considered baseline and compared to last follow-up studies. Two physicians independently reviewed the studies to evaluate for presence of abnormalities attributable to ECD. Age and sex-adjusted logistic regression models were used to examine associations between patient characteristics and vessel involvement at baseline.Among a cohort of 64 patients with ECD, 63 had baseline imaging of vascular structures. ECD involvement of at least 1 segment of the aorta was observed in 56%. Abnormalities were also observed in aortic arch branches (26%), visceral branch arteries (40%), iliofemoral arteries (31%), coronary (5%), and pulmonary (3%) arteries. Perinephric fibrosis was strongly associated with the identification of abnormalities in the thoracic aorta (OR 4.92 [1.54, 15.75]; P = .007), abdominal aorta (OR 7.57 [2.28, 25.07]; P = .001) and visceral branch arteries (OR 6.05 [1.52, 24.03]; P = .01) but not pelvic/lower extremity arteries. Complete normalization of arterial abnormalities at follow-up was only observed in 9% or less of arterial segments involved at baseline.Aortic and aortic branch vessel abnormalities are frequently observed in patients with ECD and are often asymptomatic. Partial and/or complete resolution of arterial findings is uncommon.

PMID: 30544428 [PubMed - indexed for MEDLINE]

Pulmonary Hemorrhaging as a Fatal Complication of IgA Vasculitis.

mer, 26/12/2018 - 10:19
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Pulmonary Hemorrhaging as a Fatal Complication of IgA Vasculitis.

Intern Med. 2018 Nov 01;57(21):3141-3147

Authors: Miyoshi S, Nagao T, Kukida M, Miyoshi KI, Namba C, Kitazawa S, Nakamura Y, Hamaguchi N, Higaki J

Abstract
A 64-year-old man was admitted to our hospital for purpuric rash, joint pain, and a fever. He had earlier undergone a follow-up examination for interstitial lung disease. At the current visit, the diagnosis was immunoglobulin A (IgA) vasculitis, based on skin and renal biopsy findings. He developed sudden breathlessness and hemoptysis. Chest computed tomography revealed ground glass opacity in the right lower lung fields, suggesting pulmonary hemorrhaging associated with IgA vasculitis. Despite steroid and cyclophosphamide therapy, and plasma exchange, he died 52 days after admission. Early aggressive therapies may be recommended for old patients with IgA vasculitis who have an additional comorbidities.

PMID: 29877284 [PubMed - indexed for MEDLINE]

Phenotypic characterization of patients with rheumatologic manifestations of common variable immunodeficiency.

mer, 26/12/2018 - 10:19
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Phenotypic characterization of patients with rheumatologic manifestations of common variable immunodeficiency.

Semin Arthritis Rheum. 2018 10;48(2):318-326

Authors: Gutierrez MJ, Sullivan KE, Fuleihan R, USIDNET Consortium, Bingham CO

Abstract
Patients with common variable immunodeficiency (CVID) have a higher incidence of rheumatologic disorders. To delineate this clinical association, we investigated the phenotypic features of patients with CVID affected by these conditions.
METHODS: We conducted a retrospective analysis of 870 pediatric and adult patients with CVID included in the United States Immunodeficiency Network (USIDNET) registry. Outcomes included clinical characteristics (age, gender, ethnicity, rheumatologic diagnosis, and comorbidities), infectious history and basic immunophenotype (serum immunoglobulin levels, CD19+ B cells, and CD4/CD8 ratio) in patients with CVID and rheumatologic disorders compared to those with non-inflammatory CVID. Demographic and clinical data were compared using chi-square, Fisher's exact or Wilcoxon-Mann-Whitney tests. Non-parametric tests, single and multiple logistic regression models were used to evaluate the relationship between CVID-associated rheumatologic disorders and basic immunophenotypic parameters (IgA, IgM, CD19+ B-cell counts, and CD4/CD8 ratios).
RESULTS: Physician-reported rheumatic diseases were present in 5.9% of patients with CVID (n = 51) included in the registry. Although CVID affects both sexes equally, and patients are of predominantly White-Caucasian ethnicity, there were more females (3.3:1 female to male ratio) and increased proportion of non-white patients in the rheumatologic disease group (p < 0.05). Specific disorders included: inflammatory arthritis (n = 18), Sjogren's syndrome (n = 11), SLE (n = 8), Raynaud's syndrome (n = 8), vasculitis (n = 9), MCTD (n = 3), and other (n = 5). In about one-third of patients, a rheumatologic condition was associated with an additional inflammatory complication or malignancy. In regards to the immunophenotype parameters compared (CD19+ B-cell counts, CD4/CD8 ratio, IgA, and IgM), no significant differences were demonstrated between the two groups.
CONCLUSION: Our findings highlight the coexistence of primary antibody immunodeficiencies and systemic rheumatologic disorders, describe the spectrum of rheumatologic manifestations, and contrast differences in relevant demographic, clinical and immunophenotype parameters in the largest registry of CVID patients in the U.S. In spite of its limitations, our study details the intersection of systemic autoimmunity and CVID and provides valuable insights into these two groups of disorders. Further delineating the link between systemic autoimmunity and humoral immunodeficiencies can provide novel insights into the immune abnormalities underlying these related conditions.

PMID: 29599028 [PubMed - indexed for MEDLINE]

Reducing glucocorticoid duration in ANCA-associated vasculitis: A pilot trial.

mer, 26/12/2018 - 10:19
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Reducing glucocorticoid duration in ANCA-associated vasculitis: A pilot trial.

Semin Arthritis Rheum. 2018 10;48(2):288-292

Authors: Miloslavsky EM, Niles JL, Wallace ZS, Cortazar FB, Fernandes A, Laliberte K, Stone JH

Abstract
OBJECTIVE: Therapeutic advances in ANCA-associated vasculitis (AAV) have improved patient survival, but mortality rates remain higher than the general population. Glucocorticoids contribute to AAV morbidity and mortality. We examined whether an 8-week glucocorticoid course in combination with rituximab (RTX) would induce disease remission in patients with AAV.
METHODS: Patients with active AAV received an 8-week prednisone taper and RTX 375mg/m2 weekly for 4 weeks. Patients with severe glomerulonephritis or diffuse alveolar hemorrhage requiring mechanical ventilation were excluded. In-person and telephone visits were scheduled for disease activity assessment. The primary endpoint was complete remission at 24 weeks (no disease activity while being off prednisone with no intercedent relapses). Secondary analysis included comparing study outcomes to historical controls from the Rituximab in AAV (RAVE) trial.
RESULTS: Fourteen of 20 patients (70%) achieved the primary outcome. The patients in our trial achieved the primary outcome at a rate similar to that of controls from the RAVE trial (adjusted OR 1.31 [0.26-6.56]), had fewer median adverse events per patient (2 versus 8, p < 0.001) but were more likely to relapse (30% versus 7%, p = 0.03). Most relapses occurred in patients who had severe vasculitic manifestations at trial entry. Disease damage did not differ between the two trial populations.
CONCLUSION: An 8-week course of prednisone with RTX resulted in a similar rate of complete remission at 6 months as in the RAVE trial, with fewer adverse events but more frequent relapses. Further study of this protocol is warranted in selected patient populations.

PMID: 29530330 [PubMed - indexed for MEDLINE]

Hydralazine-associated vasculitis: Overlapping features of drug-induced lupus and vasculitis.

mer, 26/12/2018 - 10:19
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Hydralazine-associated vasculitis: Overlapping features of drug-induced lupus and vasculitis.

Semin Arthritis Rheum. 2018 10;48(2):283-287

Authors: Kumar B, Strouse J, Swee M, Lenert P, Suneja M

Abstract
INTRODUCTION: Hydralazine is an antihypertensive medication that has been associated with drug-induced lupus erythematosus (DIL) as well as ANCA-associated vasculitis (AAV). Although rare, early diagnosis is critical since drug cessation is the mainstay of therapy. This retrospective study aims to characterize the clinical, laboratory, and histopathologic features of this disease.
METHODS: Once approval was obtained from the Institutional Review Board at the University of Iowa, all patients carrying a diagnosis of vasculitis (ICD9 code: 447.6 or ICD10 code: I77.6, I80, L95, M30, or M31) and positive ANCA lab results over the past 15 years were identified. Age, gender, comorbid conditions, medications taken over the prior 6 months, laboratory data, including electrolytes, urine studies and serologies, chest x-rays, CT scans, and pathologic biopsy records were abstracted from the electronic medical record.
RESULTS: 323 cases of AAV were identified, of which 12 were exposed to hydralazine, all at the time of diagnosis. The average duration of hydralazine therapy was 22 months and mean cumulative dose was 146g. Patients were typically older (70.3 years old) with slight female preponderance (7 females). Eleven patients presented with dyspnea, fatigue, and unintentional weight loss. Five had polyarthralgias and 8 had lower extremity petechiae. All 12 patients were both ANA and ANCA positive. ANA titers ranged from 1:160 and 1:2560. Ten were of diffuse pattern while 2 were nucleolar. ANCA titers ranged from 1:320 to 1:2560. Eleven had a pANCA pattern while one had cANCA. All 12 patients were positive for histone and 11 were positive for myeloperoxidase antibodies. Eleven also had dsDNA antibodies, and 4 had anti-cardiolipin IgG or IgM antibodies. Nine patients were also hypocomplementemic (mean C3 level: 88.4mg/dL; mean C4 level: 16.5mg/dL). All patients had variable levels of proteinuria (1+ to 3+) and eleven had active urine sediment. Urine protein:creatinine ratios ranged from 0.2 to 1.7. Of the 6 patients who underwent kidney biopsy, all 6 showed pauci-immune crescentic glomerulonephritis. Seven patients had bilateral pulmonary interstitial infiltrates and four had pleural effusions on CT scan. Four had pericardial effusions as demonstrated by echocardiography.
CONCLUSIONS: Hydralazine-associated vasculitis is a drug-associated autoimmune syndrome that presents with interstitial lung disease, hypocomplementemia, and pauci-immune glomerulonephritis. Patients have elements of both DIL and DIV, as manifested by high ANA and ANCA titers as well as the presence of histone and MPO antibodies. Further research is needed to understand the etiopathogenesis of this condition.

PMID: 29519741 [PubMed - indexed for MEDLINE]

Giant cell arteritis and inflammatory bowel disease - Is there a connection? Results from a population-based study.

dim, 23/12/2018 - 09:14
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Giant cell arteritis and inflammatory bowel disease - Is there a connection? Results from a population-based study.

Autoimmun Rev. 2018 Nov;17(11):1134-1137

Authors: Yavne Y, Tiosano S, Ben-Ami D, Watad A, Guy A, Comaneshter D, Cohen AD, Amital H

Abstract
BACKGROUND: Giant cell arteritis (GCA) is an autoimmune disorder which primarily affects large vessels, whilst inflammatory bowel diseases (IBD) mainly target the gut. Co-existence of the two maladies has been reported sporadically in the literature; therefore the purpose of this study was to assess the authenticity of such an association in a large, cross-sectional study.
METHODS: Utilizing data derived from the Clalit Health Services' registry, the largest health maintenance organization in Israel, we compared the proportion of CD and UC in GCA patients with age- and gender-matched controls. Univariate analysis was performed using Chi-square and student t-test and a multivariate analysis was performed using a logistic regression model.
RESULTS: The study included 3938 GCA patients and 21,623 age- and gender-matched controls. GCA patients had a significantly increased proportion of both CD and UC in comparison with controls (0.79% vs. 0.12% and 0.84% vs. 0.21%, P-value < .001, respectively). The strength of the association between GCA and IBD was negatively correlated with the patients' age; thus the association was more robust amongst middle-aged patients (ages 50-69, OR = 8.13) than in elderly patients (ages 70-85, OR = 3.81). The association between GCA and IBD remained significant when evaluated independently of confounding factors (OR = 2.63, P-value < .001).
CONCLUSIONS: The probability that GCA patients also suffer from IBD is increased in comparison with age- and gender-matched controls. Our findings indicate that this association is more prominent in middle-aged patients (50-69 years of age). Screening for IBD amongst GCA patients in this age group may be warranted.

PMID: 30217549 [PubMed - indexed for MEDLINE]

Simultaneous Presentation of Giant Cell Arteritis and Myelodysplastic Syndrome in an Elderly Japanese Man.

jeu, 20/12/2018 - 08:47
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Simultaneous Presentation of Giant Cell Arteritis and Myelodysplastic Syndrome in an Elderly Japanese Man.

Intern Med. 2018 Oct 01;57(19):2889-2893

Authors: Senjo H, Higuchi T, Morimoto M, Koyamada R, Yanaoka C, Okada S

Abstract
An 81-year-old Japanese man presented with constitutional symptoms and anemia and was diagnosed with giant cell arteritis (GCA) and myelodysplastic syndrome (MDS) simultaneously. His symptoms and anemia improved promptly with steroids; however, the MDS rapidly progressed to overt leukemia. While MDS patients are at an increased risk of autoimmune diseases, an association with GCA has rarely been reported. This case illustrates the importance of considering GCA as a cause of anemia in elderly patients if MDS is already diagnosed, even in countries where the prevalence of GCA is very low. The simultaneous development of GCA and MDS suggests a common pathogenetic link between these two diseases.

PMID: 29780154 [PubMed - indexed for MEDLINE]

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

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.

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.

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.

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.

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.

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.

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.

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.

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]

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