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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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Disseminated toxoplasmosis with atypical symptoms which developed with exacerbation of systemic lupus erythematosus.

mer, 16/01/2019 - 20:50
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Disseminated toxoplasmosis with atypical symptoms which developed with exacerbation of systemic lupus erythematosus.

Lupus. 2019 Jan;28(1):133-136

Authors: Furuya H, Ikeda K, Iida K, Suzuki K, Furuta S, Tamachi T, Suzuki K, Miura G, Hiraguri M, Hase R, Hikosaka K, Norose K, Nakajima H

Abstract
Toxoplasma is a common parasite worldwide that mainly affects the brain, lungs and eyes. Although toxoplasmic encephalitis is a lethal disease without treatment, past case reports show most patients with systemic lupus erythematosus who developed toxoplasmic encephalitis were misdiagnosed and treated as neuropsychiatric systemic lupus erythematosus, which led to unfavorable outcomes. We herein describe a case of disseminated toxoplasmosis affecting all the above organs with atypical symptoms, which developed with exacerbation of systemic lupus erythematosus. She had initially manifested with retinochoroiditis without vitritis, mild cognitive impairment and an isolated lung mass. These are completely different from the classic symptoms of toxoplasmosis that have been reported in patients with HIV infection and/or those after hematopoietic transplantation. Our case, together with previously reported cases, suggests the manifestation of toxoplasmosis that develops in systemic lupus erythematosus patients can be different from that seen in conventional cases and varies between individual patients. Our case highlights both the difficulty in and the importance of diagnosing toxoplasmosis in patients with systemic lupus erythematosus and provides helpful information to identify this rare, devastating, yet treatable disease.

PMID: 30486727 [PubMed - indexed for MEDLINE]

Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis
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mer, 16/01/2019 - 20:50
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Bacterial endocarditis manifesting as autoimmune pulmonary renal syndrome: ANCA-associated lung hemorrhage and pauci-immune crescentic glomerulonephritis
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Clin Nephrol. 2018 Dec;90(6):431-433

Authors: Mohandes S, Satoskar A, Hebert L, Ayoub I

Abstract
The etiology of pulmonary renal syndrome can be broadly divided into infectious and autoimmune (predominantly ANCA vasculitis). The importance of timely differentiating between them stems from the deleterious effects of their respective treatment if misdiagnosed. Serology and tissue evaluation by pathology are employed to aid in this, however, in rare cases, this can be difficult. We present a case of infectious endocarditis that presented with pulmonary renal syndrome but had positive ANCA serology and a pauci-immune glomerulonephritis picture on kidney biopsy that posed diagnostic difficulty. Factors most helpful in differentiating between the two conditions are highlighted as well as treatment options.
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PMID: 30369400 [PubMed - indexed for MEDLINE]

ANCA-associated vasculitis in scleroderma: A renal perspective
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mer, 16/01/2019 - 20:50
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ANCA-associated vasculitis in scleroderma: A renal perspective
.

Clin Nephrol. 2018 Dec;90(6):413-418

Authors: Kant S, Shah AA, Hummers LK, Wigley FM, Geetha D

Abstract
AIMS: Overlap syndrome of ANCA-associated vasculitis (AAV) and scleroderma (SSc) is rare with conflicting data on renal outcomes. We describe the clinical characteristics and treatment outcome of ANCA-associated glomerulonephritis (AAG) in SSc patients followed at a single center.
MATERIALS AND METHODS: We conducted a retrospective study of 3,570 patients in our SSc database to identify SSc patients who subsequently developed AAV with renal involvement. Patient demographics, serology, renal function, renal histology, and treatment outcomes were assessed.
RESULTS: Of the 3,570 patients, we identified 7 patients who developed acute glomerulonephritis, and all were ANCA positive. The mean age at SSc diagnosis was 47 years, 4 patients were female, and 6 had diffuse SSc. Anti-nuclear antibody (ANA) was positive in all. Mean time of onset of AAV from time of diagnosis of SSc was 6 years, and all were myeloperoxidase (MPO) positive. Patients presented with hematuria, proteinuria, with or without rise in serum creatinine, and all patients had biopsy-proven crescentic glomerulonephritis. One patient required dialysis at presentation. Five patients were treated with cyclophosphamide and steroids, and 2 were treated with rituximab and steroids. Of the 7 patients, 4 did not receive maintenance immunosuppression. Three patients died, and 1 of them experienced relapse with fulminant alveolar hemorrhage.
CONCLUSION: AAG in SSc is rare, with disease manifestation and course similar to that of AAV. This case series demonstrates that disease remission can be achieved with standard induction therapy. Vasculitis relapse can occur, and similar to idiopathic AAV, maintenance immunosuppression should be initiated to maintain remission.
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PMID: 30106367 [PubMed - indexed for MEDLINE]

Necrosis of the Fingers and Toes.

dim, 13/01/2019 - 18:22
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Necrosis of the Fingers and Toes.

N Engl J Med. 2018 Dec 27;379(26):2557

Authors: Taniguchi Y, Inotani S

PMID: 30586520 [PubMed - indexed for MEDLINE]

Phenotypic Characterization of Circulating CD4+ T Cells in ANCA-Associated Vasculitis.

dim, 13/01/2019 - 18:22
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Phenotypic Characterization of Circulating CD4+ T Cells in ANCA-Associated Vasculitis.

J Immunol Res. 2018;2018:6984563

Authors: Lilliebladh S, Johansson Å, Pettersson Å, Ohlsson S, Hellmark T

Abstract
T cell-mediated immune responses are thought to play an important role in the pathogenesis of anti-neutrophil cytoplasmic antibody- (ANCA-) associated vasculitides (AAV). CD4+ T cells can be divided into subsets depending on their expression of chemokine receptors. In this study, different CD4+ T cell populations in patients with AAV were analysed and compared to healthy blood donors as well as therapy controls. 18 patients with active AAV, 46 in remission, 21 healthy controls (HBD), and 15 therapy controls (TC) were enrolled. CD4+ T cells were divided into Th1, Th2, and Th17 cells and further subdivided into naïve, central memory, effector memory, and effector cells. Regulatory T cells were also analysed. Concentrations of cytokines and chemokines produced by the respective CD4+ T cell subset in plasma from 33 of the patients were measured by ELISA and compared to HBD. Clinical data were collected on all patients. CCL20 concentrations and percentages of Th17 cells (p = 0.019) were elevated in AAV patients compared to HBD. AAV patients had lower percentages of naïve CD4+ T cells (p = 0.0016) and a corresponding increase in proportion of effector memory CD4+ T cells when comparing to HBD (p = 0.027). Therapy controls showed similar results as AAV patients. In this study, we found that CD4+ T cell phenotype distribution is altered in AAV patients, in line with previously published work. However, no differences were found between AAV patients and TC, stressing the importance of treatment impact on this kind of studies.

PMID: 30510966 [PubMed - indexed for MEDLINE]

A 19-year old man with IgA vasculitis after vaccination.

dim, 13/01/2019 - 18:22
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A 19-year old man with IgA vasculitis after vaccination.

Braz J Infect Dis. 2018 Sep - Oct;22(5):442-444

Authors: Malek A, Gomez-Villegas SI, de la Hoz A, Nowbakht C, Arias CA

Abstract
A 19-year-old patient who mistakenly received two doses of influenza vaccine 10 days before presentation, was admitted with malaise, weakness, and a purpuric non-blanching rash most prominent on the ankles followed by abdominal pain and hematochezia 72h later. The diagnosis of influenza vaccine-related Henoch-Schonlein vasculitis was made. This complication, although rare, is the most common vasculitis related to immunization.

PMID: 30389353 [PubMed - indexed for MEDLINE]

Ustekinumab for refractory giant cell arteritis: A prospective 52-week trial.

dim, 13/01/2019 - 18:22
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Ustekinumab for refractory giant cell arteritis: A prospective 52-week trial.

Semin Arthritis Rheum. 2018 12;48(3):523-528

Authors: Conway R, O'Neill L, Gallagher P, McCarthy GM, Murphy CC, Veale DJ, Fearon U, Molloy ES

Abstract
OBJECTIVES: Giant cell arteritis (GCA) is the most common form of systemic vasculitis. Glucocorticoids are an effective treatment but have significant adverse events and relapses are common. Interleukins 12 (IL-12) and 23 (IL-23) stimulate TH1 and TH17 responses and are implicated in the pathogenesis of GCA. The aim of this study was to evaluate the efficacy and safety of IL-12/23 blockade with ustekinumab in GCA.
METHODS: We performed a prospective open label study of ustekinumab in patients with refractory GCA. Ustekinumab 90mg was administered subcutaneously every 12 weeks. The primary outcome was the comparison of the median glucocorticoid dose prior to commencement of ustekinumab and at 52 weeks. Secondary outcomes included physician assessed relapse, acute phase reactants, and imaging assessment of large vessel vasculitis (LVV).
RESULTS: Twenty-five GCA patients received ustekinumab. All patients had failed to taper glucocorticoids despite addition of a median of 1 other immunosuppressive agent. At week 52, median (IQR) daily prednisolone dose decreased from 20 (15, 25)mg to 5 (2.5, 5)mg (p < 0.001). Six patients (24%) stopped prednisolone completely. No patient experienced a relapse of GCA while receiving ustekinumab. Median (IQR) CRP decreased significantly from 12.9 (5.3, 42) to 6 (2.6, 12.5)mg/L (p = 0.006). CT angiography demonstrated improvement of LVV in all patients studied. No unexpected adverse events were observed with ustekinumab.
CONCLUSIONS: Ustekinumab may be effective for the treatment of GCA and warrants further assessment in a randomized controlled trial.

PMID: 29776658 [PubMed - indexed for MEDLINE]

Antineutrophil cytoplasmic antibody-positive familial Mediterranean fever and hyperthyroidism: A case report.

jeu, 10/01/2019 - 15:33
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Antineutrophil cytoplasmic antibody-positive familial Mediterranean fever and hyperthyroidism: A case report.

Medicine (Baltimore). 2018 Dec;97(51):e13805

Authors: Segoe S, Sada KE, Hayashi K, Yamamura Y, Morishita M, Watanabe H, Matsumoto Y, Wada J

Abstract
RATIONALE: Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder characterized by serositis and recurrent fever. Previous reports identified patients with antineutrophil cytoplasmic antibody (ANCA)-positive FMF, but vasculitis symptoms were not reported.
PATIENT CONCERNS: We report the case of a 44-year-old man with numbness. He had a history of 3 episodes of pleurisy and was being treated with propylthiouracil for hyperthyroidism. Because he was ANCA-positive, we suspected drug-induced ANCA-associated vasculitis and propylthiouracil was discontinued. However, his numbness was not ameliorated, and he again developed high fever with pleurisy.
DIAGNOSIS: Diagnosis of FMF was finally made, and genetic analysis revealed compound heterozygous mutations in exon 2 of the familial Mediterranean fever gene (L110P/E148Q).
INTERVENTIONS: The patient was treated with 0.5 mg/day of colchicine.
OUTCOMES: His numbness improved, and fever has not recurred.
LESSONS: Appearance of ANCA and development of vasculitis should be considered in a clinical course of FMF with hyperthyroidism.

PMID: 30572542 [PubMed - indexed for MEDLINE]

Sudden unilateral vision loss in an adult with Behcet disease: A case report.

jeu, 10/01/2019 - 15:33
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Sudden unilateral vision loss in an adult with Behcet disease: A case report.

Medicine (Baltimore). 2018 Dec;97(50):e13594

Authors: Gao Y, Lyu S, Bao L, Zhang M

Abstract
RATIONALE: Behcet disease is an autoimmune vasculitis, involving multiple systems and organs. It is often chronic and recurrent, and involves all levels of arteries and veins.
PATIENT CONCERNS: A 40-year-old Chinese male with sudden vision loss in his right eye for 3 days was reported.
DIAGNOSES: The patient was diagnosed as bilateral uveitis (obstructive retinal vasculitis in the right eye) and Behcet disease.
INTERVENTIONS: The patient was given topical corticosteroids and compound tropicamide eye drops in our department. In addition, he was also hospitalized in the Rheumatology Department, and received systemic infusion of methylprednisolone and cyclophosphamide.
OUTCOMES: Best corrected visual acuity vision acuity of the left eye was 20/20 and that of the right eye was no light perception after 2 weeks. During the telephonic follow-up after 1 year, the patient reported 2 recurrent episodes of uveitis in the left eye, which improved after treatment at the local hospital.
LESSONS: It cautions ophthalmologists of the high possibility of vasculitis in young and middle-aged patients with retinal vascular occlusion. It also reminds the ophthalmologists investigate systemic diseases, and obtain detailed medical history, not just depending on specific laboratory findings. This case further suggests that Behcet disease has diverse manifestations, complex diagnosis, and severe lesions, and the recurrence rate must be controlled to minimize irreversible consequences such as sudden vision loss during recurrence.

PMID: 30558031 [PubMed - indexed for MEDLINE]

Onset of Takotsubo Syndrome during the Clinical Course of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Report.

jeu, 10/01/2019 - 15:33
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Onset of Takotsubo Syndrome during the Clinical Course of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Report.

Tohoku J Exp Med. 2018 12;246(4):251-256

Authors: Harada M, Ichikawa T, Kobayashi M

Abstract
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis resulting in severe organ injuries. ANCA is a disease-labeled antibody of AAV, and myeloperoxidase (MPO) and proteinase 3 are the main targeted antigens of ANCA. Takotsubo syndrome, a transient cardiac dysfunction caused by emotional or physical stress, is characterized by ST-segment elevation and negative T waves in electrocardiogram, transient left ventricular asynergy, and absence of obstructive coronary disease. To the best of our knowledge, only two cases of coexistence of AAV and takotsubo syndrome have been reported. Herein, we report the case of AAV complicated with takotsubo syndrome. A 78-year-old Japanese woman presented with severe renal dysfunction, which was diagnosed as MPO-ANCA-associated systemic vasculitis. Despite the treatment with cyclophosphamide and glucocorticoid, the patient presented with severe respiratory failure due to alveolar hemorrhage and heart failure. Electrocardiography indicated newly developed T wave inversions. Echocardiography demonstrated severe left ventricular dysfunction with hypokinesis of the apical area. Moreover, coronary angiography revealed no noticeable stenotic or obstructive lesions. These findings indicate the onset of takotsubo syndrome. After immunosuppressive therapy, systemic vasculitis and takotsubo syndrome were improved. Although a coexisting case of AAV and takotsubo syndrome is rare, we have to consider the possible complication of takotsubo syndrome in case of presenting acute heart failure. Considering the present case and the previously reported coexisting cases of takotsubo syndrome and AAV, we propose that female sex, initiation of glucocorticoid therapy, and high titer of MPO-ANCA are potential risk factors of developing takotsubo syndrome.

PMID: 30555102 [PubMed - indexed for MEDLINE]

Central Retinal Arterial Occlusion in Granulomatosis with Polyangiitis.

jeu, 10/01/2019 - 15:33
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Central Retinal Arterial Occlusion in Granulomatosis with Polyangiitis.

Korean J Ophthalmol. 2018 12;32(6):519-520

Authors: Shim KY, Sohn SI, Kim YC

PMID: 30549477 [PubMed - indexed for MEDLINE]

A Surprising Cause for Intestinal Obstruction.

lun, 07/01/2019 - 14:46
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A Surprising Cause for Intestinal Obstruction.

Gastroenterology. 2018 12;155(6):e1-e3

Authors: Gherardi BE, Milito CB, Parente DB

PMID: 30205099 [PubMed - indexed for MEDLINE]

Simultaneous bilateral posterior ischemic optic neuropathy secondary to giant cell arteritis: a case presentation and review of the literature.

ven, 04/01/2019 - 13:04
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Simultaneous bilateral posterior ischemic optic neuropathy secondary to giant cell arteritis: a case presentation and review of the literature.

BMC Ophthalmol. 2018 Dec 12;18(1):317

Authors: Albarrak AM, Mohammad Y, Hussain S, Husain S, Muayqil T

Abstract
BACKGROUND: This report highlights a rare case of simultaneous bilateral blindness due to posterior ischemic optic neuropathy. Typically, ophthalmic involvement in giant cell arteritis is monocular or sequential ischemia of the anterior portion of the optic nerve, and less frequently simultaneous.
CASE PRESENTATION: An 80-year-old Saudi male came with a history of simultaneous bilateral vision loss 5 days prior to presentation. The exam showed dilated non-reactive pupils, no light perception in both eyes, and normal fundus exam. C-reactive protein and erythrocyte sedimentation rate levels were high Magnetic resonance imaging and magnetic resonance angiography of the brain showed a right posterior optic nerve lesion and absence of flow in both ophthalmic arteries respectively. A left temporal artery biopsy confirmed giant cell arteritis.
CONCLUSION: The presentation of GCA can be atypical and patients may present with simultaneous blindness. Bilateral simultaneous PION does not exclusively occur in a post surgical setting, emphasizing the importance of decreasing the threshold of suspicion of similar cases to avoid further neurological complications.

PMID: 30541489 [PubMed - indexed for MEDLINE]

Palisaded neutrophilic and granulomatous dermatitis associated with systemic lupus erythematosus: possible involvement of CD163+ M2 macrophages in two cases, and a review of published works.

ven, 04/01/2019 - 13:04
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Palisaded neutrophilic and granulomatous dermatitis associated with systemic lupus erythematosus: possible involvement of CD163+ M2 macrophages in two cases, and a review of published works.

Lupus. 2018 Dec;27(14):2220-2227

Authors: Terai S, Ueda-Hayakawa I, Nguyen CTH, Ly NTM, Yamazaki F, Kambe N, Son Y, Okamoto H

Abstract
BACKGROUND: Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a commonly occurring condition related to systemic autoimmune disease. It is characterized histopathologically by a distinct pattern of granulomatous inflammation in the presence or absence of leukocytoclastic vasculitis. The properties of granulomatous cells in PNGD are still uncertain.
OBJECTIVE: We sought further investigation on the phenotype of the infiltrated cells in PNGD from two patients with systemic lupus erythematosus (SLE) and reviewed the previous published reports in order to provide a comprehensive summary on the clinical features of PNGD in SLE.
METHODS: The immunohistochemical features of granulomatous cells in PNGD associated with SLE were analyzed. Immunohistochemical studies were performed on sections from our two cases using antibodies against CD68, CD163, CD15, Factor XIIIa, myeloperoxidase and neutrophil elastase. The clinical characteristics of the SLE patients who developed PNGD were also evaluated. We included all cases retrieved through a PubMed search with the key words PNGD and SLE.
RESULTS: Cutaneous lesions consisted of erythematous plaques distributed on the face and upper limbs in both cases. The infiltrated cells were mainly positive for CD68 and CD163, a phenotype suggestive of M2 macrophages. Some mature neutrophils and lymphocytes were also present. A review of the literature of PNGD associated with SLE revealed a predominance in females, high prevalence of lupus nephritis and a good response to systemic steroids, with frequent skin lesions relapses during tapering of the treatment.
LIMITATIONS: This study examined only two cases; the pathogenesis of the disease remains to be clarified.
CONCLUSION: PNGD lesions were abundantly infiltrated by M2 macrophages, suggesting that they may have a role in this condition. SLE accompanied by PNGD might be associated with lupus nephritis and frequent relapses of skin lesions.

PMID: 30376790 [PubMed - indexed for MEDLINE]

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]

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