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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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Severe ischemic cytomegalovirus proctocolitis with multiple perforation.

mar, 10/07/2018 - 21:02
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Severe ischemic cytomegalovirus proctocolitis with multiple perforation.

Arch Virol. 2018 Jul;163(7):1927-1931

Authors: D'cruz RT, Lau CC, Thamboo TP

Abstract
Cytomegalovirus (CMV) typically causes gastrointestinal infections in immunocompetent patients. Colonic perforations secondary to CMV are exceeding rare. We describe a 88-year-old male presenting with a week-long history of intractable abdominal discomfort, bloating, nausea and diarrhea. Flexible sigmoidoscopy revealed significant ulceration with yellowish slough. Emergency surgery was performed subsequently in view of multiple perforations in the rectosigmoid junction. CMV gastrointestinal infections demonstrated an ischemic process secondary to vasculitis, which accelerated the pathway to colonic perforation. CMV gastrointestinal infection should be considered as a differential diagnosis in patients with colonoscopy findings similar to ischemic colitis and Clostridium difficile infections.

PMID: 29532267 [PubMed - indexed for MEDLINE]

Cataract surgery combined with micro-incision vitrectomy in patients with behcet's disease uveitis.

sam, 07/07/2018 - 20:15
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Cataract surgery combined with micro-incision vitrectomy in patients with behcet's disease uveitis.

BMC Ophthalmol. 2018 Jun 28;18(1):158

Authors: Fan F, Jia Z, Li K, Zhao X, Ma Q

Abstract
BACKGROUND: This study sought to report the outcomes of a combined cataract extraction, intraocular lens (IOL) insertion and micro-incision vitrectomy (MIVS) procedure for the treatment of Behcet uveitis.
METHODS: This investigation involved the retrospective evaluation of a case series of patients with Behcet uveitis who underwent cataract extraction, IOL insertion and MIVS in a single surgical session at the same institution between January 2013 and November 2016. Outcome measures included visual acuity, inflammatory reaction, systemic anti-inflammatory medications, intraocular pressure (IOP) and complications.
RESULTS: Seven eyes of seven patients with a mean age of 39.00 ± 5.54 years (range, 32 to 48 years) and a mean follow-up duration of 13.57 ± 5.83 months (range, 6 to 24 months) were studied; five patients with a history of well-controlled uveitis were included. All patients underwent cataract extraction and IOL implantation combined with MIVS. All patients received postoperative steroids, which were slowly tapered during the weeks after surgery. There were no significant complications related to the surgery. Overall, best-corrected visual acuity (BCVA) was improved from log MAR (logarithm of the minimum angle of resolution) 1.67 ± 0.67 preoperatively to log MAR 0.74 ± 0.35 postoperatively; this improvement was statistically significant (p < 0.05). All eyes were deemed quiet at follow-up, and no patients required the escalation of therapy for long-term uveitis control.
CONCLUSIONS: This retrospective series indicates that a procedure that combines phacoemulsification, IOL implantation and MIVS is a feasible technique for the removal of cataracts and pathologic vitreous in eyes with Behcet uveitis. This approach can restore vision without obvious complications.

PMID: 29954349 [PubMed - indexed for MEDLINE]

The co-existence of Lemierre's syndrome and Bezold's abscesses due to Streptococcus constellatus: A case report.

sam, 07/07/2018 - 20:15
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The co-existence of Lemierre's syndrome and Bezold's abscesses due to Streptococcus constellatus: A case report.

Medicine (Baltimore). 2018 Jun;97(26):e11228

Authors: Yaita K, Sugi S, Hayashi M, Koga T, Ebata T, Sakai Y, Kaieda S, Ida H, Watanabe H

Abstract
RATIONALE: The ancient infectious diseases, Lemierre's Syndrome and Bezold's Abscesses are rare.
PATIENT CONCERNS: A 70-year-old Japanese woman with a 15-year history of Parkinson's disease was referred to our hospital due to fever, occipital headache and bilateral shoulder pain that had continued for three months. She had been prescribed prednisolone due to a diagnosis of polymyalgia rheumatica.
DIAGNOSES: A blood culture revealed bacteremia of Streptococcus constellatus. In addition, computed tomography revealed Bezold's abscesses and Lemierre's syndrome.
INTERVENTIONS: We administered ceftriaxone for 31 days, followed by oral amoxicillin.
OUTCOMES: The patient recovered and the abscesses improved.
LESSONS: This case underscores the importance of blood culture tests and cross-referencing with radiological imagings in the diagnoses of these rare critical infectious diseases that mimic polymyalgia rheumatica.

PMID: 29952983 [PubMed - indexed for MEDLINE]

Behçet disease associated with gastrointestinal amyloidosis manifested as hematochezia: A case report.

sam, 07/07/2018 - 20:15
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Behçet disease associated with gastrointestinal amyloidosis manifested as hematochezia: A case report.

Medicine (Baltimore). 2018 Jun;97(26):e11153

Authors: Sato S, Yashiro M, Matsuoka N, Kawana S, Asano T, Kobayashi H, Tasaki K, Watanabe H, Hashimoto Y, Migita K

Abstract
RATIONALE: Behçet disease (BD) is an inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, ocular lesions, and skin lesions. Complication of amyloidosis in patients with BD is rare. Here, we report a case of BD with immunoglobulin light chain (AL)-amyloidosis manifested as hematochezia.
PATIENT CONCERNS: A 61-year-old man developed sudden hematochezia due to bleeding from multiple small colonic ulcers; AL-amyloid deposition was found on immunohistochemical examination of biopsy specimen of colonic ulcer. Systemic investigation revealed cardiac disfunction with cardiomegaly and progressive renal dysfunction, which indicated the presence of systemic AL-amyloidosis.
DIAGNOSES: Based on the findings of colonic ulcers with cardiac and renal involvement, a diagnosis of systemic AL-amyloidosis complicated by incomplete BD was established.
INTERVENTIONS: He was treated with increased dose of oral prednisolone (20 mg/day), colchicine and mesalazine, because he was reluctant to receive aggressive chemotherapy (melphalan and dexamethasone) or autologous stem cell transplantation.
OUTCOMES: Colonic ulcers completely diminished after treatment, however, he died because of severe urinary tract infection and progressive renal failure after one year of gastrointestinal (GI) manifestations.
LESSONS: Our case shows that patients with BD may have GI manifestations due not only to entero-BD but also due to GI amyloidosis.

PMID: 29952963 [PubMed - indexed for MEDLINE]

Tongue Necrosis in Giant-Cell Arteritis.

sam, 07/07/2018 - 20:15
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Tongue Necrosis in Giant-Cell Arteritis.

N Engl J Med. 2018 Jun 28;378(26):2517

Authors: Truffaut L, Lefebvre P

PMID: 29949496 [PubMed - indexed for MEDLINE]

Case 20-2018: A 64-Year-Old Man with Fever, Arthralgias, and Testicular Pain.

sam, 07/07/2018 - 20:15
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Case 20-2018: A 64-Year-Old Man with Fever, Arthralgias, and Testicular Pain.

N Engl J Med. 2018 Jun 28;378(26):2518-2529

Authors: Sacks CA, Kilcoyne A, Wallace ZS, Glomski K

PMID: 29949483 [PubMed - indexed for MEDLINE]

Adalimumab therapy in hepatitis B virus-negative polyarteritis nodosa: A case report.

dim, 01/07/2018 - 17:05
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Adalimumab therapy in hepatitis B virus-negative polyarteritis nodosa: A case report.

Medicine (Baltimore). 2018 Jun;97(25):e11053

Authors: Wang CR, Yang CC

Abstract
INTRODUCTION: Polyarteritis nodosa (PAN) is a difficult-to-treat rheumatology disorder, and biologic agents have been used in such patients with a treatment failure or the disease relapse.
CASE PRESENTATION: We report a 42-year-old male Han Chinese who received adalimumab (ADA) alone during his vasculitis relapse, manifesting with weight loss, ankle arthritis, testicular inflammation and new-onset hypertension. Under the ADA 40 mg biweekly subcutaneous injection, there were the disappearance of clinical activity and no disease flares with a follow-up period of 26 months.
CONCLUSION: The clinical observation in this patient suggests a potential efficacy of ADA therapy in controlling the disease activity of PAN.

PMID: 29923995 [PubMed - indexed for MEDLINE]

Large-vessel vasculitis in human immunodeficiency virus-infected patients.

jeu, 28/06/2018 - 14:38
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Large-vessel vasculitis in human immunodeficiency virus-infected patients.

J Vasc Surg. 2018 05;67(5):1501-1511

Authors: Ferfar Y, Savey L, Comarmond C, Sadaghianloo N, Garrido M, Domont F, Valantin MA, Pourcher-Martinez V, Cluzel P, Fouret P, Chiche L, Gaudric J, Koskas F, Cacoub P, Saadoun D

Abstract
OBJECTIVE: The objective of this study was to describe large-vessel vasculitis (LVV) in patients with human immunodeficiency virus (HIV) infection. It is a retrospective single-center study conducted between 2000 and 2015 through a university hospital of 11 HIV-infected patients with LVV.
METHODS: The characteristics and outcome of 11 HIV-infected patients with LVV (7 patients fulfilled international criteria for Takayasu arteritis, 5 patients had histologic findings of vasculitis, and 5 patients had imaging features of aortitis) were analyzed and compared with those of 82 patients with LVV but without HIV infection.
RESULTS: Concerning the HIV-infected patients with LVV (n = 11), the mean age was 40 years (range, 36-56 years), and 55% of patients were female. At diagnosis of LLV, the mean initial CD4 cell count was 455 cells/mm3 (range, 166-837 cells/mm3), and the median HIV viral load was 9241 copies. Vascular lesions were located in the aorta (n = 7), in supra-aortic trunks (n = 7), and in digestive arteries (n = 3). Inflammatory aorta infiltrates showed a strong expression of interferon-γ and interleukin 6. In HIV-negative LVV patients (n = 82), the median age was 42 years, and 88% of the patients were women. Thirty patients had an inflammatory syndrome. Seventy patients had been treated with glucocorticosteroids and 57 with immunosuppressive treatments. Compared with their negative counterparts, HIV-positive patients with LVV were more frequently male (P = .014), had more vascular complications (ie, Ishikawa score; P = .017), and had more frequent revascularization (P = .047). After a mean follow-up of 96 months, four relapses of vasculitis were reported, and one patient died. Regardless of the HIV virologic response, antiretroviral therapy improved LVV in only one case.
CONCLUSIONS: LVV in HIV-infected patients is a rare and severe entity.

PMID: 29242069 [PubMed - indexed for MEDLINE]

Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis.

sam, 16/06/2018 - 06:56
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Pulmonary renal syndrome: A case report of diffuse alveolar hemorrhage in association with ANCA negative pauci-immune glomerulonephritis.

Medicine (Baltimore). 2018 Jun;97(23):e10954

Authors: Saladi L, Shaikh D, Saad M, Cancio-Rodriguez E, D'Agati VD, Medvedovsky B, Uday KA, Adrish M

Abstract
RATIONALE: Pulmonary renal syndrome (PRS) is a term most commonly used to describe a combination of glomerulonephritis and pulmonary hemorrhage as a manifestation of a multisystem autoimmune disease. It is usually associated with ANCA vasculitis and anti-GBM disease. Diffuse alveolar hemorrhage in a patient with ANCA and anti-GBM negative pauci-immune glomerulonephritis is rare and optimal management is unknown.
PATIENT CONCERNS: An 85-year-old man with hypertension, diabetes mellitus, prostate cancer and recently diagnosed pauci-immune necrotizing glomerulonephritis presented to our emergency department with worsening dyspnea and pedal edema for several days. Clinical presentation and radiological studies were suggestive of fluid overload but he developed worsening respiratory failure despite hemodialysis.
DIAGNOSES: Bronchoscopy confirmed diffuse alveolar hemorrhage. ANCA and anti-GBM antibodies were negative. The patient was diagnosed with pulmonary renal syndrome - diffuse alveolar hemorrhage in the setting of ANCA and anti-GBM negative pauci-immune glomerulonephritis.
INTERVENTIONS: Patient was started on intravenous pulse steroids, cyclophosphamide and received seven sessions of plasmapheresis.
OUTCOMES: There was an improvement in patient's respiratory status and repeat bronchoscopy at the end of treatment did not show diffuse alveolar hemorrhage.
LESSONS: Pauci-immune crescentic necrotizing glomerulonephritis is usually associated with the presence of ANCA, however, ANCA may be absent in 10% of these cases. Immunosuppression is the mainstay of treatment for ANCA and anti-GBM associated PRS. This case highlights the importance of immunosuppression and plasmapheresis in patients with ANCA negative vasculitis due to presence of unidentified serum antibodies. If left untreated, these patients can have a fulminant course with high mortality ranging from 25 to 50%.

PMID: 29879042 [PubMed - indexed for MEDLINE]

A Rare Cause of Gastrointestinal Bleeding and Rash in an Older Woman.

sam, 16/06/2018 - 06:56
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A Rare Cause of Gastrointestinal Bleeding and Rash in an Older Woman.

Gastroenterology. 2018 May;154(6):1592-1593

Authors: Neviackas J, Kotwal V, Attar B

PMID: 29614299 [PubMed - indexed for MEDLINE]

Progressive outer retinal necrosis after rituximab and cyclophosphamide therapy.

mer, 13/06/2018 - 06:44
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Progressive outer retinal necrosis after rituximab and cyclophosphamide therapy.

Indian J Ophthalmol. 2018 04;66(4):591-593

Authors: Dogra M, Bajgai P, Kumar A, Sharma A

Abstract
We report a case of progressive outer retinal necrosis (PORN) in a patient of microscopic polyangitis (MPA), being treated with immunosuppressive drugs such as cyclophosphamide and rituximab. Her aqueous tap was positive for Varicella Zoster virus and she was treated with oral and intravitreal antivirals, along with discontinuation of one of the immunosuppressive agents, i.e. rituximab, which might have led to reactivation of the virus causing necrotizing retinitis lesions. Rituximab and cyclophosphamide are extremely potent drugs, which are necessary to manage immunological disorders such as MPA. However, they may predispose the patient to serious complications like viral infections, including PORN.

PMID: 29582832 [PubMed - indexed for MEDLINE]

A case of inclusion body myositis complicated by microscopic polyangiitis.

mer, 13/06/2018 - 06:44
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A case of inclusion body myositis complicated by microscopic polyangiitis.

Scand J Rheumatol. 2018 05;47(3):243-245

Authors: Yamada S, Yamashita H, Taira K, Hida A, Arai N, Shimizu J, Miyaji Y, Sonoo M, Yashima A, Takahashi Y, Kaneko H

PMID: 28726537 [PubMed - indexed for MEDLINE]

Successful treatment with ustekinumab for corticosteroid- and immunosuppressant-resistant Takayasu's arteritis.

mer, 13/06/2018 - 06:44
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Successful treatment with ustekinumab for corticosteroid- and immunosuppressant-resistant Takayasu's arteritis.

Scand J Rheumatol. 2018 05;47(3):246-247

Authors: Yachoui R, Kreidy M, Siorek M, Sehgal R

PMID: 28276951 [PubMed - indexed for MEDLINE]

Retinal Vasculitis in Takayasu's Arteritis.

jeu, 07/06/2018 - 05:26
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Retinal Vasculitis in Takayasu's Arteritis.

N Engl J Med. 2018 May 24;378(21):e28

Authors: Bajgai P, Singh R

PMID: 29791818 [PubMed - indexed for MEDLINE]

Delta Neutrophil Index Is Associated with Vasculitis Activity and Risk of Relapse in ANCA-Associated Vasculitis.

lun, 04/06/2018 - 04:48
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Delta Neutrophil Index Is Associated with Vasculitis Activity and Risk of Relapse in ANCA-Associated Vasculitis.

Yonsei Med J. 2018 May;59(3):397-405

Authors: Yoo J, Ahn SS, Jung SM, Song JJ, Park YB, Lee SW

Abstract
PURPOSE: Delta neutrophil index (DNI) represents the immature granulocytes count associated with neutrophil-consumption. We investigated whether DNI might be associated with Birmingham vasculitis activity score (BVAS) at diagnosis and could predict relapse during the follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV).
MATERIALS AND METHODS: We reviewed the medical records of 97 patients having DNI results. Twenty patients had granulomatosis with polyangiitis (GPA), 58 had microscopic polyangiitis (MPA), and 19 had eosinophilic GPA (EGPA). We collected clinical and laboratory data including BVAS, five factor score (FFS), and DNI. The correlation coefficient and cumulative relapse free survival rate were obtained. The optimal cut-off of DNI was extrapolated by calculating the area under the receiver operator characteristic curve.
RESULTS: DNI was significantly related to cross-sectional BVAS. Furthermore, among continuous variables, only DNI could reflect BVAS of GPA and MPA, but not EGPA. Severe AAV was defined as BVAS ≥20 (the highest quartile). At diagnosis, patients having DNI ≥0.65% had a significantly higher risk of severe GPA and MPA than those having not (relative risk 4.255) at diagnosis. During the follow-up, DNI ≥0.65% could predict the higher relapse rate.
CONCLUSION: DNI could reflect BVAS at diagnosis and furthermore, DNI ≥0.65% could not only identify severe AAV at diagnosis, but also predict relapse during the follow-up in patients with GPA and MPA.

PMID: 29611402 [PubMed - indexed for MEDLINE]

Evaluation of efficacy of infliximab for retinal vasculitis and extraocular symptoms in Behçet disease.

lun, 04/06/2018 - 04:48
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Evaluation of efficacy of infliximab for retinal vasculitis and extraocular symptoms in Behçet disease.

Jpn J Ophthalmol. 2018 May;62(3):390-397

Authors: Umazume A, Kezuka T, Usui Y, Suzuki J, Goto H

Abstract
PURPOSE: We evaluated ocular symptoms and activity of retinal vasculitis of Behçet disease before and after infliximab therapy, using Behçet disease ocular attack score 24 (BOS24) and fluorescein angiography (FA) score. We also analyzed the efficacy of infliximab for ocular and extraocular symptoms.
STUDY DESIGN: Retrospective study.
SUBJECT AND METHODS: Using medical records, we analyzed FA and BOS24 to evaluate the association between the efficacy of infliximab therapy and FA as well as BOS24 scores. Further, we evaluated the association between FA scores and extraocular symptoms.
RESULTS: After 2 years of infliximab therapy, 6-month BOS24 was significantly reduced compared to that before treatment (PreBOS24-6M). After 4 years of infliximab therapy, 6-month BOS24 was also significantly reduced compared to preBOS24-6M. After 2 years of infliximab therapy, FA score median (interquartile range) decreased significantly compared to that before treatment [FA-2Y vs. preFA: 0 (0-0) vs.15.5 (12-24); P < 0.0001; n = 38]. The FA-4Y score in subjects followed for at least 4 years was also significantly lower than the preFA score [0 (0-0) vs.16.5 (12-24.5); P < 0.0001; n = 28]. Among 38 patients, extraocular symptoms were resolved following treatment in 29 cases (76.3%). No significant correlation was observed between the improvement in FA-2Y and FA-4Y scores and the occurrence and persistence of extraocular symptoms (P = 0.33; n = 38 or P = 0.4; n = 28).
CONCLUSION: Infliximab therapy is effective for the treatment of ocular and extraocular involvement of Behçet disease. BOS24 and FA scores are useful for evaluating the efficacy of infliximab therapy.

PMID: 29582190 [PubMed - indexed for MEDLINE]

Anti-interleukin 6 receptor tocilizumab in refractory uveitis associated with Behçet's disease: multicentre retrospective study.

lun, 04/06/2018 - 04:48
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Anti-interleukin 6 receptor tocilizumab in refractory uveitis associated with Behçet's disease: multicentre retrospective study.

Rheumatology (Oxford). 2018 05 01;57(5):856-864

Authors: Atienza-Mateo B, Calvo-Río V, Beltrán E, Martínez-Costa L, Valls-Pascual E, Hernández-Garfella M, Atanes A, Cordero-Coma M, Miquel Nolla J, Carrasco-Cubero C, Loricera J, González-Vela MC, Vegas-Revenga N, Fernández-Díaz C, Demetrio-Pablo R, Domínguez-Casas LC, Luis Martín-Varillas J, Palmou-Fontana N, Hernández JL, González-Gay MÁ, Blanco R

Abstract
Objective: To assess the efficacy of tocilizumab (TCZ) in refractory uveitis of Behçet's disease (BD).
Methods: Multicentre study of patients with BD-associated uveitis. Patients were refractory to conventional and biologic immunosuppressive drugs. The main outcome measures were intraocular inflammation, macular thickness, visual acuity and corticosteroid-sparing effects.
Results: We studied 11 patients (7 men) (20 affected eyes); median age 35 years. Uveitis was bilateral in nine patients. The patterns of ocular involvement were panuveitis (n = 8, with retinal vasculitis in 4), anterior uveitis (n = 2) and posterior uveitis (n = 1). Cystoid macular oedema was present in seven patients. The clinical course was recurrent (n = 7) or chronic (n = 4). Before TCZ, patients had received systemic corticosteroids, conventional immunosuppressants and the following biologic agents: adalimumab (n = 8), infliximab (n = 4), canakimumab (n = 1), golimumab (n = 3), etanercept (n = 1). TCZ was used as monotherapy or combined with conventional immunosuppressants at 8 mg/kg/i.v./4 weeks (n = 10) or 162 mg/s.c./week (n = 1). At TCZ onset the following extraocular manifestations were present: oral and/or genital ulcers (n = 7), arthritis (n = 4), folliculitis/pseudofolliculitis (n = 4), erythema nodosum (n = 2), livedo reticularis (n = 1) and neurological involvement (n = 2). TCZ yielded rapid and maintained improvement in all ocular parameters of the patients, with complete remission in eight of them. However, this was not the case for the extraocular manifestations, since TCZ was only effective in three of them. After a mean (s.d.) follow-up of 9.5 (8.05) months, TCZ was withdrawn in two cases, due to a severe infusion reaction and arthritis impairment, respectively.
Conclusion: TCZ could be a therapeutic option in patients with BD and refractory uveitis.

PMID: 29471416 [PubMed - indexed for MEDLINE]

Secukinumab for the maintenance of glucocorticoid-free remission in a patient with giant cell arteritis and psoriatic arthritis.

lun, 04/06/2018 - 04:48
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Secukinumab for the maintenance of glucocorticoid-free remission in a patient with giant cell arteritis and psoriatic arthritis.

Rheumatology (Oxford). 2018 May 01;57(5):934-936

Authors: Rotar Ž, Tomšic M, Hocevar A

PMID: 29394408 [PubMed - indexed for MEDLINE]

Tocilizumab and refractory Takayasu disease: Four case reports and systematic review.

ven, 01/06/2018 - 04:01
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Tocilizumab and refractory Takayasu disease: Four case reports and systematic review.

Autoimmun Rev. 2018 Apr;17(4):353-360

Authors: Decker P, Olivier P, Risse J, Zuily S, Wahl D

Abstract
BACKGROUND: Relapses upon corticosteroids tapering and immunosuppressive agents are frequent in Takayasu arteritis (TA). Interleukin-6 is highly involved in physiopathology of TA. Many reports showed efficacy of tocilizumab (TCZ) in refractory TA cases. We report four cases and an updated literature review on the TCZ efficacy and safety in patients with TA.
METHODS: Patients with TA defined by ACR 1990 criteria were included. Clinical, biological and imaging data were retrospectively reported. Disease activity was analyzed before TCZ and during the follow-up. Medline database was searched for systematic literature review.
RESULTS: One hundred and five patients (median age 28years [22-38]) were included, mostly refractory cases (76 patients, 72%). Median TCZ duration was 12months [6-20]. Among 105 patients, 90 patients (85.7%) had an initial clinical response within three months [3-6] and 43/66 patients (65.2%) had a radiological improvement. Only seven patients (9%) showed relapse on therapy. Corticosteroid dose reduction was obtained in 75/83 patients (90.4%). Relapse after TCZ discontinuation was observed in six patients (46%), with a median time of five months [2-9]. Twenty-four side-effects were noted in 18 patients (18%), with TCZ interruption in seven cases (7%): 10 infections, five cytopenia, six hepatitis, one pancreatitis, one cutaneous rash and one breast cancer.
CONCLUSIONS: This review confirms that TCZ is safe and effective in refractory cases of TA and TCZ is a corticosteroid-sparing therapy in patients with or without previous TNFα blockers therapy. However relapses after TCZ discontinuation are frequent.

PMID: 29427826 [PubMed - indexed for MEDLINE]

Large-vessel involvement and aortic dilation in giant-cell arteritis. A multicenter study of 549 patients.

ven, 01/06/2018 - 04:01
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Large-vessel involvement and aortic dilation in giant-cell arteritis. A multicenter study of 549 patients.

Autoimmun Rev. 2018 Apr;17(4):391-398

Authors: de Boysson H, Daumas A, Vautier M, Parienti JJ, Liozon E, Lambert M, Samson M, Ebbo M, Dumont A, Sultan A, Bonnotte B, Manrique A, Bienvenu B, Saadoun D, Aouba A

Abstract
OBJECTIVES: Large-vessel involvement (LVI) can occur in giant-cell arteritis (GCA) and may represent a distinct disease subgroup with a higher risk for aortic dilation. This study aimed to better characterize the presentation and evolution of LVI in patients with GCA.
PATIENTS AND METHODS: A retrospective multicenter study enrolled 248 GCA patients with LVI and 301 GCA patients without LVI on imaging. Factors associated with aortic dilation were identified in a multivariable model.
RESULTS: The patients with LVI were younger (p<0.0001), more likely to be women (p=0.01), and showed fewer cephalic symptoms (p<0.0001) and polymyalgia rheumatica (p=0.001) but more extracranial vascular symptoms (p=0.05) than the patients without LVI. Glucocorticoids (GC) management did not differ between the two groups, but the GC discontinuation rate was lower in the patients with LVI (p=0.0003). Repeated aortic imaging procedures were performed at 19months [range: 5-162months] and 17months [range: 6-168months] after diagnosis in 154 patients with LVI and 123 patients without LVI, respectively, of whom 21% and 7%, respectively, presented new aortic dilations (p=0.0008). In the patients with LVI, aortic dilation occurred on an aorta segment shown to be inflammatory on previous imaging in 94% of patients. In the multivariate analysis, LVI was the strongest predictor of aortic dilation (hazard ratio: 3.16 [range: 1.34-7.48], p=0.009).
CONCLUSIONS: LVI represents a distinct disease pattern of GCA with an increased risk of aortic dilation. Control of the aortic morphology during follow-up is required.

PMID: 29427822 [PubMed - indexed for MEDLINE]

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