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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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Management of amphotericin-induced phlebitis among HIV patients with cryptococcal meningitis in a resource-limited setting: a prospective cohort study.

jeu, 22/08/2019 - 23:43
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Management of amphotericin-induced phlebitis among HIV patients with cryptococcal meningitis in a resource-limited setting: a prospective cohort study.

BMC Infect Dis. 2019 Jun 26;19(1):558

Authors: Ahimbisibwe C, Kwizera R, Ndyetukira JF, Kugonza F, Sadiq A, Hullsiek KH, Williams DA, Rhein J, Boulware DR, Meya DB

Abstract
BACKGROUND: Amphotericin-induced phlebitis is a common infusion-related reaction in patients managed for cryptococcal meningitis. High-quality nursing care is critical component to successful cryptococcosis treatment. We highlight the magnitude and main approaches in the management of amphotericin-induced phlebitis and the challenges faced in resource-limited settings.
METHODS: We prospectively determined the incidence of amphotericin-induced phlebitis during clinical trials in Kampala, Uganda from 2013 to 2018. We relate practical strategies and challenges faced in clinical management of phlebitis.
RESULTS: Overall, 696 participants were diagnosed with HIV-related cryptococcal meningitis. Participants received 7-14 doses of intravenous (IV) amphotericin B deoxycholate 0.7-1.0 mg/kg/day for induction therapy through peripheral IV lines at a concentration of 0.1 mg/mL in 5% dextrose. Overall, 18% (125/696) developed amphotericin-induced phlebitis. We used four strategies to minimize/prevent the occurrence of phlebitis. First, after every dose of amphotericin, we gave one liter of intravenous normal saline. Second, we rotated IV catheters every three days. Third, we infused IV amphotericin over 4 h. Finally, early ambulation was encouraged to minimize phlebitis. To alleviate phlebitis symptoms, warm compresses were used. In severe cases, treatment included topical diclofenac gel and oral anti-inflammatory medicines. Antibiotics were used only when definite signs of infection developed. Patient/caregivers' education was vital in implementing these management strategies. Major challenges included implementing these interventions in participants with altered mental status and limited access to topical and oral anti-inflammatory medicines in resource-limited settings.
CONCLUSIONS: Amphotericin-induced phlebitis is common with amphotericin, yet phlebitis is a preventable complication even in resource-limited settings.
TRIAL REGISTRATION: The ASTRO-CM trial was registered prospectively. ClincalTrials.gov : NCT01802385 ; Registration date: March 1, 2013; Last verified: February 14, 2018.

PMID: 31242860 [PubMed - indexed for MEDLINE]

Immune keratitis: An unusual primary presentation of neuro-Behçet's disease.

lun, 19/08/2019 - 21:47
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Immune keratitis: An unusual primary presentation of neuro-Behçet's disease.

Eur J Ophthalmol. 2019 Jul;29(4):NP5-NP8

Authors: Jinagal J, Agarwal A, Negi A, Gupta G, Sharma V, Gupta PC, Ram J

Abstract
A 19-year-old Nepalese male presented with complaints of bilateral ocular discomfort, photophobia, watering, and redness for 1 week. Visual acuity was 6/12 and 6/60 in the right and left eye, respectively. On biomicroscopic examination, presence of peripheral stromal infiltrates with conjunctival follicles was noted; infiltrates progressed to involve central cornea with further decrease in vision over next few days. After ruling out infectious keratitis, detailed systemic examination and laboratory investigations were diagnostic of neuro-Behçet's disease. Patient responded to systemic steroidal and immunosuppressive therapy characterized by corneal healing and visual acuity improvement to 6/6 and 6/9. Although rare, but neuro-Behçet's disease can primarily present as bilateral immune keratitis and every case of bilateral keratitis needs early systemic evaluation after ruling out infective etiologies.

PMID: 30295071 [PubMed - indexed for MEDLINE]

Invasive meningococcal disease in patients with complement deficiencies: a case series (2008-2017).

ven, 16/08/2019 - 20:42
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Invasive meningococcal disease in patients with complement deficiencies: a case series (2008-2017).

BMC Infect Dis. 2019 Jun 14;19(1):522

Authors: Ladhani SN, Campbell H, Lucidarme J, Gray S, Parikh S, Willerton L, Clark SA, Lekshmi A, Walker A, Patel S, Bai X, Ramsay M, Borrow R

Abstract
BACKGROUND: To describe patients with inherited and acquired complement deficiency who developed invasive meningococcal disease (IMD) in England over the last decade.
METHODS: Public Health England conducts enhanced surveillance of IMD in England. We retrospectively identified patients with complement deficiency who developed IMD in England during 2008-2017 and retrieved information on their clinical presentation, vaccination status, medication history, recurrence of infection and outcomes, as well as characteristics of the infecting meningococcal strain.
RESULTS: A total of 16 patients with 20 IMD episodes were identified, including four with two episodes. Six patients had inherited complement deficiencies, two had immune-mediated conditions associated with complement deficiency (glomerulonephritis and vasculitis), and eight others were on Eculizumab therapy, five for paroxysmal nocturnal haemoglobinuria and three for atypical haemolytic uraemic syndrome. Cultures were available for 7 of 11 episodes among those with inherited complement deficiencies/immune-mediated conditions and the predominant capsular group was Y (7/11), followed by B (3/11) and non-groupable (1/11) strains. Among patients receiving Eculizumab therapy, 3 of the 9 episodes were due to group B (3/9), three others were NG but genotypically group B, and one case each of groups E, W and Y.
CONCLUSIONS: In England, complement deficiency is rare among IMD cases and includes inherited disorders of the late complement pathway, immune-mediated disorders associated with low complement levels and patients on Eculizumab therapy. IMD due to capsular group Y predominates in patient with inherited complement deficiency, whilst those on Eculizumab therapy develop IMD due to more diverse capsular groups including non-encapsulated strains.

PMID: 31200658 [PubMed - indexed for MEDLINE]

Wegener's Granulomatosis Presenting as Wallenberg Syndrome: A Case Report.

ven, 16/08/2019 - 20:42
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Wegener's Granulomatosis Presenting as Wallenberg Syndrome: A Case Report.

J Stroke Cerebrovasc Dis. 2019 Aug;28(8):e107-e109

Authors: Emamikhah M, Sina F, Mokhtari M, Shirani F, Asadipanah M

Abstract
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, a vasculitis affecting small and medium sized vessels usually affects the upper and lower respiratory tract, the kidneys, and the eyes. Neurologic manifestation in central nervous system (CNS) is less frequent than the peripheral and usually is in form of stroke. Few cases of lateral medullary ischemic stroke (Wallenberg syndrome) due to GPA have been reported. A 41 year-old female, presented with acute vertigo, nausea/vomiting, hiccups, dysphagia. In physical examination she had a saddled nose, horner syndrome, soft palate paralysis, crossed hypoesthesia of face, and limbs and hemi-ataxia. Brain magnetic imaging revealed a left lateral medullary infarction and sinusitis confirmed by paranasal Sinus CT scans. Chest CT showed a cavitary mass. Laboratory findings were remarkable for anemia, elevated erythrocyte sedimentation rate, and C-reactive protein. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA)/anti-proteinase 3 was positive. Diagnosis of GPA was established and treatment was started. During 6-month follow-up improvement was satisfying and no relapses occurred. Medullary infarct is reported in few GPA patients, especially at presentation. Definite diagnosis is based on tissue biopsy. Although in context of extra CNS involvement and positive ANCA diagnosis can be made confidently. Treatment of choice in CNS involvement is not clear, corticosteroids and immunosuppressives seem effective. CNS involvement, especially stroke may present GPA or accompany extra CNS symptoms. Prompt diagnosis and treatment is essential.

PMID: 31027983 [PubMed - indexed for MEDLINE]

Endovascular Recanalization of Thromboangiitis Obliterans (Buerger's Disease) in Twenty-Eight Consecutive Patients and Combined Antegrade-Retrograde Intervention in Eight Patients.

ven, 16/08/2019 - 20:42
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Endovascular Recanalization of Thromboangiitis Obliterans (Buerger's Disease) in Twenty-Eight Consecutive Patients and Combined Antegrade-Retrograde Intervention in Eight Patients.

Cardiovasc Intervent Radiol. 2019 Jun;42(6):820-828

Authors: Firat A, Igus B

Abstract
PURPOSE: The aim of the study was to evaluate the technical success of the procedure and the clinical efficacy of treatment in patients with thromboangiitis obliterans (TAO) (Buerger's disease) based on a change in the Rutherford classification.
MATERIALS AND METHODS: A total of 28 consecutive patients (26 males, 2 females, mean age 43.3 ± 5.32 years) underwent endovascular recanalization with a diagnosis of TAO, between April 2015 and July 2018. After unsuccessful attempts using the antegrade approach, retrograde approaches were used in 8 patients under ultrasound guidance. Clinical follow-up was routinely performed at 1-month, 3-month, 6-month, and 1-year intervals.
RESULTS: A total of 28 TAO patients underwent 40 procedures in 32 limbs. Technical success was achieved in 28 of the 32 limbs (87.5%). In total, 45 of 59 (76.2%) below the knee arteries were treated successfully. One major amputation was performed, providing a 96.8% rate for limb salvage both at 12 and 24 months. Amputation-free survival estimated by Kaplan-Meier analysis was 84% at 12 and 24 months. Primary patency rates at 12, 24, and 36 months were 84%, 78%, and 75%, respectively. Secondary patency rates were 87.5% both at 12 and 24 months.
CONCLUSION: Endovascular treatment is a technically feasible and potentially effective treatment modality for Buerger's disease. Combined antegrade and retrograde interventions in TAO patients may improve technical success and clinical recovery, especially in cases where the antegrade approach has failed.

PMID: 30834476 [PubMed - indexed for MEDLINE]

Golfer's vasculitis.

ven, 16/08/2019 - 20:42
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Golfer's vasculitis.

Vasc Med. 2019 06;24(3):265-266

Authors: Li W, Moran A, Kim ES

PMID: 30813867 [PubMed - indexed for MEDLINE]

Splinter haemorrhages, splenic infarcts, and pulmonary embolism in granulomatosis with polyangiitis.

ven, 16/08/2019 - 20:42
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Splinter haemorrhages, splenic infarcts, and pulmonary embolism in granulomatosis with polyangiitis.

Vasc Med. 2019 06;24(3):263-264

Authors: Laurent C, Dion J, Régent A

PMID: 30426847 [PubMed - indexed for MEDLINE]

Effects of incision location on specimen quality and complications for temporal artery biopsy.

sam, 10/08/2019 - 18:46
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Effects of incision location on specimen quality and complications for temporal artery biopsy.

Vascular. 2019 08;27(4):347-351

Authors: Czyz CN, Allen JB, Cahill KV, Nabavi CB, Foster JA

PMID: 31313985 [PubMed - indexed for MEDLINE]

Audiological and vestibular measurements in Behçet's disease.

sam, 10/08/2019 - 18:46
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Audiological and vestibular measurements in Behçet's disease.

Eur Arch Otorhinolaryngol. 2019 Jun;276(6):1625-1632

Authors: Ertugrul O, Mutlu A, Zindanci I, Cam OH, Ozluoglu L

Abstract
INTRODUCTION: Behçet's disease (BD) is a vasculitis that involves all small vessels and influences the multiple systems of the human body. This study aimed to evaluate the audio-vestibular system involvement of patients with BD and healthy individuals.
MATERIALS AND METHODS: This study was designed as a prospective case-control blinded study. Thirty-one patients with BD and 31 healthy individuals were included. All the subjects were evaluated via pure tone audiometry (PTA), video head impulse test (vHIT), post head shake nystagmus test (PHSNT) and dizziness handicap inventory (DHI) to check for audio-vestibular system involvement.
RESULTS: Patients with BD showed higher PTA scores in both speech and high frequencies. The vHIT revealed pathological saccades, particularly in horizontal canals (right ear: p = 0.002, left ear: p = 0.039). The gain values of the patients were slightly lower than those of the control group; however, gain and gain asymmetry differed significantly in a few canals. In the spontaneous nystagmus test and PHSNT, pathological nystagmus was detected to be significantly higher in the patient group than control group (p = 0.001); but the saccade presence in vHIT and nystagmus in PHNT did not differ among the patients (p = 0.106). In addition, the DHI scores of the patients group were higher than those of the control group (p < 0.001). No correlation was found between disease duration and saccade presence.
CONCLUSION: The vHIT was used preliminary for evaluating the vestibular system in BD. This study showed the influence of BD on the audio-vestibular system, in particular isolated horizontal canal involvement was discovered in patients with BD.
LEVEL OF EVIDENCE: Level III b.

PMID: 30929056 [PubMed - indexed for MEDLINE]

Endovascular Repair of Inflammatory Aortic Aneurysms: Experience in a Single Center.

mer, 07/08/2019 - 17:24
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Endovascular Repair of Inflammatory Aortic Aneurysms: Experience in a Single Center.

Ann Vasc Surg. 2019 Jul;58:255-260

Authors: Cardaci MB, Destraix R, Van Houte B, Vazquez C

Abstract
BACKGROUND: Inflammatory abdominal aortic aneurysm (IAAA) remained a rare cause of aneurysmal aortic disease, with incidences between 5% and 10%. The current treatment for IAAA consists of open surgical repair and endovascular aneurysm repair (EVAR). Avoiding an inflamed, fibrotic retroperitoneum is the driving force behind the desire to repair IAAA endovascularly. The latest published works confirm the promising results after EVAR for IAAA, but there is still a paucity of data regarding hydroureter and hydronephrosis. In this article, we describe our experience with 5 patients diagnosed with IAAA and treated by EVAR, of whom 3 presented with associated hydronephrosis.
METHODS: A retrospective review of our endovascular database identified five patients who underwent EVAR for IAAA. Unilateral ureteral involvement in the inflammatory process was seen in 3 patients, accompanied by secondary hydronephrosis. One patient presented retroperitoneal fibrosis with duodenal stenosis. Primary outcomes were primary technical success, aneurysm-related mortality, change in aneurysm size, perianeurysmal fibrosis (PAF), and hydronephrosis. Secondary outcomes were requirement for reintervention, progression/resolution of symptoms, and short-term clinical success.
RESULTS: Follow-up duration ranged from 3 to 61 months. No patients were lost to follow-up. Primary technical success was obtained in all patients. One patient died three months after the operation due to persistence of the duodenal stenosis in spite of subsequent endoscopic treatments and corticotherapy. The aneurysm sac progressively reduced in 4 patients and remained unchanged in one patient. PAF regressed in 2 patients, reduced in two, and remained unchanged in one patient. Hydronephrosis persisted in all three patients preoperatively diagnosed with this condition. No patient required subsequent intervention. Four patients had complete resolution of their symptoms at a follow-up visit at 1 month.
CONCLUSIONS: This series suggests that EVAR for IAAA is technically feasible, excludes the aneurysm effectively, and reduces PAF with an acceptable morbidity and mortality rate. EVAR does not seem to offer any benefits for hydronephrosis, and closer follow-up in patients presenting renal or ureter involvement treated by EVAR is necessary.

PMID: 30735769 [PubMed - indexed for MEDLINE]

A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

mer, 07/08/2019 - 17:24
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A new perspective for analyzing clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome.

Int Ophthalmol. 2019 Jul;39(7):1475-1482

Authors: Liu XC, Zhang MN, Chen B, Li Y, Huang HB

Abstract
PURPOSE: We aimed to analyze the clinical characteristics of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Furthermore, we aimed to correlate the number and location of retinal aneurysms with the size of retinal non-perfusion area and neovascularization.
METHODS: Six patients with IRVAN syndrome (1 male and 5 females, age 5-38 years) were enrolled in this study. Fundus fluorescein angiography (FFA) was used to determine the total number of retinal aneurysms, number of aneurysms within the first branch of the retinal artery, minimum distance between the non-perfusion margin and the optic disc, and the number of retinal aneurysms in each quadrant, as well as the type of neovascularization.
RESULTS: The size of the non-perfusion area was positively correlated with the total number of retinal aneurysms, the number of aneurysms within the first branch of the retinal artery, and the number of retinal aneurysms in each quadrant (P < 0.05). During the 5-year follow-up, one patient exhibited a dynamic change in the number and location of retinal aneurysms.
CONCLUSIONS: In IRVAN syndrome, the number and location of retinal aneurysms correlate with the size of retinal non-perfusion area and type of neovascularization.

PMID: 29934930 [PubMed - indexed for MEDLINE]

Aorta coarctation and systemic lupus erythematosus: A case report.

jeu, 01/08/2019 - 13:01
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Aorta coarctation and systemic lupus erythematosus: A case report.

Medicine (Baltimore). 2019 Jul;98(28):e16397

Authors: Li J, Wang X, Zhang H, Wang W, Pei Y, Xie T, Lin W, Zuo X, Zhang W

Abstract
RATIONALE: Vasculitis is one of the common pathological hallmarks of systemic lupus erythematosus (SLE). Vascular lesions in SLE commonly involve medium- and small-sized vessels. Rarely, vasculitis in SLE may involve large vessels such as the aorta leading to life-threatening complications. Reported cases of large vessel lesions in SLE included aortic aneurysm and aortic dissection.
PATIENT CONCERNS: Here, we report a 52-year-old Chinese woman with SLE, who was stable on oral glucocorticoid, but showed sudden intractable hypertension and heavy proteinuria before we found aorta coarctation in her computed tomography (CT) scan of the aorta.
DIAGNOSES: This patient's large vascular lesions were likely secondary and not a primary manifestation of lupus activity.
INTERVENTIONS AND OUTCOMES: After endovascular stent graft repair of the abdominal aorta, her hypertension and proteinuria were controlled.
LESSONS: In the context of reported cases of large vessel lesions in SLE, our case further supports the significance of having a wide differential for vascular lesions in SLE, especially when an SLE patient presents sudden hypertension and heavy proteinuria. This case also demonstrates that vascular lesions in SLE may lead to serious, potentially fatal consequences.

PMID: 31305448 [PubMed - indexed for MEDLINE]

Association between Drug Use and In-hospital Outcomes after Infrainguinal Bypass for Peripheral Arterial Occlusive Disease.

jeu, 01/08/2019 - 13:01
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Association between Drug Use and In-hospital Outcomes after Infrainguinal Bypass for Peripheral Arterial Occlusive Disease.

Ann Vasc Surg. 2019 Jul;58:122-133.e4

Authors: Dakour-Aridi H, Arora M, Nejim B, Locham S, Malas MB

Abstract
BACKGROUND: Drug abuse may affect lower extremity vessels due to ischemia following intra-arterial injections, vasospasm, arterial and venous pseudoaneurysms, arteriovenous fistulae, vasculitis, and complicated abscesses. Little is known about the outcomes of lower extremity bypass (LEB) for peripheral arterial disease (PAD) in patients with a history of drug abuse disorder. The aim of this study is to evaluate the outcomes of LEB in this patient population.
METHODS: A retrospective study of the Premier Healthcare Database 2009-2015 was performed. In-hospital complications, mortality, and hospitalization costs were assessed in patients with a history of drug abuse disorder (opioids, cannabis, cocaine, sedatives/hypnotics/anxiolytics, and hallucinogens/methamphetamine/psychoactive drugs) who underwent LEB for PAD. Multivariable logistic and generalized linear models were utilized to study the association between drug use/misuse and in-hospital outcomes after LEB.
RESULTS: Our cohort included 50,976 patients, of which 967 (2%) had a history of drug abuse disorder on admission. The majority of drugs were cannabis (38.5%), followed by opioids (21.5%) and cocaine (14.5%). Patients with a history of drug use/misuse were significantly at a higher risk of developing complications during their hospital stay (71.9% vs. 64.2%, P < 0.001) including acute renal failure (11.8% vs. 9.1%), stroke (1.6% vs. 0.6%), respiratory complications (pneumonia and respiratory failure) (15.0% vs. 9.6%), hemorrhage/shock (36.2% vs. 31.8%), vascular or graft-related complications (29.8% vs. 26.4%), wound complications (9.1% vs. 6.3%), cellulitis (8.5% vs. 6.8%), and sepsis (2.1% vs. 1.2%, all P < 0.001). In addition, drug users were found to have higher risk of concomitant major amputations compared to nondrug users (2.0% vs. 0.9%, P < 0.001). On multivariable analysis, no difference was noted between the 2 groups in terms of in-hospital mortality and concomitant major amputations. However, drug use/misuse was associated with 57% higher odds of overall in-hospital complications (odds ratio [OR] 1.57, 95% confidence interval [CI] 1.34-1.83, P < 0.001), a prolonged length of hospital stay (median: 7 days vs. 5 days in nonabusers, P < 0.001), and higher hospitalization costs compared to nonusers (adjusted mean difference: OR $3,075, 95% CI $2,096-$4,055, P < 0.001).
CONCLUSIONS: Drug use/misuse is significantly associated with increased odds of in-hospital complications, longer hospital stays, and higher hospitalization costs following LEB. Vascular surgeons need to pay special attention to this patient population and explore interventions to decrease the morbidity and economic burden associated with drug use.

PMID: 30769072 [PubMed - indexed for MEDLINE]

A Long Interval from a Spinal Cord Lesion to a Subsequent Brain Lesion in Primary Central Nervous System Vasculitis.

jeu, 01/08/2019 - 13:01
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A Long Interval from a Spinal Cord Lesion to a Subsequent Brain Lesion in Primary Central Nervous System Vasculitis.

Intern Med. 2019 May 15;58(10):1485-1489

Authors: Kon T, Funamizu Y, Suzuki C, Sato T, Kurotaki H, Kurihara A, Kurose A, Wakabayashi K, Tomiyama M

Abstract
Primary central nervous system vasculitis (PCNSV) is an uncommon vasculitis restricted to the small- and medium-sized vessels in the brain and spinal cord. Previously, only 9 cases have been reported that initially manifested as an isolated spinal cord lesion with subsequent brain involvement, where the longest interval from the onset to brain involvement was 1 year and 11 months. We herein report the case of an isolated spinal cord lesion with subsequent brain involvement appearing seven years and five months later. This case shows that brain lesions can develop after an extended interval from spinal onset in PCNSV.

PMID: 30449796 [PubMed - indexed for MEDLINE]

Type I cryoglobulinemic vasulitis with eosinophilia: A case report and literature review.

ven, 26/07/2019 - 09:21
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Type I cryoglobulinemic vasulitis with eosinophilia: A case report and literature review.

Medicine (Baltimore). 2019 Jul;98(28):e16382

Authors: Wen J, Xu F, Li M, Zhou Q, Qu W, Liu Y, Su J, Hu H

Abstract
RATIONALE: Type I monoclonal cryoglobulinemia is usually associated with lymphoproliferative disorders, such as monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukemia (CLL) and lymphoplasmocytic lymphoma (LPL). Clinical symptoms related to Type I cryoglobulin (CG) isotype often include skin, neurological and renal manifestations.
PATIENT CONCERNS: A 42-year-old woman who initially presented urticaria, palpable purpura in both her upper extremities and legs, eosinophilia and Raynaud phenomenon. Skin biopsy revealed eosinophil infiltration. Monoclonal immunoglobulin (Ig) G-κprotein was detected and CG was also positive.
DIAGNOSES: The patient was finally diagnosed as MGUS related Type I CG.
INTERVENTIONS: Cyclophosphamide-dexamethasone-thalidomide (CDT) therapy was initiated.
OUTCOMES: The treatment relieved the skin symptoms efficiently.
LESSONS: To our knowledge, this is a rare case of Type I cryoglobulinemic vasulitis with eosinophilia complicated by MGUS, and the effective treatment of cyclophosphamide combined with thalidomide and prednisone may provide a new therapeutic option for cryoglobulinemic vasulitis.

PMID: 31305439 [PubMed - indexed for MEDLINE]

Exclusively ocular and cardiac manifestation of granulomatosis with polyangiitis - a case report.

ven, 26/07/2019 - 09:21
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Exclusively ocular and cardiac manifestation of granulomatosis with polyangiitis - a case report.

BMC Ophthalmol. 2019 Jun 28;19(1):139

Authors: Rogaczewska M, Puszczewicz M, Stopa M

Abstract
BACKGROUND: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibodies (ANCA)-associated necrotizing granulomatous vasculitis that affects small to medium size vessels. While the classical form with renal and respiratory tract involvement is mainly seen, a limited form (i.e., with no renal disease) may also occur. We present an unusual case of GPA manifesting merely as a bilateral ocular involvement and complete heart block.
CASE PRESENTATION: We report a case of a 60-year-old male patient with a limited form of GPA who initially presented with bilateral chronic conjunctivitis and complete atrioventricular block. His visual acuity subsequently declined due to progression to bilateral panuveitis with exudative retinal detachment. The laboratory investigation revealed the elevation of acute phase reactants and strongly positive cytoplasmic ANCA (c-ANCA). Despite negative conjunctival and musculocutaneous biopsy results, the positive c-ANCA, and the clinical manifestation, i.e., heart and ocular involvement, led to the diagnosis of GPA. The remission was achieved with cyclophosphamide and methylprednisolone systemic therapy.
CONCLUSIONS: A limited form of GPA may be a diagnostic chameleon. Though rare, it is essential to consider even extremely uncommon findings. Our patient is the first case of such a unique demonstration of the limited GPA manifesting as a bilateral ocular involvement and complete heart block.

PMID: 31253129 [PubMed - indexed for MEDLINE]

Primary angiitis of the Central Nervous Sistem: 25 years of follow-up and mild motor sequelae.

ven, 26/07/2019 - 09:21
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Primary angiitis of the Central Nervous Sistem: 25 years of follow-up and mild motor sequelae.

Neurologia. 2019 May;34(4):276-278

Authors: Domínguez RO, Perdomo Rojas LM, Gonzalez SE, Bartolomé EL

PMID: 27788936 [PubMed - indexed for MEDLINE]

Intrathecal inflammation in young stroke.

sam, 20/07/2019 - 03:31
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Intrathecal inflammation in young stroke.

Acta Neurol Scand. 2019 Jul;140(1):9-16

Authors: Süße M, Hannich MJ, Holbe C, Ruhnau J, von Sarnowski B, Dressel A

Abstract
OBJECTIVES: Correct identification of inflammatory etiologies of stroke is of outmost importance as they require treatment of the underlying disease. Aim of this study was to determine the prevalence of inflammatory changes in cerebrospinal fluid (CSF) observed in young cryptogenic stroke patients.
MATERIALS AND METHODS: Of 6476 records of patients diagnosed with ischemic stroke, 278 had confirmed ischemia in brain imaging and received lumbar puncture. A total of 122 were classified as young stroke (≤55 years), and 156 were classified as older stroke patients; lumbar puncture in this cohort was indicated due to atypical clinical presentation.
RESULTS: An infectious etiology was detected in 2.5% of young stroke patients (n = 3: vasculitis due to opportunistic infection, vasculitis due to neuroborreliosis, secondary vasospasm after viral meningitis) and in 1.9% (n = 3) in the older stroke cohort (vasculitis due to neurotuberculosis, septic embolic ischemia, vasculitis post-haemophilus influenza meningoencephalitis). Isolated vasculitis was evident in one patient of the older stroke cohort (0.6%). Non-specific alterations in CSF included increased cell count in 10% in young and in 9.3% in the older stroke cohort. Intrathecal Ig synthesis was present in 3.4% of the younger and in 4% of the older stroke cohort.
CONCLUSIONS: The prevalence of an infectious etiology in young stroke is modest but slightly higher in comparison with older stroke patients. As brain imaging is not always sufficient for suspecting vasculitis, we recommend implementation of lumbar puncture in young cryptogenic stroke patients. If an infectious disease is present in ischemic stroke, it is of high therapeutic relevance.

PMID: 30939222 [PubMed - indexed for MEDLINE]

Diffusion-weighted Whole-body Imaging with Background Body Signal Suppression (DWIBS) as a Novel Imaging Modality for Disease Activity Assessment in Takayasu's Arteritis.

sam, 13/07/2019 - 23:38
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Diffusion-weighted Whole-body Imaging with Background Body Signal Suppression (DWIBS) as a Novel Imaging Modality for Disease Activity Assessment in Takayasu's Arteritis.

Intern Med. 2019 May 01;58(9):1355-1360

Authors: Oguro E, Ohshima S, Kikuchi-Taura A, Murata A, Kuzuya K, Okita Y, Matsuoka H, Teshigawara S, Yoshimura M, Yoshida Y, Isoda K, Kudo-Tanaka E, Harada Y, Kaminou T, Saeki Y

Abstract
A 26-year-old woman with Takayasu's arteritis (TAK) experienced back and neck pain during tocilizumab (TCZ) treatment. The levels of C-reactive protein were normal, and ultrasonography revealed no significant changes. Diffusion-weighted whole-body imaging with background body signal suppression (DWIBS) showed signal enhancement in the walls of several arteries. Contrast computed tomography showed arterial inflammation in the same lesion. After increasing the dose of prednisolone and TCZ, all signal enhancements decreased and continued to decrease, as observed on days 76 and 132. Thus, DWIBS may be a novel imaging modality for assessing the disease activity of TAK, particularly during follow-up.

PMID: 30626815 [PubMed - indexed for MEDLINE]

Diagnostic value of the interferon-γ release assay for tuberculosis infection in patients with Behçet's disease.

dim, 07/07/2019 - 20:12
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Diagnostic value of the interferon-γ release assay for tuberculosis infection in patients with Behçet's disease.

BMC Infect Dis. 2019 Apr 15;19(1):323

Authors: Wu X, Chen P, Wei W, Zhou M, Li C, Liu J, Zhao L, Zhang L, Zhao Y, Zeng X, Liu X, Zheng W

Abstract
BACKGROUND: To investigate the diagnostic value of the interferon-γ release assay (IGRA) for detecting tuberculosis (TB) infection in patients with Behçet's disease (BD).
METHODS: We retrospective analyzed the data collected from 173 BD patients hospitalized between 2010 and 2015. Ninety-nine healthy volunteers were enrolled as a control group. IGRA was performed using T-SPOT.TB. The diagnosis of active TB (ATB) was based on clinical, radiological, microbiological, histopathological information and the response to anti-TB therapy. Latent TB (LTB) infection was defined as asymptomatic patients with positive T-SPOT.TB.
RESULTS: TB infection was documented in 59 BD patients (34.1%). The sensitivity, specificity, positive predictive value, negative predictive value, positive likelihood ratio and negative likelihood ratio of T-SPOT.TB for the diagnosis of ATB were 88.9%, 74.8%, 29.1%, 98.3%, 3.53 and 0.15, respectively. The receiver-operating-characteristic curve demonstrated that spot-forming cells (SFCs) of 70/106 PBMC was the optimal cutoff for diagnosing ATB, with an area under the curve of 0.891. Furthermore, the median SFCs in ATB group was significantly higher than those in LTB infection (466/106 PBMC vs. 68/106 PBMC, p = 0.007) or previous TB infection (466/106 PBMC vs. 96/106 PBMC, p = 0.018). A significant discrepancy between T-SPOT.TB and tuberculin skin test was noted (kappa coefficient = 0.391, p = 0.002).
CONCLUSIONS: T-SPOT.TB, an IGRA, may assist in the diagnosis of ATB in BD patients, and the higher SFCs suggest ATB in BD patients.

PMID: 30987605 [PubMed - indexed for MEDLINE]

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