Age and sex-specific associations of carotid pulsatility with small vessel disease burden in transient ischemic attack and ischemic stroke.

Background Although large artery stiffness has been implicated in the pathogenesis of cerebral small vessel disease, whether carotid pulsatility, a convenient surrogate marker of arterial stiffness, is similarly associated with global burden of small vessel disease is unknown. Aims To determine the age and sex-specific associations of carotid pulsatility with global burden of small vessel disease. Methods We studied consecutive patients with transient ischemic attack or non-disabling ischemic stroke from the Oxford Vascular Study who had a brain MRI and carotid duplex ultrasound during 2002-2014. We determined clinical correlates of common carotid artery (CCA) and internal carotid artery (ICA) pulsatility index (PI) and their associations with the total small vessel disease score on MRI, stratified by age (median = 72). Results In 587 patients, correlates of CCA and ICA-PI were both independently associated with age, diabetes, and premorbid mean pulse pressure after adjustment for age, sex, and cardiovascular risk factors (all p < 0.05). ICA-PI was strongly associated with small vessel disease markers and burden, particularly lacunes, in patients aged<70 (age and sex-adjusted odds ratio of top vs. bottom pulsatility index quartile: 5.35, 1.95-14.70, p = 0.001; increasing small vessel disease score: 2.30, 1.01-5.25, p = 0.048), but not in patients aged ≥ 70 ( p > 0.05). No associations between CCA-PI with small vessel disease score were noted at any age. In 94 consecutive patients who also received transcranial Doppler ultrasound, strong associations between middle cerebral artery (MCA)-PI and an increasing small vessel disease score were noted (unadjusted OR-MCA: 4.26, 1.45-12.55, p = 0.009; ICA: 2.37, 0.81-6.87, p = 0.11; CCA: 1.33, 0.45-3.96, p=0.61). Conclusions ICA and MCA-PI are associated with global small vessel disease burden, especially in individuals aged<70 and may be causally related.

Treatment of digital ulcers in systemic sclerosis: Case series study of thirteen patients and discussion on outcome.

INTRODUCTION:: In systemic sclerosis (SSc), digital ulcers (DU) are debilitating and recurrent. They are markers of prognosis and are associated with disability and mortality. Treatment strategies have been developed to block the proposed mechanisms of this complication. OBJECTIVE:: Clinical description of a population of SSc patients with DU, treatment, complications and outcome. METHOD:: Analysis of 48 SSc patients meeting 2013 ACR-EULAR criteria, followed between 1999-2015; 13 patients had DU. Treatment protocol applied included cycles of 21 days of alprostadil, which can be repeated in the absence of DU healing. After DU healing, bosentan was initiated. RESULTS:: DU healing was achieved with intravenous prostanoid in 12 patients; seven patients required repeated treatment for DU healing. Twelve patients were later treated with bosentan; three of them experienced recurrence of DU, while one was anti-B2-GPI positive. Four patients had soft tissue loss and three other suffered digital amputation, these being late diagnosis. CONCLUSION:: Younger patients and early referrals had better outcomes. Endothelin receptor antagonist toxicity should be monitored, particularly in patients previously exposed to hepatotoxic drugs.

Treatment of digital ulcers in systemic sclerosis: Case series study of thirteen patients and discussion on outcome / Tratamento de úlceras digitais na esclerose sistêmica: série de casos de 13 doentes e análise crítica dos resultados

Summary Introduction: In systemic sclerosis (SSc), digital ulcers (DU) are debilitating and recurrent. They are markers of prognosis and are associated with disability and mortality. Treatment strategies have been developed to block the proposed mechanisms of this complication. Objective: Clinical description of a population of SSc patients with DU, treatment, complications and outcome. Method: Analysis of 48 SSc patients meeting 2013 ACR-EULAR criteria, followed between 1999-2015; 13 patients had DU. Treatment protocol applied included cycles of 21 days of alprostadil, which can be repeated in the absence of DU healing. After DU healing, bosentan was initiated. Results: DU healing was achieved with intravenous prostanoid in 12 patients; seven patients required repeated treatment for DU healing. Twelve patients were later treated with bosentan; three of them experienced recurrence of DU, while one was anti-B2-GPI positive. Four patients had soft tissue loss and three other suffered digital amputation, these being late diagnosis. Conclusion: Younger patients and early referrals had better outcomes. Endothelin receptor antagonist toxicity should be monitored, particularly in patients previously exposed to hepatotoxic drugs.

Resumo Introdução: As úlceras digitais (UD) são complicações incapacitantes e recorrentes, associadas a menor qualidade de vida e maior mortalidade na esclerose sistêmica (ES). O tratamento baseia-se em antagonizar os mecanismos fisiopatológicos em causa. Objetivo: Descrever uma amostra de doentes com diagnóstico de ES e UD, o tratamento, as complicações e os resultados clínicos. Método: Série de 48 casos diagnosticados com ES, critérios de classificação ACR-EULAR 2013, seguidos entre 1999 e 2015, dos quais 13 apresentavam UD. O protocolo aplicado incluía ciclos de 21 dias de alprostadil podendo ser repetidos no caso de não existir cicatrização. Nos casos em que houve cicatrização foi iniciado bosentano. Resultados: No tratamento das UD, 12 doentes realizaram prostaciclina endovenosa, com necessidade de tratamentos repetidos em sete doentes. Doze doentes foram posteriormente tratados com bosentano, com recorrência de UD em três doentes, um deles com presença de anti-B2-GPI. Quatro doentes ficaram com cicatrizes e em três houve amputação digital, sendo casos de diagnóstico tardio. Conclusão: Os doentes mais jovens tiveram melhores resultados, possivelmente em razão de melhorias globais nos cuidados de saúde prestados e de referenciação precoce. A toxicidade dos antagonistas dos receptores da endotelina deve ser monitorizada, sobretudo em doentes com exposição prévia a drogas hepatotóxicas.

Thrombolysis in Patients Aged over 80 Years Is Equally Effective and Safe.

BACKGROUND: Despite stroke's high prevalence in the elderly, intravenous thrombolysis is licensed in Europe only for patients younger than 80 years old. We aimed to compare the functional outcomes and complication rates in patients older versus younger than 80 years old treated with intravenous thrombolysis. METHODS: A retrospective observational study of patients who received intravenous thrombolysis in a stroke unit between January 1, 2009, and June 30, 2012, was conducted. Variables were compared between 2 subgroups (≤80 and >80 years). RESULTS: Overall, 512 patients underwent intravenous thrombolysis, of which 13.1% were over 80 years. The mean age was 65.4 years in the younger subgroup and 82.9 years in the older subgroup. Prior independence rates did not differ between the subgroups. Prevalence of atrial fibrillation and cardioembolic stroke was higher in the older subgroup (P = .004 and .026). Only 3% of the elderly with atrial fibrillation were taking oral anticoagulants. Symptoms-to-needle time was lower in the older subgroup (P = .048). Stroke severity was higher in patients over 80 years (P = .026). There was significant improvement in the National Institutes of Health Stroke Scale score 7 days after intravenous thrombolysis (P < .001) in both subgroups. The proportion of patients with 3 months' favorable outcome and independence, hemorrhagic transformation, and mortality rates were similar in both subgroups. CONCLUSIONS: Elderly patients' benefits and outcomes from intravenous thrombolysis treatment were identical to the younger subgroup without excess hemorrhagic transformation or mortality. These results favor the use of intravenous thrombolysis in patients over 80 years.

A Rare Case of Mediterranean Spotted Fever and Encephalitis.

Mediterranean spotted fever is a tick-borne zoonotic disease caused by Rickettsia conorii. It is transmitted by the dog tick Rhipicephalus sanguineus. It usually presents as a benign self-limited disease characterized by a skin rash, high fever, and, sometimes, a characteristic ulcer at the tick bite site called tache noir. The course of this disease is usually benign, although severe manifestations have been previously described, mainly in adults. Neurological manifestations are very unusual. We present a case of Mediterranean spotted fever with encephalitis to highlight the importance of clinical suspicion, mainly in endemic areas, the potential severity of this disease, and the need of early initiation of therapy in order to prevent severe complications.

Intravenous immunoglobulin therapy in vasculitic ulcers: a case of polyarteritis nodosa.

INTRODUCTION: Polyarteritis nodosa (PAN) is a systemic necrotizing medium-size-vessel vasculitis with variable clinical manifestations. Diagnosis is confirmed by histology or angiography. The mainstay of treatment is corticosteroids alone or combined with cyclophosphamide (CYF). CASE REPORT: Seventy-one-year-old female, follow-up started in 1997 at the age of 56 for suspected relapsing febrile viral exanthema. Skin biopsy was performed and the diagnosis of lymphomatoid papulosis was made, with complete response to treatment with dapsone. In 2005, she presented with arthralgia, lower limb (LL) edema, livedo reticularis and elevated erythrocyte sedimentation rate (ESR). PAN was confirmed on histology and visceral angiography; antineutrophil cytoplasmic antibodies (ANCA) were negative. She responded to prednisolone but relapsed in 2006. Twelve cycles of CYF were administered, with clinical, angiographic and analytical improvement. In 2008, a new relapse occured with LL neuropathic pain and ESR elevation. Electromyogram (EMG) confirmed axonal sensory polyneuropathy (PNP). Azathioprine was started with a poor response. A second EMG, 12 months later in 2009 still evidenced PNP, and nerve biopsy confirmed vasculitic neuropathy. In 2010, she had ulcers in LL and iron-deficient anemia. She started intravenous immunoglobulin (IVIG) for six cycles, achieving ulcer healing, absence of pain, no anemia and ESR normalization. DISCUSSION: IVIG therapy has proven benefit in Kawasaki disease, also showing efficacy in refractory ANCA-associated vasculitis. In PAN, only very few case reports show benefit. In this case, IVIG therapy induced total remission of LL ulcers and PNP, suggesting that it may be useful in selected cases of refractory PAN.