Lymphocytic thrombophilic arteritis with lower-limb ulcers

Abstract Lymphocytic thrombophilic arteritis is a recently described entity, histopathologically characterized by lymphocytic vasculitis that affects the arterioles of the dermo-hypodermic junction, associated with deposition of fibrin and a luminal fibrin ring. A 49-year-old female patient presented with achromic maculae and a well-defined ulcer on the medial aspect of the left lower limb. The biopsy showed intense inflammatory infiltrate in the papillary dermis with a predominance of lymphocytes, and medium-caliber vessels surrounded by mononuclear infiltrates in the deep reticular dermis. Masson's trichrome staining showed intense destruction of the muscle layer of the vascular wall and a fibrin ring. Good clinical response was attained with azathioprine. The authors believe that the ulceration might be another clinical presentation or represent an atypical progression of this condition.

Coronary arteritis as a cause of sudden cardiac death in a young girl

A case of probable coronary arteritis in a young girl who died suddenly and unexpectedly is presented. The histologic presentation of the disorder is discussed, especially the differential diagnosis of arteritis of the coronary arteries with an emphasis on tuberculosis (TB). TB myocarditis with or without concomitant lung involvement is rare, and tubercular coronary arteritis without underlying pulmonary Koch's disease is all the rarer. We herein describe a case where the cause of death was ascertained on post-mortem examination.

Arterite sifilítica / Retinal arteritis related to syphilis

Resumo Apresentamos um caso de neurosífilis em um homem jovem, com queixa de baixa acuidade visual (BAV) em olho esquerdo. Cursou com lesões eritemato-descamativas nas palmas das mãos, plantas dos pés e úlceras orais, sem lesões genitais. O exame oftalmológico revelou arterite em arcada nasal superior no olho afetado. Apresentou VDRL (1:4096) e FTA-Abs positivos. O exame do líquor cefalorraquidiano foi negativo. O tratamento foi realizado com ceftriaxona 2g/ dia por 14 dias, associado à prednisona 0,5mg/kg oral 48h após início do antibiótico. Após 15 dias de tratamento, houve melhora da AV, regressão da vasculite e redução da titulação do VDRL para 1:128.

Abstract We present a case of neurosyphilis in a young man with a complaint of low visual acuity in the left eye. He had erythematous-scaly lesions on the palms of the hands, soles of the feet and oral ulcers, without genital lesions. The ophthalmic examination revealed arteritis in the upper nasal arcade in the affected eye. He presented VDRL (1: 4096) and FTA-Abs positive. The cerebrospinal fluid cerebrospinal fluid test was negative. The treatment was performed with ceftriaxone 2g / day for 14 days, associated with prednisone 0.5mg / kg oral 48h after antibiotic onset. After 15 days of treatment, there was improvement of AV, regression of vasculitis and reduction of VDRL titration to 1: 128.

Is macular lymphocytic arteritis limited to the skin? Long-term follow-up of seven patients

Abstract Background: Macular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa. Objective: To describe clinical, histopathological, and laboratory findings of patients with the diagnosis of macular lymphocytic arteritis. Methods: A retrospective search was conducted by reviewing cases followed at the Vasculitis Clinic of the Dermatology Department, School of Medicine, University of São Paulo, between 2005 and 2017. Seven patients were included. Results: All cases were female, aged 9-46 years, and had hyperpigmented macules mainly on the legs. Three patients reported symptoms. Skin biopsies evidencing a predominantly lymphocytic infiltrate affecting arterioles at the dermal subcutaneous junction were found, as well as a typical luminal fibrin ring. None of the patients developed necrotic ulcers, neurological damage, or systemic manifestations. The follow-up ranged from 18 to 151 months, with a mean duration of 79 months. Study limitations: This study is subject to a number of limitations: small sample of patients, besides having a retrospective and uncontrolled study design. Conclusions: To the best of the authors' knowledge, this series presents the longest duration of follow-up reported to date. During this period, none of the patients showed resolution of the lesions despite treatment, nor did any progress to systemic vasculitis. Similarities between clinical and skin biopsy findings support the hypothesis that macular lymphocytic arteritis is a benign, incomplete, and less aggressive form of cutaneous polyarteritis nodosa.

Increase in serum and salivary neutrophil gelatinase-associated lipocalin levels with increased periodontal inflammation

Abstract Objective: This study aimed to determine serum and salivary levels of neutrophil gelatinase-associated lipocalin (NGAL) and evaluate NGAL correlation with key anti-interleukin 10 (IL-10) and pro-inflammatory (IL-1β) cytokines in different severities of periodontal diseases. We also calculated the systemic inflammation using the periodontal inflamed surface area (PISA) to evaluate its correlation with NGAL in the study groups. Methodology: Eighty systemically healthy and non-smoking individuals were separated into four groups of 20: clinically healthy (Group 1), gingivitis (Group 2), stage I generalized periodontitis (Group 3, Grade A), and stage III generalized periodontitis (Group 4, Grade A). Sociodemographic characteristics and periodontal parameters were recorded, and PISA was calculated. The serum and salivary levels of interleukin (IL)-1β, IL-10, and NGAL were determined using the enzyme-linked immunosorbent assay (ELISA). Results: We observed a significant increase in serum and salivary NGAL levels from healthy to periodontitis groups (p=0.000). Group 2 presented significantly higher serum and salivary IL-10 levels and salivary IL-1β levels than Group 3 (p=0.000). Serum and salivary parameters (IL-1β, IL-10, and NGAL levels) were strongly positively correlated to periodontal parameters and PISA values (p=0.000). Groups 2 and 3 showed overlapping PISA values. Conclusion: The overlapping PISA values found in Groups 2 and 3 suggest that gingivitis might progress to a systemic inflammatory burden somewhat comparable to stage I periodontitis. This finding is supported by the higher serum and salivary cytokines/mediators levels in the gingivitis group than in stage I periodontitis group. Serum and salivary NGAL levels increased proportionally to disease severity and PISA. NGAL seems to play a role in the pathogenesis of periodontal disease, within the limitation of our study.

Arteritic Anterior Ischemic Optic Neuropathy Associated with Giant-Cell Arteritis in Korean Patients: A Retrospective Single-Center Analysis and Review of the Literature

BACKGROUND AND PURPOSE: The aim of this study is to report the relative incidence of arteritic anterior ischemic optic neuropathy (AAION) associated with giant-cell arteritis (GCA) in a single-center and evaluate the clinical features of AAION in Korean patients. METHODS: The medical records of patients with presumed AION who visited our hospital from January 2013 to August 2018 were examined retrospectively. The patients were divided into two groups: AAION associated with GCA, and non AION (NAION). We additionally reviewed the literature and identified all cases of AAION in Korean and Caucasian patients. We evaluated the clinical data including the initial and final best-corrected visual acuities, fundus photographs, visual field tests, fluorescein angiography, and contrast-enhanced MRI, and compared the data with those for Caucasian patients in the literature. RESULTS: Of the 142 patients with presumed AION, 3 (2.1%) were diagnosed with AAION and 139 (97.9%) were diagnosed with NAION. Seven Korean patients with AAION associated with GCA were identified in our data and the literature review. We found no difference in any clinical features other than laterality: four of the seven Korean patients had bilateral involvement. Moreover, the optic nerve sheath was enhanced in two of our Korean patients. CONCLUSIONS: AAION associated with GCA is a very rare condition compared to NAION in Korea. However, GCA should be considered in all cases of ischemic optic neuropathy because AAION is associated with poor visual outcome, and sometimes presents bilaterally.

Immunoglobulin G4-Related Aortitis of the Abdominal Aorta / 대한흉부외과학회지

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.

Dépistage de l'artérite des membres inférieurs chez les patients ayant un pied diabétique en attente d'une chirurgie à cotonou

Objectifs : L'étude a été initiée afin de dépister l'artérite des membres inférieurs chez les patients ayant un pied diabétique. Méthodologie : L'étude est transversale, descriptive et analytique. La population d'étude est consti-tuée des diabétiques hospitalisés pour pied diabétique au CNHU-HKM de Cotonou. L'artérite des membres inférieurs est dépistée par l'échodoppler artériel.Résultats : l'artérite des membres inférieurs a été dépistée chez159 patients soit uneprévalence de 80,76%. Les facteurs associés à l'artérite sont la durée de dépistage du diabète d'au moins 10 ans, l'hypertension artérielle, les signes d'ischémie etl'absence du pouls pédieux.Conclusion : l'artérite des membres inférieurs chez les patients ayant un pied diabétique est fré-quente. Le dépistage systématique de l'artérite est fortement recommandé en cas de lésion du pied chez les diabétiques

Bilateral Involvement of Juvenile Temporal Arteritis Associated with Kimura Disease

Juvenile temporal arteritis (JTA) is a localized nodular arteritis confined to the temporal artery without evidence of systemic inflammation, and it occurs mainly in patients younger than 50 years. From the first case report, the pathological features of JTA have been suspected to be the morphological equivalent of Kimura disease (KD), which has been supported further by the concurrent cases of JTA with KD. We present the first case of bilateral JTA accompanying KD, which was confirmed by histological and ultrasound evaluations and supports the hypothesis that JTA is a manifestation of KD. The un-excised JTA lesion was resolved completely after corticosteroid therapy with no recurrence.

Effect of Pioglitazone in Combination with Moderate Dose Statin on Atherosclerotic Inflammation: Randomized Controlled Clinical Trial Using Serial FDG-PET/CT

BACKGROUND AND OBJECTIVES: Non-statin therapy plus lower intensity statin might be an alternative in patients with coronary artery disease (CAD). A recent data suggested an anti-inflammatory therapy can reduce recurrent cardiovascular events and pioglitazone is also an intriguing inflammatory-modulating agent. However, limited data exist on whether pioglitazone on top of statins further attenuates plaque inflammation. METHODS: Statin-naïve patients with stable CAD and carotid plaques of ≥3 mm were randomly prescribed moderate dose atorvastatin (20 mg/day), or moderate dose atorvastatin plus pioglitazone (30 mg/day) for 3 months. The primary endpoint was the change in the arterial inflammation of the carotid artery measured by 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) during 3 months. RESULTS: Of the 41 randomized patients, 33 underwent an evaluation by fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT; 17 atorvastatin plus pioglitazone and 16 atorvastatin patients). The addition of pioglitazone significantly improved the insulin sensitivity and increased the high-density lipoprotein cholesterol after 3 months. Although a reduction in the (FDG) uptake by pioglitazone on top of atorvastatin in carotid arteries with plaque showed marginally statistical significance in the entire patient group (atorvastatin plus pioglitazone; −0.10±0.07 and atorvastatin −0.06±0.04, p=0.058), pioglitazone showed a further reduction of the fluorodeoxyglucose (FDG) uptake among patients who had a baseline FDG uptake above the median (atorvastatin plus pioglitazone; −0.14±0.04 and atorvastatin −0.03±0.03, p < 0.001). CONCLUSIONS: Pioglitazone demonstrated marginally significant anti-inflammatory effects in addition to moderate dose atorvastatin. This may have been due to the lack of power of the study. However, pioglitazone may have an anti-inflammatory effect in those patients with high plaque inflammation (Trial registry at ClinicalTrials.gov, NCT01341730).

"In God We Trust... All Others, Must Show Data". Proverbio Científico en Estados Unidos / In God We Trust... All Others, Must Show Data. Scientific Proverb in the United States

El frecuente reclamo de datos que pretendemos y a veces exigimos los que creemos que hacemos ciencia, nos enfrenta a menudo a realidades numéricas difíciles de entender o justificar. Pero los datos, son datos, y lo único útil es tratar de analizarlos, y nunca enojarse con ellos

Manejo de arteritis de células gigantes en Argentina. Encuesta GESAR Vasculitis / Management of giant cell arteritis in Argentine: Vasculitis Study Group Survey

Introducción: Hasta ahora el diagnóstico de la Arteritis de Células gigantes (ACg) se ha basado fundamentalmente en la clínica y la biopsia de arteria temporal y el tratamiento en corticoides. En los últimos años, han aparecido nuevos métodos que ayudan en el diagnóstico, y recientemente nuevos tratamientos. Objetivos: Describir el manejo actual de ACg en Argentina. Métodos: Una encuesta corta online de 10 preguntas, diseñada por miembros del grupo de Estudio de Vasculitis de la Sociedad Argentina de Reumatología, fue enviada vía mail a los médicos socios de la Sociedad Argentina de Reumatología (SAR). Resultados: Se obtuvieron las respuestas de 188 médicos. Solo un 13,4% de los reumatólogos estima que logra hacerle una biopsia temporal a la mayoría de sus pacientes con sospecha de ACg, mientras que un 45% puede realizarles ecodoppler. Las dosis de corticoides utilizadas y la duración del tratamiento es variable. No es frecuente el uso de otros tratamientos distintos de los corticoides. Conclusión: El uso prolongado de corticoides es el tratamiento más usado para pacientes con ACg en Argentina con escaso uso de otros inmunosupresores. El uso de ecodoppler de arteria temporal parecería estar más fácilmente disponible como herramienta diagnóstica que la biopsia

Concurrence of Diabetic Ketoacidosis and Acute Ischemic Stroke due to Moyamoya Disease / 대한소아신경학회지

Although the etiology of moyamoya disease (MMD) remains unknown, autoimmunity is one of the proposed pathogeneses. Unlike other autoimmune disorders that are associated with cerebral arteritis, concurrence of MMD and diabetes mellitus (DM) is rare. However, we encountered a patient with concurrent diabetic ketoacidosis (DKA) and acute ischemic stroke due to MMD. Our patient was diagnosed with glutamic acid decarboxylase antibody-positive type 2 DM (T2DM) based on laboratory and physical examination findings. Brain magnetic resonance images revealed an acute ischemic stroke in the left cerebral hemisphere and bilateral diffuse stenosis/occlusion in the middle cerebral artery and multiple collaterals. Thus, here, we report a patient with both T2DM and MMD who developed an acute ischemic stroke that was complicated by DKA.

Characterisation of the vascular pathology in Sigmodon hispidus (Rodentia: Cricetidae) following experimental infection with Angiostrongylus costaricensis (Nematoda: Metastrongylidae)

BACKGROUND Angiostrongylus costaricensis is a nematode that causes human abdominal angiostrongyliasis, a disease found mainly in Latin American countries and particularly in Brazil and Costa Rica. Its life cycle involves exploitation of both invertebrate and vertebrate hosts. Its natural reservoir is a vertebrate host, the cotton rat Sigmodon hispidus. The adult worms live in the ileo-colic branches of the upper mesenteric artery of S. hispidus, causing periarteritis. However, there is a lack of data on the development of vasculitis in the course of infection. OBJECTIVE To describe the histopathology of vascular lesions in S. hispidus following infection with A. costaricensis. METHODS Twenty-one S. hispidus were euthanised at 30, 50, 90 and 114 days post-infection (dpi), and guts and mesentery (including the cecal artery) were collected. Tissues were fixed in Carson’s Millonig formalin, histologically processed for paraffin embedding, sectioned with a rotary microtome, and stained with hematoxylin-eosin, resorcin-fuchsin, Perls, Sirius Red (pH = 10.2), Congo Red, and Azan trichrome for brightfield microscopy analysis. FINDINGS At 30 and 50 dpi, live eggs and larvae were present inside the vasa vasorum of the cecal artery, leading to eosinophil infiltrates throughout the vessel adventitia and promoting centripetal vasculitis with disruption of the elastic layers. Disease severity increased at 90 and 114 dpi, when many worms had died and the intensity of the vascular lesions was greatest, with intimal alterations, thrombus formation, iron accumulation, and atherosclerosis. CONCLUSION In addition to abdominal angiostrongyliasis, our data suggest that this model could be very useful for autoimune vasculitis and atherosclerosis studies.

Neurogenic Pain Disorder in the Foot and Ankle: Peripheral Neuropathy / 대한정형외과학회잡지

Most common peripheral neuropathy around foot and ankle is diabetic neuropathy, but there are another cause of peripheral neuropathy, such as rheumatoid arthritis, metabolic disease, genetic disease, toxic material, and so on. The main symptom of peripheral neuropathy is pain. The disturbance of sensory and balancing, weakness of muscle, deformity of foot and neuropathic arthropathy are also the symptoms of the peripheral neuropathy. History taking is most important to identify the cause of peripheral neuropathy. Neurological exam have to include the pin prick test, vibration test, 10 g-monofilamant test and ankle reflex test. Simple radiography is essential to observe the deformities or neuropathic arthropathy at foot and ankle. The presence of peripheral neuropathy, involvement and severity can be identified from nerve conduction study. The study of occlusive arteritis is essential for diabetic neuropathy. The medical treatment of associated disease is important but the pain of peripheral neuropathy should be controlled simultaneously. Medicine include the antidepressants, anticonvulsants, opioids and topical agents. The surgical treatment of peripheral neuropathy include lengthening of Achilles tendon, correction of deformity, the total contact cast and arthrodesis. Surgical decompression of specific nerve might helpful in pain control of peripheral neuropathy.

Vasculitis de miembros superiores e inferiores por arteritis de células gigantes / Vasculitis of upper and lower limbs due to giant cell arteritis

El compromiso extracraneal de la arteritis de células gigantes está dado fundamentalmente por la afectación del cayado aórtico y la aorta torácica. La vasculitis de grandes vasos en miembros superiores e inferiores es una manifestación infrecuente que puede determinar una importante morbimortalidad, siendo difícilmente reconocida si no existe la sospecha. Presentamos el caso de un varón de 63 años que en el contexto de un síndrome constitucional se le realizó una tomografía por emisión de positrones (PET) en la cual se identificaron imágenes sugestivas de arteritis en miembros superiores e inferiores

A Case of Rheumatoid Vasculitis Involving Hepatic Artery in Early Rheumatoid Arthritis

Rheumatoid vasculitis is a rare, but most serious extra-articular complications of long-standing, seropositive rheumatoid arthritis (RA). Vasculitis of hepatic artery is an extremely rare but severe manifestation of rheumatoid vasculitis. A 72-year-old woman who presented with polyarthralgia for 2 months was diagnosed with early RA. Since she had manifestations of livedo reticularis, and liver dysfunction which was atypical for RA patients, a percutaneous needle liver biopsy was performed revealing arteritis of a medium-sized hepatic artery. Extensive investigations did not reveal evidences of other systemic causes such as malignancy or systemic vasculitis. The patient was diagnosed with rheumatoid vasculitis involving hepatic arteries based on Bacon and Scott criteria for rheumatoid vasculitis. With high dose corticosteroid and cyclophosphamide induction and methotrexate and tacrolimus maintenance treatment, she was successfully recovered. Association of rheumatoid vasculitis at very early stages of the disease may represent an early aggressive form of RA.

Linking resistin, inflammation, and cardiometabolic diseases

Adipose tissue secretes a variety of bioactive substances that are associated with chronic inflammation, insulin resistance, and an increased risk of type 2 diabetes mellitus. While resistin was first known as an adipocyte-secreted hormone (adipokine) linked to obesity and insulin resistance in rodents, it is predominantly expressed and secreted by macrophages in humans. Epidemiological and genetic studies indicate that increased resistin levels are associated with the development of insulin resistance, diabetes, and cardiovascular disease. Resistin also appears to mediate the pathogenesis of atherosclerosis by promoting endothelial dysfunction, vascular smooth muscle cell proliferation, arterial inflammation, and the formation of foam cells. Thus, resistin is predictive of atherosclerosis and poor clinical outcomes in patients with cardiovascular disease and heart failure. Furthermore, recent evidence suggests that resistin is associated with atherogenic dyslipidemia and hypertension. The present review will focus on the role of human resistin in the pathogeneses of inflammation and obesity-related diseases.

Estudo da evolução das lesões vasculares presentes na angiostrongilíase abdominal experimental em Sigmodon hispidus (Rodentia: Cricetidae) / Study of the vascular lesions evolution which present in experimentalabdominal angiostrongyliasis in Sigmodon hispidus (Rodentia: Cricetidae)

Introdução: O Angiostrongylus costaricensis é um nematódeo que causa aangiostrongilíase abdominal. O ciclo de vida deste parasita envolve um hospedeiro invertebrado (intermediário) e um hospedeiro vertebrado mamífero (definitivo). Na natureza, um dos principais hospedeiros definitivos deste parasita é o roedor Sigmodon hispidus. O nematódeo instala-se na artéria mesentérica superior do hospedeiro definitivo, causando inicialmente uma periarterite e o sinofílica e posteriormente um trombo. Histopatologicamente, uma tríade de achados fundamentais define esta patologia: massivo infiltrado e o sinofílico; vasculiteeosinofílica e reação granulomatosa. Apesar da importância clínica, pouco se sabe a cerca dessa patologia. Objetivos: Caracterizar as lesões histopatológicas do território vascular e intestinal; acompanhar a dinâmica hematológica periférica e central; avaliar colesterolemia total e a presença de bactérias na lesão de S. hispidusinfectados com A. costaricensis. Material e Métodos: S. hispidus infectados com A. costaricensis foram eutanasiados em 30, 50, 90 e 114 dpi e foram coletados o intestino e mesentério (incluindo a artéria cecal). Os tecidos foram fixados emformalina Millonig de Carson e, histologicamente processados para microscopia de luz ou imunohistoquímica. O sangue também foi coletado através de punção cardíaca ou do plexo braquial e utilizado para realização de hemograma e análise da colesterolemia total e análise microbiológica. A lesão local também foi coletada para análise microbiológica. Resultados e discussão:...

Introduction: Angiostrongylus costaricensis is a roundworm which causes theabdominal angiostrongyliasis. This parasite’s life cycle involves an invertebrate host(intermediary) and a vertebrate host (definitive). In nature, the usual definitive host forthis parasite is the rodent Sigmodon hispidus. Adult worms install in the uppermesenteric artery of the definitive host, causing in the beginning an eosinophilicpolyarteritis and then a thrombus. Histopathologically, a triad of essential findingdefines this pathology as: massive eosinophilic infiltrate; eosinophilic vasculitis andthe granulomatous reaction. Despite the clinical importance, we know just a fewabout this pathology. Objectives: To characterize the histopathological lesions of thevascular and intestinal territories; to follow the peripheral and central hematologicdynamics; to evaluate the total cholesterol and the bacteria presence in the lesion onthe infected S. hispidus by A. costaricensis. Material and Methods: S. hispidusinfected by A. costaricensis were euthanized in 30, 50, 90 and 114 dpi and werecolected the intestine and mesentery (including the cecal artery). Tissues were fixedin Carson’s Millonig formalin and, histopathologically processed to light microscopy orimmunohistochemistry. The blood was also gathered through the cardiac puncture orfrom the brachial plexus and used to the CBC conduction and analysis of totalcholesterol and microbiological analysis. The local lesion was also gathered tomicrobiological analysis. Results and discussion:...