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1.
Rev. med. Chile ; 150(6): 832-835, jun. 2022. ilus
Article in Spanish | LILACS | ID: biblio-1424127

ABSTRACT

Multiple Myeloma (MM) is characterized by a clonal expansion of plasma cells in the bone marrow. These cells typically produce a monoclonal immunoglobulin, and its symptoms arise either from plasma cell infiltration in several organs, or secondary to the presence of a monoclonal protein peak. Symptoms can be summarized by the acronym CRAB (hypercalcemia, renal failure, anemia and bone lesions). Sometimes, in the setting of a protein secreting monoclonal gammopathy, formation of cryoglobulins develops. Cryoglobulins are plasma proteins that precipitate at low temperatures, forming a cold - induced precipitate at small vessels, causing a wide range of clinical manifestations. We report a female consulting for ulcers lasting 2 months in the left foot associated with purpuric lesions in both lower limbs. Protein electrophoresis showed a monoclonal peak in the gamma region. Bone marrow aspirate showed 27% of plasma cells with kappa chain restriction by cytometry. The presence of cryoglobulins was confirmed. The patient was treated with dexamethasone and bortezomib, with a progressive healing of lower limb lesions and disappearance of cryoglobulins. She was discharged in good conditions.


Subject(s)
Humans , Female , Vasculitis/complications , Cryoglobulinemia/complications , Cryoglobulinemia/diagnosis , Multiple Myeloma/complications , Multiple Myeloma/diagnosis , Cryogels , Bortezomib/therapeutic use
3.
Rev. chil. reumatol ; 36(3): 101-108, 2020. tab, ilus
Article in Spanish | LILACS | ID: biblio-1282548

ABSTRACT

La paquimeningitis hipertrófica (PH), es una manifestación poco frecuente de la vasculitis asociada a anticuerpos anti-citoplasma de neutrófilo (ANCA). La literatura describe compromiso de sistema nervioso central (SNC) en 2-8% de los casos en pacientes con vasculitis pauciinmune. Se presenta el caso de un paciente con antecedente de vasculitis anti-mieloperoxidasa (MPO) con un mes de evolución de cefalea hemicraneana izquierda. La resonancia magnética cerebral contrastada evidencia marcado engrosamiento y realce meníngeo dural en el hemicráneo izquierdo, predominante en el tentorio y la fosa posterior. Se descartaron causas infecciosas por lo que se llegó a la conclusión de compromiso meníngeo asociado a vasculitis. Se inició manejo inmunosupresor con mejoría del cuadro clínico. La rápida identificación y manejo de esta entidad puede cambiar su pronóstico sombrío. Se realizó una revisión de la literatura para brindar una herramienta para la toma de decisiones para los médicos que se enfrentan a esta entidad.


Hypertrophic pachymeningitis (PH) is a rare manifestation of vasculitis associated with anti-neutrophil cytoplasm antibodies (ANCA). The literature describes central nervous system (CNS) involvement in 2-8% of cases in patients with pauciimmune vasculitis. We present the case of a patient with a history of anti-Myeloperoxidase (MPO) vasculitis with a 1-month history of left-sided headache. Contrast brain magnetic resonance was performed with evidence of marked thickening and dural meningeal enhancement in the left hemicranium, predominantly in the region of the tentorium and posterior fossa. Infectious causes were ruled out and the meningeal compromise associated with vasculitis was concluded. Immunosuppressive management was started with improvement of the clinical picture. Rapid identification and management of this entity can change its bleak outlook. A systematic review of the literature was carried out in order to provide a decision-making tool for physicians facing this entity.


Subject(s)
Humans , Female , Middle Aged , Vasculitis/immunology , Antibodies, Antineutrophil Cytoplasmic/immunology , Meningitis/etiology , Vasculitis/complications , Magnetic Resonance Spectroscopy , Antibodies, Antineutrophil Cytoplasmic/drug effects , Immunosuppressive Agents/therapeutic use , Meningitis/diagnostic imaging
4.
Rev. chil. radiol ; 24(3): 94-104, jul. 2018. ilus
Article in Spanish | LILACS | ID: biblio-978162

ABSTRACT

La hemorragia subaracnoidea (HSA) no traumática es un subtipo de ictus hemorrágico que representa aproximadamente el 5% de todos los accidentes vasculares encefálicos (AVE). El 85% de los casos de HSA espontánea (no traumática) son secundarios a un aneurisma intracraneano roto, el 10% a hemorragia perimesencefálica no aneurismática y el otro 5% a otras causas. Entre estas se incluyen malformaciones arterio-venosas, fístulas durales, vasculits, trombosis de vena cortical, síndrome de vasoconstricción reversible, angiopatía amiloidea y síndrome de encefalopatía posterior reversible. La aproximación inicial a una HSA no traumática requiere un estudio angiográfico no invasivo con tomografía computada para la toma de decisiones terapéuticas. Si no se detecta un aneurisma sacular intradural que explique el sangrado, las conductas a seguir dependerán del patrón de distribución de la sangre. En esta revisión sugerimos una aproximación basada en 1) revisar el estudio inicial tomando en cuenta los puntos ciegos para la detección de aneurismas, 2) analizar el patrón de distribución de la sangre y 3) analizar los hallazgos en imágenes de acuerdo a las posibles causas según patrón.


Non-traumatic subarachnoid hemorrhage represents approximately 5% of strokes. From these, 85% of nontraumatic subarachnoid hemorrhage are secondary to a ruptured aneurysm, 10% to nonaneurysmal perimesencephalic hemorrhage and the other 5% to other causes. These include but are not limited to arteriovenous malformations, dural fistulae, vasculitis, cortical vein thrombosis, reversible cerebral vasoconstriction syndrome, amyloid angiopathy and posterior reversible encephalopathy syndrome. Initial workup of nontraumatic subarachnoid hemorrhage requires a non-enhanced CT and CT angiography for decision making and management. If there is no aneurysm as a source of hemorrhage, subsequent imaging studies will depend on blood distribution pattern. In this review we suggest an approach: 1) review blind spots for aneurysm detection in the initial CT angiography, 2) analyze blood distribution pattern and 3) evaluate imaging findings and possible causes according to each pattern.


Subject(s)
Humans , Subarachnoid Hemorrhage/etiology , Subarachnoid Hemorrhage/diagnostic imaging , Vasculitis/complications , Intracranial Aneurysm/complications , Cerebral Amyloid Angiopathy/complications , Venous Thrombosis/complications , Computed Tomography Angiography
5.
Acta pediátr. hondu ; 8(2): 819-828, oct. 2017-mar. 2018. ilus
Article in Spanish | LILACS | ID: biblio-1015029

ABSTRACT

La enfermedad de Kawasaki (EK); vasculitis aguda de etiología desconocida, ocurre predominantemente durante la infancia. Las manifestaciones iniciales son fiebre alta, inflamación mucocutánea, linfadenopatía cervical, puede producir aneurismas en las arterias coronarias, depresión de la contracti-lidad miocárdica e insuficiencia cardíaca, infarto de miocardio, arritmias y una morbi-mortalidad significativa, su diagnóstico es clínico. La EK clásica se diagnostica con fiebre mayor de 5 días y al menos 4 de las siguientes características clínicas: inyección conjuntival bilateral, cambios en los labios y cavidad oral, adenopatía cervical, cambios en las extremidades y exantema polimorfo. Si se presentan pocos hallazgos clínicos, pero se encuentran anormalidades en las arterias coronarias en el ecocardiograma, se puede establecer el diagnóstico. La EK atípi-ca se sospecha cuando hay fiebre, al menos 5 días con dos o tres de los síntomas princi-pales, en algunas ocasiones puede presen-tarse como abdomen agudo, meningitis aséptica, pleuritis. La meta del tratamiento es evitar la inflamación sistémica, además prevenir trombosis en los aneurismas desa-rrollados. La inmunoglobulina (IG) es la piedra angular en el tratamiento, se inicia en los primeros 10 días de inicio de la fiebre (2 gr/kg dosis única), la aspirina (80-100 mg/kg por día VO) administrada en combinación con IG como tratamiento inicial durante 4 a 6 semanas. Es importante conocer los criterios de diagnóstico clínico para su detección yKawasaki's DiseaseEnfermedad de Kawasakiasí poder evitar las complicaciones vascula-res que representan una amenaza para la vida del paciente. En la presente revisión se describen su epidemiología, fisiopatología, manifestaciones clínicas, tratamiento y com-plicaciones...(AU)


Subject(s)
Humans , Child , Vasculitis/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Lymphadenopathy , Heart Failure
6.
Journal of Korean Medical Science ; : 542-546, 2016.
Article in English | WPRIM | ID: wpr-58429

ABSTRACT

Few studies have reported on the long-term prognosis of anti-neutrophil cytoplasmic antibody (ANCA)-negative renal vasculitis. Between April 2003 and December 2013, 48 patients were diagnosed with renal vasculitis. Their ANCA status was tested using indirect immunofluorescence and enzyme-linked immunosorbent assays. During a median (interquartile range) follow-up duration of 933.5 (257.5-2,079.0) days, 41.7% of patients progressed to end stage renal disease (ESRD) and 43.8% died from any cause. Of 48 patients, 6 and 42 were ANCA-negative and positive, respectively. The rate of ESRD within 3 months was higher in ANCA-negative patients than in ANCA-positive patients (P = 0.038). In Kaplan-Meier survival analysis, ANCA-negative patients showed shorter renal survival than did ANCA-positive patients (log-rank P = 0.033). In univariate Cox-proportional hazard regression analysis, ANCA-negative patients showed increased risk of ESRD, with a hazard ratio 3.190 (95% confidence interval, 1.028-9.895, P = 0.045). However, the effect of ANCA status on renal survival was not statistically significant in multivariate analysis. Finally, ANCA status did not significantly affect patient survival. In conclusion, long-term patient and renal survival of ANCA-negative renal vasculitis patients did not differ from those of ANCA-positive renal vasculitis patients. Therefore, different treatment strategy depending on ANCA status might be unnecessary.


Subject(s)
Aged , Female , Humans , Male , Middle Aged , Age Factors , Antibodies, Antineutrophil Cytoplasmic/analysis , Cohort Studies , Enzyme-Linked Immunosorbent Assay , Follow-Up Studies , Kaplan-Meier Estimate , Kidney Diseases/diagnosis , Kidney Failure, Chronic/etiology , Microscopy, Fluorescence , Prognosis , Proportional Hazards Models , Republic of Korea , Retrospective Studies , Risk Factors , Severity of Illness Index , Sex Factors , Vasculitis/complications
7.
Einstein (Säo Paulo) ; 13(4): 627-635, Oct.-Dec. 2015. tab
Article in Portuguese | LILACS | ID: lil-770504

ABSTRACT

Vasculitides comprise a heterogeneous group of autoimmune disorders, occurring as primary or secondary to a broad variety of systemic infectious, malignant or connective tissue diseases. The latter occur more often but their pathogenic mechanisms have not been fully established. Frequent and varied central and peripheral nervous system complications occur in vasculitides and connective tissue diseases. In many cases, the neurological disorders have an atypical clinical course or even an early onset, and the healthcare professionals should be aware of them. The purpose of this brief review was to give an update of the main neurological disorders of common vasculitis and connective tissue diseases, aiming at accurate diagnosis and management, with an emphasis on pathophysiologic mechanisms.


As vasculites são um grupo heterogêneo de doenças autoimunes primárias ou secundárias a uma grande variedade de doenças infecciosas sistêmicas, malignas ou do tecido conjuntivo. Estas últimas são as que ocorrem com mais frequência, porém os mecanismos patogênicos ainda não foram plenamente determinados. Diversas e frequentes complicações do sistema nervoso central e periférico ocorrem nas vasculites e doenças do tecido conjuntivo. Em muitos casos, os distúrbios neurológicos têm evolução clínica atípica ou mesmo início precoce, ao que todos os profissionais de saúde devem estar cientes. O objetivo desta breve revisão foi atualizar os principais distúrbios neurológicos da vasculite comum e das doenças do tecido conjuntivo, visando ao diagnóstico e ao tratamento corretos, com ênfase nos mecanismos fisiopatológicos.


Subject(s)
Humans , Autoimmune Diseases/complications , Connective Tissue Diseases/complications , Vasculitis/complications , Arthritis, Rheumatoid/complications , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/physiopathology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/physiopathology , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/physiopathology , Churg-Strauss Syndrome/complications , Churg-Strauss Syndrome/diagnosis , Churg-Strauss Syndrome/physiopathology , Connective Tissue Diseases/diagnosis , Connective Tissue Diseases/physiopathology , Giant Cell Arteritis/complications , Giant Cell Arteritis/diagnosis , Giant Cell Arteritis/physiopathology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/physiopathology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/physiopathology , Polyarteritis Nodosa/complications , Polyarteritis Nodosa/diagnosis , Polyarteritis Nodosa/physiopathology , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Scleroderma, Systemic/physiopathology , Sjogren's Syndrome/complications , Sjogren's Syndrome/diagnosis , Sjogren's Syndrome/physiopathology , Takayasu Arteritis/complications , Takayasu Arteritis/diagnosis , Takayasu Arteritis/physiopathology , Vasculitis, Central Nervous System/complications , Vasculitis, Central Nervous System/diagnosis , Vasculitis, Central Nervous System/physiopathology , Vasculitis/diagnosis , Vasculitis/physiopathology
8.
Rev. chil. infectol ; 32(1): 73-79, feb. 2015. ilus, tab
Article in Spanish | LILACS | ID: lil-742541

ABSTRACT

Cryptococcal infections are classically associated to HIV/AIDS patients without therapy, but its presence among other immunosuppressed patients is less recognized. We report 3 lethal cases in non HIV-patients. Two of them presented with meningitis associated to renal transplant or corticosteroid use and, the third, with a necrotic skin infection in the context of progressive liver cirrhosis. In the former two patients, meningeal infection was suspected late, and in the latter, the diagnosis was established postmortem. Cryptococcal infections in non-HIV immunosupressed patients can affect different sites, are suspected late and have a high case-fatality ratio.


La mayoría de los casos de infecciones criptocócicas se presenta en pacientes con infección por VIH/SIDA con inmunosupresión avanzada. En otro tipo de pacientes inmunosuprimidos es menos conocida, pero ha ido aumentando en frecuencia. Presentamos tres casos de infecciones criptocócicas en pacientes inmunosuprimidos no infectados por VIH, que se manifestaron como meningitis en un receptor de trasplante renal y en un paciente con terapia corticosteroidal y una infección cutánea progresiva necrótica con diseminación secundaria en un paciente con cirrosis hepática avanzada. En todos los casos, la infección fue identificada tardíamente. La infección en el paciente con cirrosis se estableció postmortem. Las infecciones criptocócicas en pacientes sin infección por VIH pueden ocurrir en diferentes sitios, se sospechan tardíamente y tienen alta letalidad.


Subject(s)
Humans , Male , Female , Middle Aged , Aged , Cryptococcosis/immunology , Cryptococcus neoformans/isolation & purification , Immunocompromised Host , Meningitis, Cryptococcal/immunology , Chile , Dermatomycoses/pathology , Fatal Outcome , Kidney Transplantation/adverse effects , Liver Cirrhosis/complications , Renal Insufficiency/complications , Vasculitis/complications
9.
Biomédica (Bogotá) ; 33(4): 503-512, Dec. 2013. ilus, tab
Article in Spanish | LILACS | ID: lil-700468

ABSTRACT

Introducción. Aproximadamente el 50 % de los casos de urticaria crónica no mejoran adecuadamente con las dosis convencionales de antihistamínicos, por lo cual se han planteado múltiples opciones terapéuticas, entre las cuales el omalizumab es una herramienta novedosa que ahora cuenta con evidencia de alta calidad que soporta su uso en los casos difíciles, que mejora rápidamente el índice sintomático y el uso de medicamentos, y cuenta con un buen perfil de seguridad. Objetivo. Presentar tres casos de mujeres adultas con urticaria crónica espontánea de más de ocho años de evolución, que no mejoraron con el tratamiento con altas dosis de antihistamínicos, asociados a antileucotrienos e inmunomoduladores y en quienes se combinaban varios mecanismos fisiopatológicos: urticaria crónica espontánea con componente de autoinmunidad, componente de presión y urticaria vasculítica. Materiales y métodos. Se reportan los casos con sus respectivas evaluaciones clínicas y de laboratorio, los medicamentos usados y la respuesta después del inicio de omalizumab y se hace una revisión de la literatura científica sobre uso de este medicamento en la urticaria crónica. Resultados. En los tres casos presentados se obtuvo una mejoría completa de los síntomas tras el inicio del omalizumab. Conclusión. El omalizumab es una opción terapéutica exitosa en casos de urticaria crónica de difícil control con vasculitis asociada, cuando se han agotado las opciones propuestas por las guías internacionales.


Introduction: Approximately 50% of chronic urticaria cases do not respond adequately to conventional doses of antihistamines, so a number of other therapeutic options have been suggested. Among these, omalizumab is an innovative tool, which now has high-quality evidence that supports its use in difficult cases, rapidly improving the symptom index and the use of medications with a good safety profile. Objective: To report three cases of adult women with spontaneous chronic urticaria with an evolution of more than eight years, which did not improve with high doses of antihistamines and leukotriene receptor blockers, associated with immunomodulatory therapy in which several etiologic mechanisms were combined: chronic spontaneous urticaria with autoimmune and pressure components, and vasculitis. Materials and methods: We report the cases with their clinical and laboratory evaluations, used medication, the response after the start of omalizumab and we performed a review of the literature on the use of this drug in chronic urticaria. Results: In all the presented cases, we obtained complete improvement of symptoms after starting omalizumab. Conclusion: Omalizumab is a successful treatment option in cases of difficult to control chronic urticaria with associated vasculitis in which the options proposed by international guidelines have been exhausted.


Subject(s)
Adult , Female , Humans , Middle Aged , Anti-Allergic Agents/therapeutic use , Antibodies, Anti-Idiotypic/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , Urticaria/complications , Urticaria/drug therapy , Vasculitis/complications , Chronic Disease
10.
Rev. chil. enferm. respir ; 29(1): 39-42, mar. 2013. ilus
Article in Spanish | LILACS | ID: lil-678048

ABSTRACT

El diagnóstico diferencial de nódulos pulmonares cavitados constituye un gran desafo en el cual, la clínica, la imagenología y los exámenes de laboratorio permiten una orientación etiológica en cutro grandes grupos: infecciosa, neoplásica, reumatológica y miscelánea. Presentamos el caso de una paciente gran fumadora y portadora de una diabetes mellitus descompensada, con nódulos pulmonares cavitados, en la cual el contexto clínico obligaba a plantear ciertas etiologías como las infecciosas y neoplásicas, pero cuya biopsia fue compatible con Granulomatosis con Poliangeítis (ex Granulomatosis de Wegener), sin una clínica ni exámenes de laboratorio concordantes con dicho hallazgo.


The differential diagnosis of cavitary pulmonary nodules is a great challenge, where the clinical context in addition to the image studies and laboratory tests are part of the key tools to guide the diagnostic process through 4 major etiological groups: infectious, neoplastic, rheumatologic and miscellaneous. We describe a case of a heavy smoker and complicated diabetic patient with cavitary pulmonary nodules, with a clinical context that induces certain etiologies such as infectious and neoplastic, but whose biopsy was compatible with Granulomatosis with polyangiitis (ex Wegener 's granulomatosis), without a clinical exam nor laboratory tests suggesting this finding.


Subject(s)
Humans , Female , Middle Aged , Lung Diseases/etiology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/diagnosis , Vasculitis/complications , Vasculitis/diagnosis , Diagnosis, Differential
11.
Neumol. pediátr ; 8(1): 10-16, 2013. ilus, tab
Article in Spanish | LILACS | ID: lil-701684

ABSTRACT

Rheumatic diseases are a heterogeneous group of autoimmune basis and mainly joint and skin manifestations. Respiratory manifestations of these are less common in children than in adults, can affect all respiratory structures, however the most common are the pleural space with pleuritis and effusion and interstitial lung disease. Alterations are usually subclinical beginning, which are evident to the tomography of chest or lung function tests as spirometry or diffusion test for carbon monoxide. Some cases presents with severe respiratory impairment as respiratory bleeding or pneumonia. The comprehensive evaluation included clinical history, imaging studies and pulmonary function is key in the evaluation and management of these patients.


Las enfermedades reumáticas son un grupo heterogéneo de patologías de base autoinmune y manifestaciones principalmente articulares y cutáneas. Las expresiones respiratorias de estas en niños son menos frecuentes que en los adultos, pueden afectar todas las estructuras respiratorias, sin embargo las más frecuentes son el espacio pleural con pleuritis y derrame y la afectación intersticial pulmonar. Las alteraciones suelen ser de comienzo subclínico, que se hacen evidentes a la exploración con tomografía de tórax o en pruebas de función respiratoria como espirometría o prueba de difusión de monóxido de carbono. Algunos casos cursan con afectación respiratoria severa, como hemorragia respiratoria o neumonías sobre agregadas. La evaluación integral con historia clínica, estudios de imágenes y de función respiratoria son la clave en la evaluación y manejo de estos pacientes.


Subject(s)
Humans , Child , Respiratory Tract Diseases/etiology , Rheumatic Diseases/complications , Antirheumatic Agents/adverse effects , Arthritis, Juvenile/complications , Dermatomyositis/complications , Respiratory Tract Diseases/chemically induced , Respiratory Tract Diseases/immunology , Lupus Erythematosus, Systemic/complications , Sjogren's Syndrome/complications , Vasculitis/complications
12.
J. vasc. bras ; 10(4): 319-324, dez. 2011. ilus
Article in Portuguese | LILACS | ID: lil-610955

ABSTRACT

A isquemia aguda de membros pode se manifestar, embora de forma incomum, como consequência à vasculite associada ao vírus da imunodeficiência humana (HIV). O presente caso descreve a evolução de uma paciente soropositiva para o HIV, que apresentou quadro de isquemia distal bilateral, com diminuição da temperatura de terço distal das pernas e pés, dor intensa, cianose fixa de pododátilos e ausência de pulsos distais. Submetida ao tratamento com terapia trombolítica, apresentou sinais de lesões decorrentes da isquemia e lesão tecidual de reperfusão com perda tecidual em regiões distais dos dedos, porém com melhora dos sinais e sintomas dos membros inferiores. Trata-se de um caso raro na literatura em função da associação da vasculite com o HIV e do acometimento dos vasos distais nos membros inferiores. Entretanto, o conhecimento desta associação é de extrema importância devido à repercussão na vida dos pacientes acometidos.


The acute limb ischemia may manifest itself, albeit unusual, as a consequence of vasculitis associated with human immunodeficiency virus (HIV). This case report described a patient seropositive for HIV who developed bilateral distal ischemia with temperature decrease of distal legs and feet, severe pain, cyanosis of fixed toes, and absence of distal pulses. She underwent treatment with thrombolytic therapy, showed signs of injury resulting from ischemia and reperfusion tissue injury with tissue loss in the distal regions of the fingers, but with improvement of the signs and symptoms of lower limbs. It is a rare case in literature due to the association of vasculitis with HIV and to the torment of distal vases of the lower limbs. Despite of that, the knowledge of the pathology is extremely important because of the repercussion in the patients' lives.


Subject(s)
Humans , HIV , Ischemia/blood , Ischemia/therapy , Thrombolytic Therapy/nursing , Vasculitis/complications , Lower Extremity
14.
Bol. Hosp. Viña del Mar ; 67(1/2): 30-35, mar. 2011.
Article in Spanish | LILACS | ID: lil-658314

ABSTRACT

El propiltiouracilo (PTU) es un fármaco de uso frecuente en el tratamiento del hipotiroidismo. La vasculitis ANCA positivo secundaria a su uso es una complicación poco frecuente, pero varios casos han sido reportados en la literatura. Se describe un caso de una paciente de 16 años, usuaria de PTU hace 5 años por enfermedad de Graves, la cual desarrolla una vasculitis ANCA (+) con compromiso cutáneo, cerebral y poliarticular, que constituye una presentación severa poco habitual de esta patología. Tras la suspensión del PTU más tratamiento esteroidal la paciente presentó remisión de la vasculitis.


Propylthiouracil (PTU) is a frecuent treatment for hyperthyroidism. The secondary ANCA positive vasculitis to its use, is a uncommon complication, but several cases have been described in the literature. A case of a 16 years old patient with Graves'disease using PTU for over 5 years is described. The patient develop a ANCA (+) vasculitis with cutaneous, cerebral and polyarticular compromise, that it is a severe unusual presentation of this pathology. With the suspension of PTU plus esteroids treatment, clinical improvement of the patient was observed.


Subject(s)
Humans , Adolescent , Female , Hypothyroidism , Vasculitis/complications , Vasculitis/diagnosis , Chile
15.
Journal of Korean Medical Science ; : 444-446, 2011.
Article in English | WPRIM | ID: wpr-52126

ABSTRACT

Endovascular repair of inflammatory aortic aneurysms has been reported as an alternative to open surgical treatment. In selective cases, adjunctive bypass surgery may be required to provide an adequate landing zone. We report a case of endovascular repair of an inflammatory aortic aneurysm in a patient with Behcet's disease using a carotid-carotid bypass graft to provide an adequate landing zone. A 45-yr-old man with a voice change was referred to our hospital with the diagnosis of saccular aneurysm of the distal aortic arch resulting from vasculitis. Computed tomography showed a thoracic aortic aneurysm with thrombosis. Right to left carotid-carotid bypass grafting was performed. After 8 days, the patient underwent an endovascular stent graft placement distal to the origin of the innominate artery. The patient was discharged with medication and without postoperative complications after 5 days. Hybrid endovascular treatment may be suitable a complementary modality for repairing inflammatory aortic aneurysms.


Subject(s)
Humans , Male , Middle Aged , Aortic Aneurysm, Thoracic/complications , Behcet Syndrome/complications , Blood Vessel Prosthesis Implantation/methods , Carotid Arteries/physiopathology , Coronary Artery Bypass , Endovascular Procedures , Stents , Thrombosis/complications , Tomography Scanners, X-Ray Computed , Vasculitis/complications
16.
Rev. méd. hondur ; 78(3): 132-135, jul.-sept. 2010. ilus
Article in Spanish | LILACS | ID: lil-644921

ABSTRACT

Introducción. Las micosis intramedulares son infrecuentes. Asimismo, la manifestación neurológica de la histoplasmosis es una entidad poco común y más infrecuente aún es la manifestación de la histoplasmosis a través de un granuloma intramedular. Caso Clínico. Se presenta el caso de una niña con síndrome medular a quien se realizó resección quirúrgica de una lesión patológicamente compatible con histoplasmosis, quien desarrolló hidrocefalia, lesiones isquémicas en fosa posterior y falleció a pesar del l tratamiento establecido. Conclusión. Aunque las micosis de médula espinal son infrecuentes, es conveniente considerar a la hora de realizar los diagnósticos diferenciales de lesiones granulomatosas en esta localización anatómica...


Subject(s)
Humans , Female , Child , Glaucoma/diagnosis , Histoplasmosis/diagnosis , Central Nervous System Fungal Infections/complications , Spinal Cord/abnormalities , Vasculitis/complications
17.
Article in Spanish | LILACS | ID: lil-652009

ABSTRACT

Se presenta el caso de una niña de 11 meses de edad con cuadro febril asociado a deposiciones diarreicas, exantema, hiperemia conjuntival, lengua en fresa, queilitis, adenopatía cervical, edema de manos y pies, derrame pericárdico y alteración de los exámenes paraclínicos. Se diagnosticó enfermedad de Kawasaki en fase aguda; se trató con inmunoglobulina humana y aspirina, con buena evolución.


Subject(s)
Infant , Mucocutaneous Lymph Node Syndrome , Vasculitis/complications
18.
Arq. bras. cardiol ; 94(2): 273-279, fev. 2010. ilus, tab
Article in Portuguese | LILACS | ID: lil-544892

ABSTRACT

A obesidade vem se tornando uma epidemia global. Cerca de 1,1 bilhões de adultos e 10 por cento das crianças do mundo são atualmente considerados portadores de sobrepeso ou obesos. Classicamente associada a fatores de risco para doença cardiovascular, como diabete melito e hipertensão arterial sistêmica, a obesidade vem sendo cada vez mais encarada como fator de risco independente para doença arterial coronariana (DAC). A aterosclerose coronariana compreende uma série de respostas inflamatórias em nível celular e molecular, cujas reações se encontram mais exacerbadas em pacientes obesos. Antes considerado mero depósito de gordura, o tecido adiposo é visto hoje em dia como órgão endócrino e parácrino ativo, produtor de diversas citocinas inflamatórias, como as adipocinas. Este artigo visa alertar para o grave problema de saúde pública em que a obesidade se tornou nas últimas décadas e correlacionar o processo inflamatório exacerbado nos indivíduos obesos com a maior incidência de DAC nessa população.


Obesity is becoming a global epidemic. Around 1.1 billion adults and 10 percent of the world's children are currently overweight or considered obese. Generally associated with risk factors for cardiovascular disease, such as Diabetes Mellitus and systemic arterial high blood pressure, the obesity has been more and more seen as an independent risk factor for Coronary Artery Disease (CAD). Coronary arteriosclerosis comprises a series of inflammatory responses at cellular and molecular level, whose reactions are stronger in obese patients. In the past, the adipose tissue was regarded as a mere fat deposition. Now it is seen from a totally different standpoint, as an active endocrine and paracrine organ that produces several inflammatory cytokines, such as the adipokines. This article aims to raise awareness about obesity as an increasingly significant public health issue over the past decades, as well as to relate the intense inflammatory process in obese individuals with an increased tendency for this group of individuals to develop CAD.


La obesidad se está tornando una epidemia global. Cerca de 1,1 billones de adultos y el 10 por ciento de los niños del mundo están considerados actualmente portadores de sobrepeso u obesos. Clásicamente asociada a factores de riesgo para enfermedad cardiovascular, como diabetes melitus e hipertensión arterial sistémica, la obesidad se está considerando cada vez más factor de riesgo independiente para enfermedad arterial coronaria (EAC). La aterosclerosis coronaria comprende una serie de respuestas inflamatorias a nivel celular y molecular, cuyas reacciones se encuentran más exacerbadas en pacientes obesos. Antes considerado mero depósito de grasa, el tejido adiposo está visto hoy en día como órgano endócrino y parácrino activo, productor de diversas citocinas inflamatorias, como las adipocinas. Este artículo apunta a alertar sobre el grave problema de salud pública en que se convirtió la obesidad en las últimas décadas y correlacionar el proceso inflamatorio exacerbado en los individuos obesos con la mayor incidencia de EAC en esta población.


Subject(s)
Humans , Coronary Artery Disease/etiology , Obesity/complications , Vasculitis/complications , Adiponectin/blood , C-Reactive Protein/physiology , Endothelium, Vascular/physiopathology , Inflammation Mediators/physiology , Leptin/physiology , Obesity/therapy , Risk Factors
19.
An. bras. dermatol ; 84(1): 55-67, jan.-fev. 2009. ilus, tab
Article in Portuguese | LILACS | ID: lil-511465

ABSTRACT

As vasculites são constituídas por um grande grupo de síndromes caracterizadas por inflamação e necrose da parede dos vasos sangüíneos, resultando em estreitamento ou oclusão do lúmen. A distribuição dos vasos envolvidos varia consideravelmente e serve de base para a classificação das síndromes vasculíticas: grandes vasos (arterite de Takayasu, arterite temporal); pequenos e médios vasos (poliarterite nodosa, síndrome de Churg-Strauss, granulomatose de Wegener, vasculites nas doenças reumáticas); e pequenos vasos (vasculite de hipersensibilidade, púrpura de Henoch-Schonlein, poliangeíte microscópica, crioglobulinemia). Este artigo revisa as vasculites dos médios e grandes vasos e opções terapêuticas atuais; além de oferecer orientação sobre abordagem diagnóstica e tratamento dos pacientes com suspeita de vasculite cutânea.


Vasculitis comprises a broad group of syndromes characterized by inflammation and necrosis on thewalls of blood vessels, resulting in narrowing or occlusion of the lumen. The distribution of involved blood vesselvaries considerably and serves as the basis for one classification of vasculitic syndromes: large vessels (Takayasu arteritis, giant cell arteritis); medium and small muscular arteries (polyarteritis nodosa, Churg-Strausssyndrome, Wegener's granulomatosis, vasculitis in rheumatic diseases); and small vessels ("hypersensitivity" vasculitis, Henoch-Schonlein purpura, microscopic polyangiitis, cryoglobulinemia). This article reviews medium and large vessel vasculitis and current treatment options; it also presents a comprehensive approach to diagnosing and treating patients with suspected cutaneous vasculitis.


Subject(s)
Humans , Vasculitis , Blood Vessels/anatomy & histology , Organ Size , Vasculitis/complications , Vasculitis/diagnosis , Vasculitis/etiology
20.
Article in English | IMSEAR | ID: sea-88616

ABSTRACT

A 48 years male presented with acute axonal neuropathy and palpable purpura over bilateral lower limb, RA factor, and cryoglobulins were present in the serum. Nerve biopsy revealed myelinated fibre loss, axonal degeneration and necrotizing vasculitis of epineural vessels.


Subject(s)
Acute Disease , Cryoglobulinemia/complications , Cryoglobulins/immunology , Diagnosis, Differential , Enzyme-Linked Immunosorbent Assay , Humans , Immunologic Factors , Lower Extremity , Male , Middle Aged , Polyneuropathies/etiology , Prednisolone/administration & dosage , Vasculitis/complications
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