Taxane-induced scleroderma: report of two cases / Esclerodermia inducida por taxanos: informe de dos casos

ABSTRACT Taxanes are a class of chemotherapeutic agents with common associated dermatologic adverse events, such as skin hyperpigmentation, hand-foot skin syndrome, paronychia and onycholysis. Taxane-induced scleroderma is rare. Few cases with skin findings resembling systemic sclerosis, have been reported after the administration of these agents. We report two cases with stage IV breast cancer, aged 66 and 71 years, who developed sclerodermic skin lesions in their extremities after starting treatment with placlitaxel and nabplaclitaxel respectively.

Los taxanos son agentes quimioterapéuticos cuyo uso se asocia a problemas dermatológicos tales como hiperpigmentación, síndrome manos-pies, paroniquia y onicolisis. La esclerodermia inducida por taxanos es rara, con pocos casos informados en la literatura. Informamos los casos de dos pacientes con cáncer de mama en estado IV, de 66 y 71 años, que desarrollaron lesiones esclerodérmicas en las extremidades después de ser tratadas con placlitaxel y nabplaclitaxel, respectivamente.

Evolución del índice de actividad en pacientes con esclerosis sistémica tratados con ciclofosfamida y prednisona / Evolution of the index of activity in patients with systemic sclerosis treated with cyclophosphamide and prednisone

Introducción: La esclerosis sistémica es una enfermedad autoinmune del tejido conectivo donde ocurre inicialmente la vasculopatía y persiste durante toda la enfermedad. El índice de actividad revela un periodo crítico de la enfermedad. Objetivo: Evaluar la evolución clínica del índice de actividad de pacientes con esclerosis sistémica para determinar si el esquema terapéutico aplicado disminuye los síntomas de actividad sistémica. Métodos: Estudio cuasi experimental terapéutico de 31 pacientes atendidos en el Hospital Lucía Íñiguez Landín de Holguín que se dividieron en dos grupos según las etapas clínicas obtenidas del índice de desarrollo integral desde marzo del 2013 hasta marzo del 2016: el grupo A (etapas clínicas I y II) con 16 pacientes y el grupo B (etapas clínicas III y IV) con 15 pacientes. La evolución se evaluó según variables del instrumento al inicio, a los 6 y 12 meses de aplicado el esquema terapéutico. Se utilizó la prueba T o la prueba exacta de Fisher cuando los valores eran igual a 3 o menores. El cálculo de la media, análisis porcentual y la prueba de Wilcoxon se usaron para conocer la relación de variables en el tiempo. Resultados: El esquema terapéutico aplicado, previa validación, mejoró el índice de actividad de los pacientes de ambos grupos A y B (en etapas clínicas tempranas y tardías). Al evaluar el índice de actividad, en esta serie predominó la actividad moderada, tanto a los 6 como a los 12 meses durante el tratamiento médico. En ambos grupos la mejoría del índice de actividad fue significativa, tanto para la actividad moderada como para la intensa, más notable a partir de los 12 meses con p≤0,05 para el grupo A. Hubo baja susceptibilidad para la mejoría de los sistemas gastrointestinal y respiratorio, en el trascurso de la evaluación de este índice. Conclusiones: Se alcanzó mejoría en el índice de actividad de pacientes con esclerosis sistémica, con el esquema terapéutico aplicado, con estabilidad clínica y humoral desde las etapas iniciales de la enfermedad(AU)

Introduction: The systemic sclerosis is an autoimmune disease of the connective tissue where the vasculopathy happens initially and persist during all the disease. The immune component starts since the inflammatory process triggers off but he diminishes until you dwell on the evolutionary course and it is substituted for fibrosis, this ends pathogenic acquires great significance in the process. The index of activity reveals a critical period of the disease. Objective: Evaluating patients' clinical evolution of the index of activity with systemic sclerosis with the applied therapeutics. Methods: The study was quasi-experiences (or secondary prevention). In order to determine if the therapeutic applied scheme decreases symptomatology of its systemic activity. You started in March of the 2013 to March of the 2016, with duration of 24 months. They were 31 patients that split into two groups according to the clinical stages obtained of Comprehensive Development Index. In the group to (clinical stages I and II) 16 patients and in the group B (clinical stages III and IV) 15 patients. The evolution evaluated according to variables of the instrument of evaluation the start, to the six and 12 months itself of once the therapeutic scheme was applied. The T utilized the proof itself, or exact Fisher's proof when moral values were all the same or minor to three, the statistical significance determined in p≥ 0.05 itself. The calculation of the stocking, percentage analysis, and Wilcoxon's proof to know the relation of variables through the time. Results: The therapeutic applied scheme, previous validation, you improved the index of activity of the patients of both groups A and B that is in clinical premature and overdue stages. In the activity moderated for the group A statistical significance for system microvascular (0.023) and respiratory (0.025) to the six months, and to the 12 months' skin (0.023) and microvascular (0.006). For the intense activity significant improvement to the six months for muscleskelettic (0.005) and rheumatoid positive factor (0.008), to the 12 months' significant improvement for muscleskelettic (0.004); and examine of laboratory like erythrocyte sedimentation rate (0.008) circulating immune complexes (0.005), and rheumatoid factor (0.003). For the group B in the moderate activity significant improvement for respiratory system existed (0.014), and cardiovascular (0.020) that kept to the 12 months, added up its digestive system (0.008). Evident level improvement of skin (0.004), circulating immune complexes (0.008) and rheumatoid factor were caught up within the intense activity to the 12 months (0.014). Conclusions: Improvement in the index of activity of patients with systemic sclerosis, with the therapeutic scheme applied, with clinical stability and humoral from initial stages of the disease was caught up with(AU)

Is rituximab effective for systemic sclerosis? A systematic review and meta-analysis

Abstract Background: Systemic sclerosis (SSc) Is a clinically complex and challenging disease, that leads to skin fibrosis. Its most frequent complication is interstitial lung disease (ILD), which leads to a worse prognosis. In this situation, cyclophosphamide is considered the gold standard for its treatment, despite the controversies regarding its efficacy and toxicity. However, studies using rituximab (RTX) have shown that this drug may be a promising therapeutic option. Objectives: This paper objective was to analyze the scientific evidence on the RTX effects on SSc. Methods: A systematic review (SR) was performed including clinical trials (CTs) on the use of RTX in SSc, published up to May 2020. The studies were identified through systematic searches in bibliographic databases using a predefined search strategy. The following databases were used: PUBMED, SCOPUS, SCIELO, LILACS, SCIENCE DIRECT, WEB OF SCIENCE, COCHRANE, WHOLIS, PAHO and EMBASE. Also, a manual search was performed. The methodological quality of the studies was determined using Jadad scale, Risk of Bias Tool (RoB 2.0) and Risk of Bias in Non-Randomized Studies - of Interventions tool (ROBINS-I). A meta-analysis of the randomized CTs was performed, using Review Manager. Results: Ten CTs were included in this SR. Of these, three were randomized and seven were non-randomized. Five showed a statistically significant improvement in forced vital capacity (FVC) at some time during follow-up. Regarding the skin, eight studies showed statistically significant improvements according toa the modified Rodnan skin score. The meta-analysis found positive effects of RTX in SSc, with a statistical significance for lung disease. Conclusion: Rituximab is a promising strategy for the SSc-associated ILD and cutaneous fibrosis treatment. PROSPERO registration number: CRD42019132018.(AU)

Comparação do Escore de Rodnan modificado e durômetro em pacientes com esclerose sistêmica difusa / Comparison of the modified Rodnan skin score and durometer in patients with diffuse systemic sclerosis

esclerose sistêmica é uma enfermidade autoimune, causadora de danos vasculares e fibroses. É dentre as doenças de sistema conjuntivo, bem conhecida, devido à alta mortalidade, principalmente pelas causas pulmonares e cardíacas. Para a avaliação do tecido cutâneo de portadores de esclerose sistêmica é utilizado o Escore de Rodnan Modificado, que consiste no pregueamento da pele, porém esta avaliação pode ser subjetiva, devido às limitações na mensuração do escore, tanto pela variabilidade do profissional, quanto pela percepção na aferição do procedimento. Na busca de uma forma de avaliação embasada em evidências científicas, propôs-se este estudo para subsidiar a aferição objetiva do tecido cutâneo. O objetivo do estudo foi correlacionar os Escores de Rodnan Modificados e as medidas do aparelho durômetro, obtidos por dois pesquisadores distintos, em pacientes com esclerose sistêmica difusa. Trata-se de um estudo analítico e transversal, realizado com pacientes maiores de 16 anos, de ambos os sexos. A amostra desta pesquisa foi constituída por 58 pacientes, com maior predominância entre pacientes com cor da pele branca. Houve correlação discretamente moderada e moderada entre as avaliações das medidas obtidas com o Escore de Rodnan Modificado e também com o aparelho durômetro realizadas pelos pesquisadores 1 e 2. A concordância entre os pesquisadores foi avaliada com Coeficiente de Kappa para Escore de Rodnan Modificado e Coeficiente de Correlação Interclasse para o aparelho durômetro, com melhores resultados para as medidas obtidas com este aparelho. Houve também forte correlação positiva entre o escore de Rodnan modificado, média durômetro total e espessura da derme. Portanto, concluí-se que o aparelho durômetro poderá ser utilizado como método de avaliação do tecido cutâneo em pacientes com esclerose sistêmica difusa. Além disso, é um aparelho de fácil manuseio, o que possibilita os demais integrantes da equipe de saúde realizarem as medidas

Systemic sclerosis is an autoimmune disease that causes vascular damage and fibrosis. It belongs to the group of connective tissue diseases and is well known because of the high mortality associated with it, especially as a consequence of lung and heart problems. The modified Rodnan skin score is used to evaluate the skin tissue of people with systemic sclerosis. It consists of assessing skin wrinkling, but it can be subjective because of the limitations in the process of obtaining the score, both because of the variability of the professionals who perform the procedure and the perception during the measurement. The present study was proposed to provide resources to objectively measure skin tissue, as an attempt to design an evaluation form based on scientific evidence. Its objective was to correlate modified Rodnan skin scores and measures obtained with durometers collected by two different researchers in patients with diffuse systemic sclerosis. It is an analytical and cross-sectional study, carried out with patients at least 16 years old, of both genders. The sample was made up of 58 patients, with a predominance of people with white skin. There were discreetly moderate and moderate correlations between the evaluations of the measurements obtained with the modified Rodnan score and the durometer equipment carried out by researchers 1 and 2. Agreement between the researchers was assessed by using the kappa coefficient for the modified Rodnan skin score and the interclass correlation coefficient for the durometer equipment, with better results for the measurements collected with the latter method. There was a strong positive correlation between the modified Rodnan skin score, the total average obtained with the durometer, and dermis thickness. It was concluded that durometers can be used as a method to evaluate the skin tissue in patients with diffuse systemic sclerosis. The equipment is easy to handle, which allows other members of the health team to carry out the measurements

Rituximab for the therapy of systemic sclerosis: a series of 10 cases in a single center / Tratamento da esclerose sistêmica com rituximabe: uma série de 10 casos em centro único

Abstract Systemic sclerosis (SSc) is a chronic autoimmune disease with a high morbidity and mortality. Although cyclophosphamide is effective for severe and refractory cases, there is demand for new treatments. The biological treatment with B-cell depletion with rituximab (RTX) has demonstrated efficacy for this demand in open-label studies. Objective This study was conducted with the aim to retrospectively evaluate all patients who used RTX for the treatment of SSc in our center. Patients and methods We retrospectively evaluated medical records of all patients with SSc who used RTX to treat this disease from January 2009 to January 2015. Systemic, cutaneous, and pulmonary involvement data and laboratory results before and six months after the first infusion of RTX were collected. Results Ten patients received treatment during the study period and were included in this series. All patients had a diffuse form of the disease. Five patients suffered from an early (duration of disease shorter or equal to four years), rapidly progressive disease, and another five received RTX at late stages of the disease. In both groups of patients, stabilization of the pulmonary picture was observed, with a fall in the skin score in those patients with early forms of the disease. Discussion Similar to findings in previous studies, RTX was effective in treating early and rapidly progressive forms of SSc. We also found that patients with long-term illness may benefit from the treatment.

Resumo A esclerose sistêmica (ES) é uma doença autoimune crônica de alta morbimortalidade. Ainda que a ciclofosfamida seja eficaz, para casos graves e refratários há demanda para novos tratamentos. A terapia biológica com depleção de células B com rituximabe (RTX) demonstrou eficácia para tal demanda em estudos abertos. Objetivo Avaliar retrospectivamente todos os pacientes que fizeram uso do RTX para tratamento de ES em nosso centro. Pacientes e métodos Foram avaliados retrospectivamente todos os prontuários de pacientes com ES que fizeram uso de RTX para tratamento da ES de janeiro de 2009 a janeiro de 2015. Dados de acometimento sistêmico, cutâneo, pulmonar e laboratoriais antes e seis meses após a primeira infusão de RTX foram coletados. Resultados Dez pacientes receberam o tratamento no período de estudo e foram incluídos na presente série de casos. Todos os pacientes tinham a forma difusa da doença. Cinco pacientes tinham formas iniciais (tempo de doença menor ou igual a quatro anos) e rapidamente progressiva da doença e cinco receberam o RTX em fases tardias da doença. Houve estabilização do quadro pulmonar em ambos os grupos de pacientes e redução no escore cutâneo nos pacientes com formas iniciais da doença. Discussão Similar ao encontrado em estudos prévios, o RTX foi eficaz no tratamento de formas iniciais e rapidamente progressivas da ES. Verificamos também benefício em pacientes com longa duração da doença.

Antifibrotic effects of crocetin in scleroderma fibroblasts and in bleomycin-induced sclerotic mice

OBJECTIVE: To investigate the antifibrotic effects of crocetin in scleroderma fibroblasts and in sclerotic mice. METHODS: Skin fibroblasts that were isolated from three systemic scleroderma (SSc) patients and three healthy subjects were treated with crocetin (0.1, 1 or 10 μM). Cell proliferation was measured with an MTT assay. Alpha-smooth muscle actin was detected via an immunohistochemical method. Alpha 1 (I) procollagen (COL1A1), alpha 1 (III) procollagen (COL3A1), matrix metalloproteinase (MMP)-1 and tissue inhibitor of matrix metalloproteinase (TIMP)-1 mRNA levels were measured using real-time PCR. SSc mice were established by the subcutaneous injection of bleomycin. Crocetin (50 mg/kg/d) was injected intraperitoneally for 14 days. Dermal thickness and lung fibrosis were assessed with Masson's trichrome staining. Plasma ET-1 was detected with an enzyme-linked immunosorbent assay (ELISA). Skin and lung ET-1 and COL1A1 mRNA levels were measured via real-time PCR. RESULTS: Crocetin inhibited the proliferation of SSc and normal fibroblasts, an effect that increased with crocetin concentration and incubation time. Crocetin decreased the expression of α-SMA and the levels of mRNA for COL1A1, COL3A1 and matrix metalloproteinase-1, while crocetin increased TIMP-1 mRNA levels in both SSc and normal fibroblasts. Skin and lung fibrosis was induced, and the levels of ET-1 in the plasma, skin and lungs were elevated in bleomycin-injected mice. Crocetin alleviated the thickening of the dermis and lung fibrosis; decreased COL1A1 mRNA levels in the skin and lung; and simultaneously decreased ET-1 concentrations in the plasma and ET-1 mRNA levels in the skin and lungs of the bleomycin-induced sclerotic mice, especially during the early phase (weeks 1-3). CONCLUSION: Crocetin inhibits cell proliferation, differentiation and collagen production in SSc fibroblasts. Crocetin alleviates skin and lung fibrosis in a bleomycin-induced SSc ...

Esclerose sistêmica e o fenômeno de Raynaud: manejo terapêutico / Systemic sclerosis and Raynaud's phenomenon: therapeutic management

O fenômeno de Raynaud (FRy) caracteriza-se por episódios reversíveis de vasoespasmos de extremidades, que ocorrem usualmente após estresse ou exposição ao frio. O FRy pode ser primário ou secundário a uma série de condições, principalmente a doenças do espectro da esclerose sistêmica (ES). Na ES, o FRy costuma ser mais grave, e lesões isquêmicas de extremidades são frequentes. Nos últimos anos, avanços no estudo da fisiopatologia do FRy e da doença vascular na ES propiciaram o surgimento de novas opções terapêuticas para esta manifestação. Os bloqueadores de canal de cálcio devem ser utilizados como tratamento de primeira escolha para o FRy. Novas drogas, como os inibidores da fosfodiesterase V e os prostanoides, podem ser utilizados em pacientes com FRy grave, e a bosentana (antagonista do receptor da endotelina-1) é indicada para a prevenção de úlceras digitais recorrentes.

Raynaud's phenomenon (RP) is characterized by episodic vasospasm of the extremities, usually in response to stress or cold exposure. It can be primary or secondary to several conditions, especially systemic sclerosis-related diseases. In systemic sclerosis (SSc), RP is usually more severe and digital ischemic lesions are a frequent problem. In recent years, advances in the understanding of the pathophysiology of RP and of SSc vasculopathy led to the development of new therapeutic options for this condition. Calcium-channel blockers are the first choice for the treatment of RP. New drugs including phosphodiesterase type V inhibitors and prostanoids can be used for severe RP, and bosentan (endothelin-1 receptor antagonist) for prevention of recurrent digital ulcers.

Hiponatremia y terapia con inmunoglobulina endovenosa: a propósito de un paciente consíndrome de sobreposición / Hyponatremia and therapy with intravenous immunoglobulin: about a patient with overlap syndrome

El uso de inmunoglobulina endovenosa está cada vez más difundido, tanto para inmunodeficiencias como para enfermedades de orden autoinmune, infecciosas, así como de tipo neurológico. Si bien la infusión de ésta se asocia con algunos efectos adversos sintomáticos, también es cierto que varios pasan desapercibidos. Se presenta el caso de una paciente con síndrome de sobreposición (polimiositis y esclerodermia), la que durante el procedimiento presenta una pseudohiponatremia asociada a una excelente respuesta clínica a este fármaco. Es importante recalcar que esta complicación sólo corresponde a un hallazgo y no tiene indicación de suspender la terapia.

The use of intravenous immunoglobulin is becoming increasingly widespread, for immunodeficiencies, autoimmune, infectious and neurological diseases. Although this infusion is associated with some symptomatic adverse effects, it is also true that many go unnoticed. A case of a patient with overlap syndrome (polymyositis and scleroderma) is reported, who presented with a pseudohiponatremia associated with excellent clinical response to this. It is important to emphasize that this complication only corresponds to a finding and it is not an indication to discontinue the therapy.

Ocular side effects of chloroquine in patients with rheumatoid arthritis, systemic lupus erythematosus and scleroderma.

OBJECTIVE: Ocular complication is a major long term adverse event of chloroquine. The present study was carried out to determine the ocular side effects of chloroquine in patients with rheumatic diseases. MATERIAL AND METHOD: Medical records of patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma (Scl), who received chloroquine for their treatment, at the Division of Rheumatology, Faculty of Medicine, Chiang Mai University between 1 January 1992 and 31 August 2005 were reviewed. Only patients who were older than 16 years, had a clear total accumulative dose and duration of chloroquine therapy, and a regular ophthalmologic examination by ophthalmologists were included in the present study. RESULTS: One hundred and thirty-nine patients (54, 49, and 36 cases of RA, SLE and Scl, respectively) were studied. Forty-eight patients (34.5%) had ocular adverse effects (retinopathy in 37 and corneal deposition in 13 while two patients had both defects). There was no statistical difference in age, mean lean body weight adjusted daily dose, total dosage, and duration of treatment between those with and without ocular side effects. However those with ocular side effects had significantly lower creatinine clearance (66.9 +/- 26.9 vs 72.3 +/- 20.0 ml/min, p = 0.046). CONCLUSION: Ocular side effects of chloroquine were more common in patients with connective tissue diseases who had decreased creatinine clearance. The use of chloroquine in patients with impaired renal function should be of greater concern.

Tratamiento de esclerosis sistémica. Evidencia actual / Treatment of systemic sclerosis. Current evidence

La esclerosis sistémica es una enfermedad del tejido conectivo que de acuerdo al tipo, severidad y momento de diagnóstico puede afectar significativamente el funcionamiento del organismo, incluso llevándolo a la muerte cuando se presenta compromiso fibrótico y endotelial extenso en órganos vitales como pulmón y riñones. En este artículo hacemos una revisión extensa y actualizada del tratamiento de esclerosis sistémica, haciendo énfasis en compromiso de órganos internos y desarrollo de nuevos medicamentos que puedan modificar el curso de la enfermedad. A pesar de que evaluamos la mejor evidencia disponible, se requieren en la mayoría de los casos estudios con mejor diseño metodológico para establecer conclusiones definitivas

Placebo controlled trial of methotrexate in systemic sclerosis.

Eighteen adult patients of systemic sclerosis were included in this prospective study from Rheumatology Clinic of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka during the period of July 1997 to December 1999 to observe the effectiveness of treatment with methotrexate (MTX) versus placebo. Among the eighteen patients 9 patients were randomly assigned to MTX and 9 to placebo therapy. Nine patients were dropped out (6 in placebo and 3 in the MTX group), because of toxicity and non-compliance. Clinical improvement following treatment was observed in 33.33% of the patient in MTX group but none in placebo group, but this difference was not statistically significant. Anorexia, nausea and occasional vomiting were common side effects in MTX group and subsided in most cases with the passage of time despite the continuation of therapy.

Correlacion entre los niveles de citoquinas en el lavado broncoalveolar en una cohorte de pacientes del Hospital Militar Central con esclerosis sistemica (SS), con y sin alveolitis / Cytokines in bronchoalveolar lavage in systemic sclerosis patient

Debido a la preocupación que produce la osteomielitis crónica y los problemas sociales y laborales que conlleva esta enfermedad, nos propusimos sacar adelante un protocolo de tratamiento médico-ortopédico que hemos implementando y desarrollado con la colaboración del Dr. Cesar Arango (Medico Infectólogo) en nuestros pacientes durante 20 años. Hemos utilizado esta metodología en 180 pacientes, 100 de sexo masculino y 80 de sexo femenino. Los segmentos anatómicos afectados fueron: tibia 56 por ciento, fémur 33 por ciento, antebrazo 6 por ciento, pie 4 por ciento y dedos de pie 1 por ciento. La base fundamental del tratamiento es el desbridamiento seriado de las lesiones con el paciente hospitalizado, la toma de cuatro cultivos en la primera intervención, la administración de antibióticos se lleva a cabo de acuerdo a determinación del medico infectólogo que se modifica según el resultado de los cultivos, y el cuidado postoperatorio domiciliario hasta obtener una granulación adecuada de las zonas desbridadas con cultivos negativos. Se rellena el espacio vado producido por los desbridamientos con injertos óseos extraídos de las espinas ilíacas provista la estabilización de la fractura. De estos 180 pacientes, el 97 por ciento se encuentra hasta el momento libre de recidivas, 5 pacientes no cumplieron con las expectativas del tratamiento y 1 paciente fue amputado. Consideramos que esta forma de tratamiento es adecuada para nuestro medio y mejora el pronóstico de los pacientes que sufren esta penosa enfermedad

Papel da lidocaína por via venosa no tratamento da dor na esclerodermia: relato de caso / Intravenous lidocaine to treat scleroderma pain: case report

JUSTIFICATIVA E OBJETIVOS: A esclerodermia ou esclerose sistêmica progressiva é uma doença sistêmica do tecido conjuntivo, de causa desconhecida, que costuma cursar com microangiopatia, isquemia de extremidades e dor intensa. O objetivo deste relato é descrever um caso do emprego de lidocaína por via venosa no tratamento da dor no curso de isquemia e enfatizar a possível ação antiinflamatória dos anestésicos locais nos pacientes com esclerodermia. RELATO DO CASO: Paciente do sexo feminino, 34 anos, auxiliar de enfermagem, portadora de esclerodermia há aproximadamente 8 anos, apresentava dor de elevada intensidade (escala numérica =10) nos membros superiores e inferiores, contínua, diária, acompanhada de alterações tróficas, da cor e da temperatura e pequenas úlceras nas extremidades. A paciente foi submetida a uma sessão semanal de lidocaína a 2 por cento (400 mg) sem vasoconstritor por via venosa durante 10 semanas com alívio da dor, do turgor, da elasticidade da pele e da perfusão periférica. CONCLUSÕES: O alívio da dor e de outros sintomas após a administração de lidocaína por via venosa sugere que os anestésicos locais podem modular a resposta inflamatória em vários estágios da esclerodermia

BACKGROUND AND OBJECTIVES: Scleroderma or progressive systemic sclerosis is a systemic connective tissue disease of unknown origin, which normally courses with microangiopathy, extremities ischemia and severe pain. This report aimed at describing a case of intravenous lidocaine to treat ischemic pain and at emphasizing potential anti-inflammatory action of local anesthetics in scleroderma patients. CASE REPORT: Female patient, clear mulatto 34 years old, nursing assistant, with scleroderma for approximately 8 years, presented with severe continuous, daily pain (numeric scale = 10) in upper and lower limbs, followed by trophic, color and temperature changes, and small ulcers on extremities. Patient was submitted to 1 weekly session of intravenous 2% lidocaine (400 mg) without vasoconstrictor for 10 weeks with pain, turgor, skin elasticity and peripheral perfusion improvement. CONCLUSIONS: Pain and other symptoms relief after intravenous lidocaine suggests that local anesthetics are able to modulate inflammatory response in different scleroderma stages.

JUSTIFICATIVA Y OBJETIVOS: La esclerodermia o esclerosis sistémica progresiva es una enfermedad sistémica del tejido conjuntivo, de causa desconocida, que acostumbra acontecer con microangiopatía, isquemia de extremidades y dolor intenso. El objetivo de este relato es describir un caso del empleo de lidocaína por vía venosa en el tratamiento del dolor en el curso de isquemia y dar énfasis a una posible acción antiinflamatoria de los anestésicos locales en los pacientes con esclerodermia. RELATO DE CASO: Paciente del sexo femenino, 34 anos, auxiliar de enfermera, portadora de esclerodermia hace aproximadamente 8 años, presentaba dolor de elevada intensidad (escala numérica =10) en los miembros superiores e inferiores, continua, diaria, acompañada de alteraciones tróficas, de color y de temperatura y pequeñas úlceras en las extremidades. La paciente fue sometida a una sesión semanal de lidocaína a 2% (400 mg) sin vasoconstrictor por vía venosa durante 10 semanas con alivio del dolor, del turgor, de la elasticidad de la piel y de la perfusión periférica. CONCLUSIONES: El alivio del dolor y de otros síntomas después de la administración de lidocaína por vía venosa sugiere que los anestésicos locales pueden modular la respuesta inflamatoria en varios aprendizajes de la esclerodermia.

Uso da ciclofosfamida endovenosa na esclerose sistêmica / Intravenous cyclophosphamide therapy in systemic sclerosis

Objetivo: Avaliar a eficácia dos pulsos endovenosos de ciclofosfamida no tratamento da esclerose sistêmica (ES) rapidamente progressiva e da doença pulmonar restritiva (DPR). Pacientes e métodos: Estudo retrospectivo avaliando 45 pacientes com ES submetidos a pulsos endovenosos mensais de ciclofosfamida, na dose de 15 mg/Kg. O grupo A era constituído por 13 pacientes com ES difusa rapidamente progressiva, sem comprometimento visceral (pulmonar, cardíaco e renal), com menos de cinco anos de duração de doença, submetidos a 12 pulsos mensais. O método de avaliação seriada da extensão e intensidade do espessamento cutâneo foi o escore cutâneo total (ECT), método original de Rodnan. O grupo B era constituído por 32 pacientes com DPR, sendo 14 com ES difusa (subgrupo B1) e 18 com ES limitada (subgrupo B2), submetidos a 24 pulsos mensais. O principal parâmetro espirométrico para avaliação seriada do acometimento pulmonar foi a capacidade vital forçada (CVF). Resultados: No grupo A, houve significativa redução do ECT, após 12 meses de tratamento (diminuiu de 29,1 mais ou menos 6,7 para 17,8 mais ou menos 5,8, em média) (p=0,003), acompanhado por diminuição dos níveis médios de VHS diminuiu de 36,8 mais ou menos 16,0 mm para 27,5 mais ou menos 17,0 mm, em média) (p=0,05). No grupo B, não houve variação estatisticamente significativa da CVF, ao final do tratamento. Na análise dos subgrupos, o subgrupo B1 não apresentou significância estatística quanto ao ECT e à CVF, avaliados ao início e ao final do tratamento. Com relação ao subgrupo B2, houve diferença estatisticamente significativa quanto à variação do ECT (diminuiu de 16,0 mais ou menos 7,5 para 11,6 mais ou menos 6,5) (p=0,005) e da CVF (aumentou de 68,3 mais ou menos 8,3 para 74,9 mais ou menos 10,5) (p=0,02), ao final do tratamento. Efeitos colaterais ocorreram em 60 por cento dos pacientes predominando náuseas (14 pacientes), leucopenia (10 casos) e infecções agudas (4 pacientes). Dez pacientes (22 por cento) tiveram de suspender o tratamento, sendo cinco casos por ineficácia, três casos por efeitos colaterais (dois por lelucopenia persistente e um devido à cistite hemorrágica) e dois pacientes por óbito devido ao agravamento do quadro pulmonar. Conclusões: Pulsos endovenosos mensais de ciclofosfamida mostraram-se eficientes no tratamento da ES rapidamente progressiva. Sua eficácia terapêutica na DPR ainda necessita ser melhor estabelecida, com o diagnóstico precoce e a definição de padrões...