Síndrome de CREST asociado a shock séptico por osteomielitis hematógena aguda / CREST syndrome associated with septic shock due to acute osteomyelitis hematologic

El síndrome de CREST (calcinosis, fenómeno de Raynaud, dismotilidad esofágica, esclerodactilia, telangiectasias) forma parte del espectro clínico de la esclerosis sistémica, enfermedad del colágeno, denominada en la clasificación clínica como esclerodermia cutánea limitada. Se presenta un paciente masculino de 53 años, raza blanca y procedencia rural, que desde hace 10 años presenta poliartritis, cambios en zonas distales de la piel que subyace adherida al hueso, falanges de manos y pies, fenómeno de Raynaud, aparición de nódulos subcutáneos de pequeño tamaño en varias localizaciones, que luego se tornan calcificaciones. Llega con una úlcera en el quinto metatarsiano del pie izquierdo, de 14 meses de evolución con varios ingresos para tratamiento de la lesión con resolución aparente y luego recidiva. Desarrolla un cuadro de osteomielitis hematógena aguda con shock séptico. El tratamiento multidisciplinario y oportuno permitió la sobrevida del paciente(AU)

CREST's Syndrome (calcinosis, Raynaud's phenomenon, dysmotilitic esofhagical, sclerodactilitys, telanghiectasis), the form departs from the clinical spectrum of the Systemic Sclerosis, disease of collagen, named in the clinical classification like cutaneous limited scleroderma. Patient, masculine of 53 years, white race, peasant procedence. 10 years ago with changes at zones level distally of the skin that underlies once the bone was adhered, phalanges of hands and feet, Raynaud's phenomenon so big a child's appearing of subcutaneous nodules at several locations, that next calcifications appear. Ulcer in metatarsal foot left-hand fifth, of 14 months of evolution with multiple entrances for treatment of the lesion with apparent resolution and next relapse. Develop acute osteomyelitis hematologic with septic shock. The multi-disciplinary and opportune treatment enabled the patient's over-life(AU)

Estudio Retrospectivo: Características Clínicas de la Esclerosis Sistémica en Pacientes de los Hospitales Vicente Corral Moscoso y José Carrasco Arteaga. Enero 2008 ­ Junio 2015 / Retrospective Research: Clinical Features of Systemic Sclerosis in Patients from Vicente Corral Moscoso and José Carrasco Arteaga Hospitals. January 2008 - June 2015

INTRODUCCIÓN: La esclerosis sistémica es una patología crónica del tejido conectivo; de etiología desconocida y caracterizada por presentar vasculopatía, autoinmunidad y fibrosis. Genera importantes repercusiones socio sanitarias y hasta la actualidad no tiene cura. El objetivo de la presente investigación fue determinar las características clínicas de esa enfermedad en la población local. MÉTODOS: Se realizó un estudio de tipo descriptivo retrospectivo. El universo incluyó a 58 pacientes diagnosticados de esclerosis sistémica en el Hospital Vicente Corral Moscoso y José Carrasco Arteaga durante el período comprendido entre Enero 2008 ­ Julio 2015. La información fue recopilada de las historias clínicas digitales y se realizó estadística descriptiva utilizando el programa SPSS versión 20.0. RESULTADOS: La esclerosis sistémica fue más común en el sexo femenino (relación hombre ­ mujer: 1/8.6), el subtipo limitado fue la forma de presentación principal. Las manifestaciones clínicas más frecuentes fueron las cutáneas (93.1%), seguidas de las osteomusculares (84.5%) y el fenómeno de Raynaud (81%). Los anticuerpos antinucleares fueron positivos en más del 70% de los pacientes. CONCLUSIÓN: La esclerosis sistémica es una enfermedad crónica con afección multiorgánica. El conocimiento de las distintas manifestaciones clínicas de esta patología en la población local, facilitará el diagnóstico oportuno y la instauración de un tratamiento apropiado, con el objetivo de mejorar la calidad de vida del paciente y prevenir complicaciones.(au)

BACKGROUND: Systemic sclerosis is a chronic connective tissue disease; of unknown etiology and characterized by vasculopathy, autoimmunity and fibrosis. The aim of this study was to determine the clinical features of this pathology in local population. METHODS: A retrospective descriptive study was performed. The universe included 58 patients with systemic sclerosis at Vicente Corral Moscoso Hospital and Hospital of Specialties José Carrasco Arteaga during January 2008 - July 2015. The information was compiled from medical records; descriptive statistics were performed using the statistical program SPSS version 20.0. RESULTS: Systemic sclerosis was more common in the females (male - female ratio: 1/8.6), the limited subtype was the main presentation form. The most frequent clinical features were skin disorders (93.1%), followed by osteomuscular manifestations (84.5%) and Raynaud's syndrome (81%). Antinuclear antibodies were positive in more than 70% of patients. CONCLUSION: Systemic sclerosis is a chronic entity with multisystem involvement. The knowledge of the different clinical manifestations will facilitate the opportune diagnosis and the establishment of an appropriate treatment; with the purpose of improve the quality of life and preventing complications.(au)

Manejo prostodóntico convencional de una paciente con síndrome de CREST: reporte de caso / Conventional prosthodontics management of a patient with Crest syndrome: case report

El síndrome de CREST se caracteriza por calcinosis, síndrome de Raynaud, dismotilidad esofágica, esclerodactilia y telangiectasia. Presenta signos y síntomas en el sistema estomatognático que merecen consideración en un tratamiento. El objetivo del presente artículo fue reportar las manifestaciones orales de un caso de síndrome de CREST y describir su tratamiento protésico realizado en el Departamento de Rehabilitación Oral de la Universidad Nacional de Colombia. Se reporta una paciente mujer de 42 años, con diagnóstico de síndrome de CREST por su médico tratante, que acude a la facultad de odontología para un tratamiento restaurador. El manejo prostodóntico incluyó coronas, prótesis parcial fija metal-cerámicas y prótesis parcial removible. Los resultados obtenidos nos permiten recomendar las alternativas restaurativas convencionales similares a las usadas en un paciente sin esta condición sistémica, pero manteniendo un control estricto.

The CREST syndrome is characterized by calcinosis, Raynaud's syndrome, esophageal dysmotility, sclerodactyly, and telangiectasia. It has signs and symptoms in the stomatognathic system that deserve consideration in its treatment. The objective of this paper is to report the oral manifestations of a case of CREST syndrome and describe the prosthetic treatment performed in the Department of Oral Rehabilitation in the National University of Colombia. The case is presented of a patient female aged 42 years, diagnosed with CREST syndrome by the treating doctor. She came to the Faculty of Dentistry for restorative treatment. The prosthodontic management included metal-ceramic crowns, fixed partial denture, and removable partial denture. The results demonstrate that conventional restorative alternatives, similar to that used in a patient without this systemic condition, while maintaining strict control of it, can be recommended.

Síndrome de CREST: presentación de un caso / CREST syndrome: presentation of a case

Como esclerodermia, se designa un grupo de enfermedades y síndromes que tienen como característica común la induración y el engrosamiento cutáneos. El síndrome CREST (calcinosis, fenómeno de Raynaud, alteraciones de la motilidad esofágica, esclerodactilia y telangiectasias) es una forma limitada de esclerodermia. En esta modalidad de la entidad es típico que el síndrome de Raynaud anteceda en años a la presentación del resto de los síntomas de enfermedad. En él pueden aparecer manifestaciones de fibrosis insterticial pulmonar, la cual se evidencia clínicamente por estertores húmedos bibasales, muchas veces sin otra forma de expresión, causa hipertensión pulmonar y fallo miocárdico. Es una enfermedad rara, más frecuente en mujeres que en hombres, de 35 a 50 años de edad. Está descrita en ancianos, pero la forma de CREST es muy infrecuente después de los 25 años. El objetivo fue presentar el caso de una mujer de 55 años de edad con un síndrome de CREST, un cor pulmonale y una evolución favorable. Es una entidad poco frecuente, pero que frente a su evidencia clínica debe insistirse en el diagnóstico positivo. Se trata de un caso interesante pues cursó con hipertensión pulmonar, cor pulmonale y su evolución fue satisfactoria...

As scleroderma are called a group of diseases and syndromes having as a common characteristic the skin hardening and thickening. The CREST syndrome (calcinosis, Raynaud phenomena, alterations of the esophageal motility, sclerodactily and telangiectasia) is a limited form of scleroderma. In this modality of the entity, it is typical that Raynaud syndrome precedes in years the presentation of the rest of the disease symptoms. There may appear manifestations of interstitial pulmonary fibrosis, clinically evidenced by bibasilar humid rales, without any other clinical expression, and it causes pulmonary hypertension and myocardial failure. It is a rare disease, more frequent in women than in men, aged 35 to 50 years. It is described in elder people, but the CREST form is very infrequent after the age of 25. Our aim was presenting the case of a 55-years-old woman with the CREST syndrome, a cor pulmonale and a favorable evolution. It is a rarely frequent entity, but when there are clinical evidences, we should insist in the positive diagnosis. It is an interesting case because she presented pulmonary hypertension, cor pulmonale and developed successfully...

Artropatía de Jaccoud y fibrosis pulmonar en síndrome CREST / Jaccoud’s arthropathy and pulmonary fibrosis in CREST syndrome

Se describe el caso de una paciente de 48 años de edad con diagnóstico de síndrome CREST incompleto (variante de esclerosis sistémica limitada), en quien se documenta la presencia de artropatía de Jaccoud de las manos y compromiso pulmonar intersticial por fibrosis pulmonar tipo neumonía intersticial usual, con positividad para factor reumatoide y anticuerpos contra el péptido citrulinado.Palabras clave: síndrome CREST, artropatía, enfermedad pulmonar intersticial, fibrosis pulmonar.

We report a case of a 48 years old patient with diagnosis of incomplete CREST syndrome (variant limited systemic sclerosis) in who we documented the presence of Jaccoud’s arthropathy of the hands and pulmonary involvement by pulmonary fibrosis type usual interstitial pneumonia, with positivity for rheumatoid factor and anti-cyclic citrullinated peptide antibody.Key words: CREST syndrome, joint disease, interstitial lung disease, pulmonary fibrosis.

Talidomida no tratamento de angiodisplasias intestinais em paciente com síndrome CREST: relato de caso e revisão da literatura / Thalidomide as a treatment of intestinal angiodisplastic lesions in a patient with CREST syndrome: case report and literature review

A anemia ferropriva grave secundária à hemorragia digestiva por angiodisplasias intestinais representa um grande desafio terapêutico. Comumente, as ectasias vasculares são múltiplas e dispersas ao longo do intestino, limitando a eficácia do tratamento hemostático local. Nos últimos anos houve significativo avanço no tratamento anti-angiogênico sistêmico das angiodisplasias intestinais, sendo talidomida a droga mais empregada para tal fim. Relatamos o caso de uma paciente de 49 anos com angiodisplasias intestinais secundárias a síndrome CREST (Calcinose, Raynaud, Dismotilidade Esofágica, Esclerodactilia e Telangiectasias). A paciente apresentava quadro de melena recorrente e alta necessidade transfusional, e não obteve resposta clínica após realização de enteroscopia e eletro-coagulação das lesões com plasma de argônio. Após a introdução de talidomida 100mg ao dia, a paciente evoluiu de forma bastante satisfatória. O caso apresentado neste texto, além de demonstrar sucesso da talidomida no tratamento de angiodisplasias intestinais refratárias à eletro-coagulação com plasma de argônio, também revela eficácia da droga na situação específica da síndrome CREST. Tal fato pode ser de grande valia quando da abordagem de hemorragia intestinal por angiodisplasias nesses pacientes, representando nova opção terapêutica...

The severe ferropenic anemia secondary to digestive bleeding due to intestinal angiodisplastic lesions represents a great challenge. Commonly, angiodisplastic lesions are multiples and disperse through the intestine and that fact limits local treatments. Over the last years, there was a great advance in the antiangiogenic treatment of intestinal angiodisplastic lesions and thalidomide was the most employed drug for this purpose. We report a case of a 49 year-old patient with intestinal angiodisplastic lesions due to CREST syndrome (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia). The patient presented repeated episodes of digestive bleeding and did not achieve clinical improvement after enteroscopy and argon plasma coagulation. The treatment consisting of the introduction of thalidomide 100mg per day demonstrated success. The case presented in this text reveals success in the use of thalidomide in the treatment of intestinal angiodisplastic lesions, probably representing a new therapeutic option...

Scleroderma and coronary artery disease: a case report.

We present the case of a 35-year-old woman presenting with the rare combination of scleroderma and ischemic heart disease. Her ECG suggested old inferior wall myocardial infarction. Coronary angiography revealed significant coronary artery disease.

Síndrome de Crest, sclerodermia sistémica progresiva: ¿cómo tratar y mantener un periodonto? Caso clínico / Crest syndrome, progressive systemic scleroderma: how to treat and mantein a periodontium? Clinical case

El síndrome de Crest es una enfermedad autoinmune. Su diagnóstico es un desafío, los tratamientos sistémicos yperiodontal son muy difíciles. El caso clínico que presentamos, tratado en la práctica privada, ofrece una visión de esta enfermedad. Asimismo, permite hacer una crítica a los sistemas de salud que no la contemplan y categorizan como discapacitados a este tipo de pacientes.

A Case of CREST Syndrome with Extensive Calcinosis Cutis / 대한피부과학회지

CREST syndrome is an indolent form of systemic sclerosis and refers to the manifestations of calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasias. We report a typical case of CREST syndrome in a 67-year-old woman, who presented with extensive, hard, calcified nodules and plaques on her shins, fingers, pelvis, and shoulder.

Clinical Significance of Anti-centromere Antibody in Rheumatoid Arthritis / 대한류마티스학회지

OBJECTIVE: Anti-centromere antibody (ACA) is known to be specific for CREST syndrome, but individual studies showed variations in its distribution among related diseases. According to the authors'study on 56 ACA positive patients, 37 patients were known to have rheumatoid arthritis (RA). As a consequence, the authors studied the clinical significance of ACA positive RA patients. MEHTODS: Specific clinical findings, radiologic studies, and laboratory data were investigated on 72 ACA positive and on 50 ACA negative RA patients. ACA tests were performed by indirect immunoflourescence assay with IT-1 cell line using IT-AIT kit (ImmunoThink(r), Korea) RESULTS: No specific differences were noted between the ACA positive and the negative group of RA. However, there were a few notable findings between the low titer and the high titer group of ACA positive RA. In comparison with the low titer group, the high titer group showed lesser disease activity, more cases of seronegative RA (39.2%4.8%) and thyroid diseases (11.8%>0%). They generally showed atypical RA patterns and the antibodies tend to remain at high titer state. CONCLUSION: Since the high titer ACA group of RA patients showed specific clinical findings, it is thought to be necessary to classify such group into a new subset of RA. And such classification would be helpful in diagnosing some atypical forms of RA patients. More studies on these new types of patients as well as their prognoses should be investigated in the future.

Fibrose pulmonar secundária à esclerodermia CREST / Secondary pulmonary fibrosis to CREST scleroderma associated to sjögren syndrome

A esclerodermia (esclerose sistêmica progressiva-ESP) é um estado inflamatório crônico do tecido conjuntivo, de etiologia desconhecida e caracterizada por alteraçöes vasculares em nível de vasos de médio e pequeno calibre. Paralelamente, verifica-se uma diminuiçäo da elasticidade da pele e de outrros tecidos, resultante de uma fibrose que acomete progressivamente todos os órgäos, aparelhos e sistemas. A síndrome de Sjögren (SS) é uma exocrinopatia auto-imune inflamatória que acomete principalmente glândulas salivares e exócrinas, podendo apresentar-se associadas a outras colagenoses como o ESP. Conquanto, a incidência de SS na forma CREST de esclerodermia (calcinose, Raynaud, desmotilidade esofagiana, esclerodactilia,telangiectasia) näo foi até o momento estimada com precisäo. O presente relato descreve o caso de um extenso acometimento pulmonar secundário a sobreposiçäo de SS à esclerodermia CREST. A paciente desenvolveu uma fibrose pulmonar intensa, sobretudo em bases, traduzindo-se clinicamente por franca dispnéia. O interesse do relato dete caso resulta tanto da forma de apresentaçäo das colagenoses como do acometimento pulmonar demonstrado; destacando as características clínicas, achados laboratoriais e aspectos propedêuticos da doença e despertando a atençäo ao valor do diagnóstico preciso e à conduta terapêutica mais apropriada como fatores decisivos à boa evoluçäo do caso.(au)

Primary Biliary Cirrhosis Associated with Multiple Renal Abscess and IgA Nephropathy: A Case Report / 대한간학회지

Primary biliary cirrhosis is a chronic progressive disease most often affecting women. It is characterized by the progressive destruction of the small intrahepatic bile ducts with portal inflammation leading to hepatic fibrosis and cirrhosis. Autoimmune diseases such as Sjogren's syndrome, rheumatoid arthritis, dermatomyositis, CREST syndrome, and autoimmune thyroiditis may be found in up to 80% of the patients. Primary biliary cirrhosis is also associated with renal diseases such as distal renal tubular acidosis, bacteriuria and glomerulopathy, but there is no case report of primary biliary cirhosis with renal manifestations in Korea. We are reporting a case of primary biliary cirrhosis with multiple renal abscess and IgA nephropathy with a review of the literature.

Study on the Clonality of Endothelial Cell Proliferation in Plexiform Lesions in Patients with Pulmonary Hypertension Associated with CREST Syndrome / 결핵

BACKGROUND: The CREST syndrome is an indolent form of progressive systemic sclerosis. Although its clinical progress is indolent, pulmonary hypertension(PH) associated with CREST syndrome have grave prognosis with over 40 percent mortality rate at 2 year follow-up. But the pathogenesis of pulmonary hypertension in this disease is not known, and classified as either primary or secondary PH. Clonality of endothelial cell proliferation in plexiform lesion is a molecular marker which allows distinction between primary and secondary PH. We performed this study to know whether the PH associated with CREST syndrome is a variant of primary PH or is a secondary PH. METHODS: We assessed the X-chromosome inactivation based on the methylation pattern of the human androgen-receptor gene by PCR(HUMARA). Endothelial cells in plexiform lesions from female patients(n=3) with PH associated with CREST syndrome were microdissected from paraffin blocks. Vascular smooth muscle cells and lung parenchyma were also microdissected for clonality studies. RESULTS: The proliferating endothelial cells in 14 plexiform lesions were all polyclonal. Similarly proliferated smooth muscle cells from 5 vessels with medial hypertrophy were also polyclonal. CONCLUSION: These results suggest that the pulmonary hypertension associated with CREST syndrome has different pathogenesis from primary PH and to be classified as secondary PH.

Oclusão de grandes vasos em esclerodermia: relato de caso / Large vessel occlusive diseae in scleroderma: case report

O caso clínico relatado sugere uma associação incomum entre síndrome CREST e trombose de grandes vasos com evolução potencialmente catastrófica como complicação de esclerose sistêmica. Relata-se o caso de um paciente de 54 anos que, após dez anos do diagnóstico de síndrome CREST, apresentou quadro de oclusão de artérias femoral e polplítea, evoluindo para amputação de membro em razão de insucesso terapêutico. A revisão de literatura dos últimos 15 anos mostra poucas descrições de comprometimento de grandes vasos em esclerose sistêmica, sem prevalência exata dos eventos trombóticos. Trata-se de pacientes jovens do sexo feminino com história de fênomeno de Raynaud e longa data e, exceto pelo envolvimento macrovascular, este grupo de doentes não se distinguiu dos demais sem quadro macrovascular, porém conferindo curso clínico com maiores morbidade e mortalidade, por infecções graves e amputações amplas. O estudo anatomopatológico dos vasos envolvidos revela alterações mínimas de aterosclerose, sendo a trombose vascular causa do evento isquêmico da esclerodermia. Estudos histoquímicos sugerem a redução de atividade fibrinolítica no sítio do trombo dos vasos acometidos como possível causa oclusiva.