Enfermedad de Whipple

Descripción General

Definición

La enfermedad de Whipple es un síndrome de malabsorción con manifestaciones sistémicas que es causado por la infección por Tropheryma Tropheryma A genus of gram-positive bacteria in the family cellulomonadaceae. Whipple’s Disease whipplei.

Epidemiología

• Trastorno infrecuente con distribución a nivel mundial
• Incidencia global: 1–3 por cada millón de individuos
• Mayor prevalencia en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria agricultores (dado que T. whipplei habita en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el suelo) y en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria trabajadores de las aguas residuales
• Posiblemente asociado al AL Amyloidosis haplotipo HLA-B27
• Más común en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum hombres blancos (proporción hombre:mujer = 8:1)
• Edad media al AL Amyloidosis inicio de los LOS Neisseria síntomas: 49 años

Etiología

El agente causante es T. whipplei:

• Bacilo grampositivo, inmóvil, con forma de bastón
• Relacionado con Actinomicetos
• Frotis ácido–negativo
• Ácido periódico de Schiff positivo
• Envuelto en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum una membrana plasmática trilaminar y cubierto por una pared celular
• Bacteria Bacteria Bacteria are prokaryotic single-celled microorganisms that are metabolically active and divide by binary fission. Some of these organisms play a significant role in the pathogenesis of diseases. Bacteriology que habita en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el suelo y en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las aguas residuales
• Coloniza el tracto intestinal, sistema linfático y sistema nervioso central (SNC)

La inmunodeficiencia del huésped ha HA Hemolytic anemia (HA) is the term given to a large group of anemias that are caused by the premature destruction/hemolysis of circulating red blood cells (RBCs). Hemolysis can occur within (intravascular hemolysis) or outside the blood vessels (extravascular hemolysis). Hemolytic Anemia sido implicada en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las infecciones sintomáticas.

Fisiopatología

• La patogénesis no ha HA Hemolytic anemia (HA) is the term given to a large group of anemias that are caused by the premature destruction/hemolysis of circulating red blood cells (RBCs). Hemolysis can occur within (intravascular hemolysis) or outside the blood vessels (extravascular hemolysis). Hemolytic Anemia sido descrita por completo.
• Se caracteriza por una ausencia general de respuesta inflamatoria del huésped a T. whipplei
• Las alteraciones inmunológicas específicas incluyen:
  • Baja actividad de las células T colaboradoras tipo 1 y aumento de las células T colaboradoras tipo 2
  • Baja proporción de células T CD4:CD8
  • Expresión deficiente del receptor Receptor Receptors are proteins located either on the surface of or within a cell that can bind to signaling molecules known as ligands (e.g., hormones) and cause some type of response within the cell. Receptors del complemento tipo 3 en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las células mononucleares del huésped
  • Disminución de la presentación de antígenos por el aparato del complejo mayor de histocompatibilidad (CMH) clase 2 en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las células epiteliales intestinales
• No hay efectos citotóxicos pronunciados en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las células del huésped, por lo que el organismo puede acumularse en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum grandes cantidades en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria tejidos.
• El organismo es ingerido y se incorpora a los LOS Neisseria macrófagos de los LOS Neisseria tejidos.
• T. whipplei invade diversos tejidos y órganos:
  • Epitelio intestinal
  • Macrófagos
  • Endotelio capilar y linfático
  • Hígado
  • Cerebro
  • Riñones
  • Médula ósea
  • Sinovia
  • Piel
• La infiltración de la lámina propia de los LOS Neisseria intestinos altera la función de las vellosidades y causa malabsorción.
• La infiltración de otros órganos provoca síntomas específicos de cada órgano (e.g., artralgia con infiltración sinovial).

Presentación Clínica

Enfermedad de Whipple clásica

• Artralgia:
  • Con frecuencia, el primer síntoma que se presenta
  • Afecta las grandes articulaciones
  • Migratoria y no destructiva
• Pérdida de peso
• Diarrea:
  • Acuosa o esteatorreicas
  • También puede producirse hemorragia digestiva oculta o grave.
• Dolor Dolor Inflammation abdominal (intermitente, cólico)

Otras manifestaciones

• Cardíacas:
  • Endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis (afectación valvular; la más frecuente)
  • Miocarditis, pericarditis Pericarditis Pericarditis is an inflammation of the pericardium, often with fluid accumulation. It can be caused by infection (often viral), myocardial infarction, drugs, malignancies, metabolic disorders, autoimmune disorders, or trauma. Acute, subacute, and chronic forms exist. Pericarditis
  • Insuficiencia cardíaca congestiva
• Neurológicas:
  • Confusión
  • Convulsiones
  • Delirio
  • Deterioro cognitivo
  • Hipersomnia
  • Síntomas extrapiramidales
  • Ataxia Ataxia Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. Ataxia-telangiectasia y clonus Clonus Ehrlichiosis and Anaplasmosis
  • Hallazgos patognomónicos:
    • Miorritmia oculomasticatoria (movimientos continuos de convergencia ocular con contracciones musculares masticatorias concurrentes)
    • Miorritmia óculo-facial-esquelética
    • Parálisis de la mirada vertical
• Sistémicas:
  • Fiebre
  • Pérdida de peso
  • Linfadenopatía mesentérica o mediastínica (50% de los LOS Neisseria casos)
  • Edema Edema Edema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema periférico (debido a hipoproteinemia secundaria a la nutrición deficiente y a la enteropatía con pérdida de proteínas)

Tratamiento y Pronóstico

Tratamiento

El pilar del tratamiento son antibióticos:

• Dado que existe un riesgo de afectación del SNC, se necesita un antibiótico que pueda penetrar la barrera hematoencefálica.
• Régimen de 1ra línea:
  • Ceftriaxona intravenosa o penicilina G durante 2 semanas (4 semanas para endocarditis Endocarditis Endocarditis is an inflammatory disease involving the inner lining (endometrium) of the heart, most commonly affecting the cardiac valves. Both infectious and noninfectious etiologies lead to vegetations on the valve leaflets. Patients may present with nonspecific symptoms such as fever and fatigue. Endocarditis)
  • Trimetoprim–sulfametoxazol oral durante 12 meses
• Regímenes alternativos para pacientes con alergia a la penicilina y/o sulfas:
  • Meropenem Meropenem A thienamycin derivative antibacterial agent that is more stable to renal dehydropeptidase I than imipenem, but does not need to be given with an enzyme inhibitor such as cilastatin. It is used in the treatment of bacterial infections, including infections in immunocompromised patients. Carbapenems and Aztreonam intravenoso durante 2 o 4 semanas
  • Doxiciclina oral + hidroxicloroquina durante 12 meses

Pronóstico

• La mayoría de los LOS Neisseria pacientes con un tratamiento adecuado evolucionan muy bien.
• Se puede observar una mejora notable en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum un plazo de 7–21 días.
• Los LOS Neisseria daños neurológicos pueden ser a veces irreversibles.
• Complicaciones:
  • Síndrome inflamatorio de reconstitución inmune:
    • Puede desarrollarse en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las primeras semanas de iniciar el régimen de antibióticos
    • Fiebre alta y síntomas que imitan una recaída
  • Recaída:
    • Falta de respuesta clínica a los LOS Neisseria antibióticos
    • PCR PCR Polymerase chain reaction (PCR) is a technique that amplifies DNA fragments exponentially for analysis. The process is highly specific, allowing for the targeting of specific genomic sequences, even with minuscule sample amounts. The PCR cycles multiple times through 3 phases: denaturation of the template DNA, annealing of a specific primer to the individual DNA strands, and synthesis/elongation of new DNA molecules. Polymerase Chain Reaction (PCR) positiva al AL Amyloidosis repetir la biopsia

Diagnóstico Diferencial

• Infección por VIH: el VIH pertenece a la familia Retroviridae Retroviridae The human immunodeficiency virus (HIV) is a species of Lentivirus, a genus of the family Retroviridae, which causes HIV infections and acquired immunodeficiency syndrome (AIDS). The virus has high genetic variability and is divided into 2 major types, HIV type 1 (HIV-1) and HIV type 2 (HIV-2). The human immunodeficiency virus is a single-stranded, positive-sense, enveloped RNA virus, which targets and destroys WBCs, leading to frequent opportunistic infections and, eventually, death. Human Immunodeficiency Virus (HIV). Los LOS Neisseria síntomas de la infección primaria son similares a los LOS Neisseria de la gripe, con fiebre baja, mialgias y linfadenopatías. Tras décadas de infección latente y debilitamiento de la inmunidad del huésped, se desarrolla el SIDA, que se caracteriza por infecciones oportunistas y cánceres. El diagnóstico se establece mediante pruebas de reacción en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum cadena de la polimerasa en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum sangre, y el tratamiento es con medicamentos antirretrovirales.
• Tuberculosis Tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis: enfermedad infecciosa causada por Mycobacterium tuberculosis Mycobacterium tuberculosis Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes. Tuberculosis, una bacteria Bacteria Bacteria are prokaryotic single-celled microorganisms that are metabolically active and divide by binary fission. Some of these organisms play a significant role in the pathogenesis of diseases. Bacteriology ácido-alcohol resistente que puede sobrevivir en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria macrófagos, lo que permite que se produzcan infecciones latentes y asintomáticas durante décadas: los LOS Neisseria síntomas incluyen fiebre, tos TOS Thoracic outlet syndrome (TOS) is a broad term used for a spectrum of syndromes related to the general region of the thoracic outlet, which involves the compression or irritation of elements of the brachial plexus, subclavian artery, or subclavian vein. Thoracic Outlet Syndrome, anorexia Anorexia The lack or loss of appetite accompanied by an aversion to food and the inability to eat. It is the defining characteristic of the disorder anorexia nervosa. Anorexia Nervosa, pérdida de peso, malestar general y manifestaciones extrapulmonares, incluida la pericarditis Pericarditis Pericarditis is an inflammation of the pericardium, often with fluid accumulation. It can be caused by infection (often viral), myocardial infarction, drugs, malignancies, metabolic disorders, autoimmune disorders, or trauma. Acute, subacute, and chronic forms exist. Pericarditis. El diagnóstico se establece con una prueba cutánea de tuberculina, cultivo de esputo e imagenología pulmonar, y la base del tratamiento son los LOS Neisseria medicamentos antimicobacterianos.
• Enfermedad del tejido conectivo: las enfermedades del tejido conectivo comprenden una serie de afecciones: (1) lupus eritematoso sistémico (LES), (2) síndrome antifosfolípido, (3) esclerodermia, (4) miositis y (5) síndrome de Sjögren. Estas enfermedades autoinmunes se caracterizan por la producción de autoanticuerpos y se presentan con síntomas de disfunción inmunitaria. Los LOS Neisseria pacientes presentan síntomas dermatológicos, artropáticos y sistémicos. Cada enfermedad tiene sus propios marcadores serológicos y su propia patogénesis. El tratamiento suele incluir esteroides y medicamentos inmunomoduladores.
• Hipertiroidismo: estado de exceso de síntesis y secreción de hormonas tiroideas; el hipertiroidismo está causado con mayor frecuencia por la enfermedad de Graves o por un bocio multinodular tóxico. Los LOS Neisseria pacientes refieren palpitaciones, trastornos del sueño, ansiedad, pérdida de peso, diarrea, sudoración e intolerancia al AL Amyloidosis calor Calor Inflammation. El examen físico muestra taquicardia, pérdida de peso y temblores en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum las extremidades. El diagnóstico se establece midiendo los LOS Neisseria niveles de hormonas tiroideas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum sangre, y el tratamiento consiste en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum medicamentos antitiroideos y betabloqueadores.
• Enfermedad inflamatoria intestinal con artropatía:
  • Enfermedad de Crohn: afección crónica y recurrente que provoca inflamación transmural en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum parches que puede afectar a cualquier parte del tracto gastrointestinal: la enfermedad de Crohn suele presentarse con diarrea intermitente sin sangre y dolor Dolor Inflammation abdominal tipo cólico. La artropatía es una manifestación extraintestinal para ambos tipos de enfermedad inflamatoria intestinal. El diagnóstico se confirma mediante una biopsia intestinal y el tratamiento incluye regímenes inmunosupresores.
  • Colitis Colitis Inflammation of the colon section of the large intestine, usually with symptoms such as diarrhea (often with blood and mucus), abdominal pain, and fever. Pseudomembranous Colitis ulcerosa: enfermedad inflamatoria idiopática que afecta la superficie de la mucosa del colon Colon The large intestines constitute the last portion of the digestive system. The large intestine consists of the cecum, appendix, colon (with ascending, transverse, descending, and sigmoid segments), rectum, and anal canal. The primary function of the colon is to remove water and compact the stool prior to expulsion from the body via the rectum and anal canal. Colon, Cecum, and Appendix: Anatomy: el recto siempre se ve VE Ventilation: Mechanics of Breathing afectado y la inflamación puede extenderse a lo largo del colon Colon The large intestines constitute the last portion of the digestive system. The large intestine consists of the cecum, appendix, colon (with ascending, transverse, descending, and sigmoid segments), rectum, and anal canal. The primary function of the colon is to remove water and compact the stool prior to expulsion from the body via the rectum and anal canal. Colon, Cecum, and Appendix: Anatomy. La colitis Colitis Inflammation of the colon section of the large intestine, usually with symptoms such as diarrhea (often with blood and mucus), abdominal pain, and fever. Pseudomembranous Colitis ulcerosa suele presentarse con diarrea intermitente, no sanguinolenta, y dolor Dolor Inflammation abdominal tipo cólico. La artropatía es una manifestación extraintestinal para ambos tipos de enfermedad inflamatoria intestinal. El diagnóstico se confirma mediante una biopsia intestinal y el tratamiento incluye regímenes inmunosupresores.