ABSTRACT
We report on a case of paraneoplastic pemphigus associated with Castleman's disease. Clinical pathologic features were not conclusive. Diagnosis was established thanks to the detection of seric autoantibodies directed against intercellular substance by indirect immunofluorescence on monkey esophagus. The positive result of this test prompted us to reevaluate the patient and to detect the occult neoplasia. The demonstration of autoantibodies against plakins is the key marker of this disease but depends on tests that may not be readily available in many places like immunoprecipitation, immunoblotting, or indirect immunofluorescence over rat bladder. In this setting, tests like indirect immunofluorescence over monkey esophagus, although unspecific, may aid in reaching the appropriate diagnosis. This case illustrates the importance of the laboratory of autoimmunity in the diagnosis of this type of pemphigus.
Subject(s)
Autoimmunity/immunology , Castleman Disease/diagnosis , Castleman Disease/immunology , Pemphigus/diagnosis , Pemphigus/immunology , Skin Neoplasms/diagnosis , Skin Neoplasms/immunology , Acantholysis/pathology , Adult , Castleman Disease/complications , Female , Humans , Pemphigus/complications , Skin Neoplasms/complicationsABSTRACT
Autoimmune hepatitis (AIH) is a hepatocellular inflammation that is characterised by a wide range of histopathologic (periportal interface hepatitis with plasma cell infiltration and piecemeal necrosis), biochemical (hypertransaminasemia, hypergammaglobulinaemia) and autoimmune (several autoantibodies presence) features. This relatively rare disorder frequently affects middle-aged women. There is no pathognomonic marker for AIH diagnosis, therefore it requires a careful rule out of other causes of liver disease together with the detection of a suggestive pattern of clinical and laboratory abnormalities. Scoring system for AIH diagnosis proposed by International Autoimmune Hepatitis Group has been used as a tool in clinical practice but is not sufficiently exclusive in terms of defining prognosis or treatment. AIH has been classified in two subtypes according to autoantibodies detected: 1 and 2, but this classification results in poor clinical implications. Previously known as subtype 3 is at the present included in subtype 1 because no clinical significant differences has been found between them. Aetiology, and molecular mechanisms still remain to be elucitaded in this disease, although viruses, drugs and molecular mimicry act presumably as a trigger in genetically predisposed patients (associated with HLA-DR3 and DR4 haplotypes). On the other hand, immunosuppressive therapy (corticosteroid or azathioprine) generally offers favourable response. Our aim is to review this disease from different points of view, considering: clinical, histopathological, etiologic, genetic, biochemical, autoimmune, treatment and prognosis features.
Subject(s)
Autoimmune Diseases , Hepatitis , Autoantibodies , Autoimmune Diseases/diagnosis , Autoimmune Diseases/epidemiology , Autoimmune Diseases/genetics , Autoimmune Diseases/immunology , Autoimmune Diseases/therapy , Hepatitis/diagnosis , Hepatitis/epidemiology , Hepatitis/genetics , Hepatitis/immunology , Hepatitis/therapy , Humans , PrognosisABSTRACT
La hepatitis autoinmune es una inflamación hepatocelular que se presenta cuando el sistema inmunológico actúa frente a los hepatocitos y que se caracteriza por hallazgos histológicos (hepatitis de interfase con afectación periportal, infiltración de células plasmáticas y necrosis en sacabocados), bioquímicos (hipertransaminasemia e hipergammaglobulinemia) y autoinmunes (presencia de ciertos autoanticuerpos). Este trastorno es relativamente poco frecuente y suele afectar a mujeres de mediana edad. Al no existir un marcador patognomónico para su diagnóstico se requiere una exclusión cuidadosa de otras causas de enfermedad hepática conjuntamente con un patrón clínico y analítico compatible. El criterio de puntuación propuesto por el Grupo Internacional de la Hepatitis Autoinmune para su diagnóstico no es suficientemente específico como para definir la prognosis y el tratamiento. La hepatitis autoinmune se clasifica, según los autoanticuerpos presentes, en subtipos 1 y 2, aunque esta clasificación no muestra repercusiones clínicas importantes. El anteriormente conocido como subtipo 3 no difiere clinicamente del 1 en forma significativa y por tanto debe incluirse dentro de este último grupo.Todavía permanecen por dilucidar la etiología y las bases moleculares de esta enfermedad, originada probablemente por la interacción de diversos factores como la predisposición genética (haplotipos HLA-DR3 y DR4), pérdida de tolerancia inmunológica, formación de neoantígenos por factores desencadenantes como virus o fármacos, y por mimetismo molecular. La terapia inmunosupresora (corticosteroides, azatioprina) ofrece excelentes resultados. Nuestro objetivo es revisar esta enfermedad bajo diferentes puntos de vista, considerando: los aspectos clínicos, histopatológicos, etiológicos, genéticos, bioquímicos, autoinmunes, de tratamiento y pronóstico. (AU)
