ABSTRACT
BACKGROUND: Pyoderma gangrenosum is a serious cutaneous complication seen in approximately 1 % of patients with inflammatory bowel disease (IBD). Oral corticosteroids are the mainstay treatment, although the evidence supporting their use is weak. AIMS: The purpose of this study was to investigate the characteristics of pyoderma gangrenosum associated with Crohn's disease or ulcerative colitis and which treatments are prescribed in Spanish clinical practice. METHODS: In this retrospective, observational study, the medical records from all patients with IBD and a diagnosis of pyoderma gangrenosum attended by the gastroenterology departments of 12 Spanish hospitals were reviewed. Data on patient demographics and characteristics, underlying IBD and treatment, and pyoderma gangrenosum characteristics, treatment, and outcome were collected and analyzed. RESULTS: The data from 67 patients were analyzed (41 [61.2 %] women, 41 [61.2 %] with Crohn's disease, 25 [37.3 %] with ulcerative colitis, and 1 [1.5 %] with indeterminate disease). The underlying disease was in remission in approximately one-third of patients at the time of presentation of pyoderma gangrenosum. Healing was achieved in all patients (in 3 without any systemic therapy). Oral corticosteroids were taken by 51 patients (76.1 %), almost always as first-line treatment, although definitive healing was attained in 19 (28.4 %). Biologic agents such as infliximab and adalimumab were taken by 31 patients (46.3 %) at some point (first-line in 6 patients [9.0 %]), with definitive healing in 29 patients (93.5 %). CONCLUSIONS: Oral corticosteroid therapy remains the most common treatment for pyoderma gangrenosum associated with inflammatory bowel disease. Biologic therapies such as infliximab and adalimumab should also be considered.
Subject(s)
Colitis, Ulcerative/complications , Crohn Disease/complications , Inflammatory Bowel Diseases/complications , Pyoderma Gangrenosum/drug therapy , Pyoderma Gangrenosum/pathology , Adalimumab , Administration, Oral , Administration, Topical , Adrenal Cortex Hormones/administration & dosage , Adrenal Cortex Hormones/therapeutic use , Adult , Antibodies, Monoclonal/administration & dosage , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized/administration & dosage , Antibodies, Monoclonal, Humanized/therapeutic use , Female , Humans , Infliximab , Male , Middle Aged , Pyoderma Gangrenosum/etiology , Retrospective Studies , Spain , Treatment OutcomeABSTRACT
In the last few years, reports of pancreatic inflammatory diseases caused by immunological mechanisms and with good response to steroid treatment have increased. Although this entity has been known by a number of names, at present the most widely accepted is that of autoimmune chronic pancreatitis (ACP). The present report describes the clinical, immunological, morphological, functional and pathological characteristics of two patients recently studied at our unit and discusses currently used diagnostic tests. The two patients had a complete response to steroid therapy. In our opinion, ACP is probably underdiagnosed in Spain. The availability of morphological, pathological and serological diagnostic tools developed in recent years will help to precisely determine the epidemiology of this process. Thus, quantification of serum levels of anti-carbonic anhydrase II and IgG4 has greatly contributed to the diagnosis of ACP. These tests should be performed in patients with a possible diagnosis of ACP, those suffering from diabetes mellitus type I with impairment of exocrine function, and those with alcoholic pancreatitis and a poor response to alcohol elimination. Once we are able to diagnose and determine the real prevalence of ACP in our setting, the most appropriate therapy and prognosis of this disease can be established.
Subject(s)
Autoimmune Diseases/diagnosis , Pancreatitis/diagnosis , Aged , Alcoholism/complications , Autoantibodies/blood , Autoantibodies/immunology , Autoimmune Diseases/drug therapy , Autoimmune Diseases/epidemiology , Autoimmune Diseases/immunology , Autoimmune Diseases/pathology , Carbonic Anhydrase II/immunology , Cholangiopancreatography, Endoscopic Retrograde , Chronic Disease , Common Bile Duct/pathology , Constriction, Pathologic , Diabetes Complications/diagnosis , Humans , Immunoglobulin G/blood , Immunosuppressive Agents/therapeutic use , Male , Methylprednisolone/therapeutic use , Pancreas/pathology , Pancreatic Ducts/pathology , Pancreatitis/drug therapy , Pancreatitis/epidemiology , Pancreatitis/immunology , Pancreatitis/pathology , Prevalence , Spain/epidemiologyABSTRACT
En los últimos años se han descrito cada vez con mayor frecuencia enfermedades inflamatorias pancreáticas provocadas por mecanismos inmunológicos, y que responde a un tratamiento esteroideo. Aunque esta entidad ha recibido multitud de denominaciones, la más aceptada actualmente es la de pancreatitis crónica autoinmune. En este trabajo describimos los rasgos clínicos, inmunológicos, morfológicos, funcionales y anatomopatológicos de 2 casos que hemos tenido la oportunidad de estudiar recientemente, a la vez que se comentan las pruebas diagnósticas utilizables actualmente. Estos pacientes tuvieron una respuesta completa al tratamiento esteroideo. Probablemente, la pancreatitis autoinmune es una entidad infradiagnosticada hasta el momento en España. La disponibilidad de herramientas diagnósticas basadas en la imagen, la histología y la serología establecidas en los últimos años servirá para determinar con exactitud la epidemiología de esta enfermedad. En este sentido, la determinación de los valores de anticuerpos anti-AC II y de IgG4 en suero ha contribuido enormemente a la sospecha diagnóstica de esta enfermedad. Estas pruebas deberían solicitarse en los pacientes que tengan un diagnóstico posible de pancreatitis autoinmune, que sufran una diabetes mellitus tipo I con deterioro de la función exocrina y en los pacientes con pancreatitis alcohólica con una mala respuesta a la supresión del alcohol. Una vez consigamos diagnosticar y conocer la verdadera prevalen cia de esta enfermedad en nuestro entorno, queda pendiente establecer el tratamiento más correcto junto con su pronóstico
In the last few years, reports of pancreatic inflammatory diseases caused by immunological mechanisms and with good response to steroid treatment have increased. Although this entity has been known by a number of names, at present the most widely accepted is that of autoimmune chronic pancreatitis (ACP). The present report describes the clinical, immunological, morphological, functional and pathological characteristics of two patients recently studied at our unit and discusses currently used diagnostic tests. The two patients had a complete response to steroid therapy. In our opinion, ACP is probably underdiagnosed in Spain. The availability of morphological, pathological and serological diagnostic tools developed in recent years will help to precisely determine the epidemiology of this process. Thus, quantification of serum levels of anti-carbonic anhydrase II and IgG4 has greatly contributed to the diagnosis of ACP. These tests should be performed in patients with a possible diagnosis of ACP, those suffering from diabetes mellitus type I with impairment of exocrine function, and those with alcoholic pancreatitis and a poor response to alcohol elimination. Once we are able to diagnose and determine the real prevalence of ACP in our setting, the most appropriate therapy and prognosis of this disease can be establishe
