Asociación lupus eritematoso sistémico y esclerosis tuberosa, un caso / Coincidence of tuberous sclerosis and systemic lupus erythematosus-a case report

La esclerosis tuberosa (ET), también llamada enfermedad de Pringle Bourneville, es una facomatosis con posible afectación dérmica, neurológica, renal y pulmonar. Se caracteriza por el desarrollo de proliferaciones benignas en numerosos órganos, que dan lugar a las diferentes manifestaciones clínicas. Se asocia a la mutación de 2 genes: TSC1 (hamartina) y TSC2 (tuberina), con la alteración funcional del complejo diana de la rapamicina (mTOR). La activación de la señal mTOR ha sido descrita recientemente en el lupus eritematoso sistémico (LES), y su inhibición podría resultar beneficiosa en pacientes con nefritis lúpica. Presentamos el caso de una paciente que 30 años después del inicio de LES con afectación renal grave (glomerulonefritis tipo IV), resuelta con pulsos intravenosos de ciclofosfamida, comenzó con manifestaciones clínicas del complejo esclerosis tuberosa (CET). Consideramos de interés la coexistencia de estas 2 entidades, ya que solo hemos encontrado 2 casos similares en la literatura (AU)

Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest (AU)

Coincidence of tuberous sclerosis and systemic lupus erythematosus-a case report. / Asociación lupus eritematoso sistémico y esclerosis tuberosa, un caso.

Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest.

Lanreotide autogel-induced acute pancreatitis in a patient with acromegaly / Pancreatitis aguda inducida por lanreótida autogel en un paciente con acromegalia

Somatostatin and somatostatin analogues are considered very useful for the treatment of hormone producing tumors and acute variceal bleeding. They have also been proposed for the treatment of acute pancreatitis and for the prevention of post-endoscopic retrograde cholangiopancreatography pancreatitis although clinical trials have failed to show any efficacy. The authors report the case of a 45-year-old man, recently diagnosed of acromegaly, which developed an acute pancreatitis shortly after his first injection of lanreotide autogel. The patient developed a severe dilatation of his hypocontractile gallbladder with distension of the intra and extrahepatic biliary ducts, the choledochus and the main pancreatic duct, without lithiasis or other abnormalities at the papilla, which resolved spontaneously in a month. We consider that lanreotide most likely induced a functional spasm of the Sphincter of Oddi, with impairment of the biliary-pancreatic outflow, leading to an acute pancreatitis, and review the literature concerning this drug related pancreatitis (AU)

La somatostatina y los análogos de la somatostatina se consideran muy útiles para el tratamiento de tumores productores de hormonas y de hemorragias agudas por varices. También se han propuesto para el tratamiento de la pancreatitis aguda y para la prevención de pancreatitis tras una colangiopancreatografía retrógrada endoscópica, aunque los estudios clínicos no han podido demostrar eficacia alguna. Los autores reportan el caso de un hombre de 45 años de edad, con un diagnóstico reciente de acromegalia, que desarrolló una pancreatitis aguda poco después de su primera inyección de lanreótida autogel. El paciente desarrolló una severa dilatación de la vesícula biliar hipocontráctil con distensión de las vías biliares intra y extrahepáticas, el colédoco y el conducto pancreático principal, sin litiasis ni otras anormalidades en la papila, y que se resolvió espontáneamente al mes. Consideramos la posibilidad de que la lanreótida causara un espasmo funcional del esfínter de Oddi, que afectase el flujo biliar-pancrático y condujese a la pancreatitis aguda, y revisamos la literatura sobre pancreatitis relacionada con este fármaco (AU)

Lanreotide autogel-induced acute pancreatitis in a patient with acromegaly.

Somatostatin and somatostatin analogues are considered very useful for the treatment of hormone producing tumors and acute variceal bleeding. They have also been proposed for the treatment of acute pancreatitis and for the prevention of post-endoscopic retrograde cholangiopancreatography pancreatitis although clinical trials have failed to show any efficacy. The authors report the case of a 45-year-old man, recently diagnosed of acromegaly, which developed an acute pancreatitis shortly after his first injection of lanreotide autogel. The patient developed a severe dilatation of his hypocontractile gallbladder with distension of the intra and extrahepatic biliary ducts, the choledochus and the main pancreatic duct, without lithiasis or other abnormalities at the papilla, which resolved spontaneously in a month. We consider that lanreotide most likely induced a functional spasm of the Sphincter of Oddi, with impairment of the biliary-pancreatic outflow, leading to an acute pancreatitis, and review the literature concerning this drug related pancreatitis.