Miopatías inflamatorias idiopáticas: una mirada actualizada al diagnóstico y el manejo / Idiopathic inflammatory myopathies: a review

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired immune-mediated diseases, which typically involve the striated muscle with a variable involvement of the skin and other organs. Clinically, they are characterized by proximal muscle weakness, elevation of muscle enzymes, myopathic changes on electromyography and an abnormal muscle biopsy. The different IIM have been classified according to their distinctive histopathologic features in dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). Several myositis-specific antibodies are associated with the different phenotypes, as well as with different risk of neoplastic disease and systemic complications. The basis for the treatment of DM, PM, and IMNM is immunosuppression. For IBM there are only symptomatic treatments. Steroids, associated or not with other immunosuppressant drugs, are the first line of treatment. Biologic drugs will allow future individualized therapies. The 10-year survival of DM, PM and IMNM is 62 to 90%. The leading causes of death are neoplastic, lung and cardiac complications. IBM does not impair survival, although it affects the quality of life.

Pseudoquiste pancreático intramural gástrico en páncreas heterotópico, una causa infrecuente de retención gástrica: caso clínico / Gastric intramural pseudocyst in a heterotopic pancreas causing gastric outlet obstruction: report of one case

Heterotopic pancreas is a silent gastrointestinal malformation that may become clinically evident when complicated by inflammation and pseudocyst formation. We report a 26 year-old male presenting with vomiting, pain and abdominal distention. An abdominal CT scan showed an important gastric distention secondary to a 4-cm cystic lesion located in the antrum wall. An endosonography showed that the lesion obstructed the gastric outlet and was compatible with a pseudocyst. A cysto-gastrostomy was performed draining the cyst. Its high lipase and amylase content confirmed that it was a pancreatic pseudocyst. Six months later, the lesion appeared again and a subtotal gastrectomy was performed Histopathology confirmed ectopic pancreatic tissue.