ABSTRACT
Las enfermedades del tejido conectivo vinculadas a implantes mamarios de siliconas han sido tema de discusión. En la última década, la siliconosis ha sido incluida dentro del síndrome autoinmune/inflamatorio inducido por adyuvante (ASIA) junto al síndrome de la guerra del Golfo, síndrome de miofascitis macrofágica y fenómenos post vacunales. El ASIA puede manifestarse como lupus, artritis reumatoidea, o más raramente como enfermedad de Still del adulto. Presentamos el caso de una paciente con fiebre prolongada y criterios clínicos compatibles con ASIA y enfermedad de Still. Se resecaron las prótesis y la anatomía patológica descartó linfoma anaplásico ALK (-) vinculado a prótesis. Los médicos debemos estar alertas ante la aparición de estas nuevas entidades asociadas a los implantes mamarios de siliconas.
Connective tissue diseases associated with silicone breast implants have been widely discussed. In the last decade, siliconosis has been included in the autoimmune/inflammatory syndrome induced by adjuvants (ASIA) next to Gulf War syndrome, macrophage myofascitis and postvaccination phenomena. The ASIA syndrome may appear as lupus, rheumatoid arthritis, or more rarely, as adult Still's disease. We discuss the case of a patient with prolonged fever and clinical criteria for ASIA and Still's disease. The prostheses were resected and pathology showed absence of breast implant associated anaplastic lymphoma ALK (-). Physicians should be alert to these new entities linked to silicone breast implants.
Subject(s)
Humans , Female , Middle Aged , Still's Disease, Adult-Onset/etiology , Breast Implants/adverse effects , Silicone Elastomers/adverse effects , Still's Disease, Adult-Onset/diagnosisABSTRACT
It is an important turning point that early diagnosis and prompts initiation of adequate treatment improve the outcomes of primary or secondary hemophagocytic lymphohistiocytosis (HLH) which is a rare life-threatening syndrome.Even though the survival rate of the patients with HLH has been improved because of the immunochemotherapy or targeted immunotherapy based on new findings in the immunopathogenesis and the genetic defects,reducing the mortality will be main challenge for the pediatricians in pediatric intensive care unit.All of the clinical features are significant clues for the diagnosis,including continuing fever,specific liver dysfunction with coagulation abnormalities,pancytopenia,hypertriglyceridemia and hyperferritinemia.Defining patients' natural killer cells status during the different stages of sepsis and autoimmune diseases will be guiding innovative immunointerventions.
ABSTRACT
La enfermedad de Still del adulto es una enfermedad inflamatoria con compromiso sistémico de origen desconocido, de poca frecuencia en la población general y evolución variable,desde un curso relativamente benigno, hasta enfermedad severa con complicaciones que pueden ser mortales. Se presenta un caso de un paciente de 49 años quien ingresó por un cuadro febril prolongado de un mes de evolución, y dentro de los estudios para fiebre de origen desconocidose descartaron diferentes etiologías, cumpliendo criterios para el diagnóstico de enfermedad de Still del adulto. Posteriormente se hace una revisión de la literatura, evaluando la epidemiología, etiología, clínica, hallazgos de laboratorio, diagnóstico, tratamiento y pronóstico de la enfermedad de Still del adulto.
Adult Stills disease is an inflammatory disease with systemic involvement of unknown origin, not frequent in the general population and with variable evolution, from a relatively benigncourse to a severe disease with potentially fatal complications. We present a case of 49-yearoldpatient who consulted due to a 1-month history of prolonged febrile illness, and among the diagnostic work-up for fever of unknown origin, different etiologies were ruled out while fulfillingthe criteria for diagnosis of adult Stills disease. Furthermore, we review the literature on the epidemiology,etiology, clinical manifestations, laboratory findings, diagnosis, treatment and prognosis of adult Stills disease.
Subject(s)
Humans , Fever , Fever of Unknown Origin , Still's Disease, Adult-OnsetABSTRACT
Adult onset Still 's disease (AOSD)is a rare,distinct clinical entity which affects predominantly young adults aged 16-35.It affects multiple organs,and at present,the etiology is still unclear.Because this disease has few diagnostic or confirmative test,the diagnosis is made by the differential diagnosis and by excluding other diseases.Several reports have suggested a viral trigger in the pathogenesis of this disease.It has some common clinical features ;abrupt onset, high fever,sore throat,transient maculopapular rash,lymphadenopathy,and hepatosplenomegaly.We describe a 17-year-old man who fulfilled the proposed diagnostic criteria of AOSD and had evidence of acute Epstein-Barr virus infection.He complained of intermittent high fever and myalgia for a week.He also had maculopapular rash,sore throat,multiple right cervical lymphadenopahty, and right ankle pain and swelling.After admission,intermittent fever persisted for a month,but diffuse myalgia and migrating arthralgia fluctuated.