Macrophage activation syndrome in a pediatric patient suffering from Still's disease
Introduction: Still's disease in childhood constitutes an inflammatory disorder, of autoimmune origin, which is framed within the group of juvenile idiopathic arthritis, probably being the most peculiar entity of the group. It usually occurs in the form of repeated outbreaks of activity, combin...
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| Format: | Article |
| Language: | English |
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Universidad de Ciencias Médicas de Pinar del Río
2019-01-01
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| Series: | Universidad Médica Pinareña |
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| Online Access: | http://www.revgaleno.sld.cu/index.php/ump/article/view/330 |
| _version_ | 1797720447903596544 |
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| author | Laura Elena Blanco de la Portilla Raúl Basanta Ampudia María del Carmen Blanco de la Portilla |
| author_facet | Laura Elena Blanco de la Portilla Raúl Basanta Ampudia María del Carmen Blanco de la Portilla |
| author_sort | Laura Elena Blanco de la Portilla |
| collection | DOAJ |
| description | Introduction: Still's disease in childhood constitutes an inflammatory disorder, of autoimmune origin, which is framed within the group of juvenile idiopathic arthritis, probably being the most peculiar entity of the group. It usually occurs in the form of repeated outbreaks of activity, combined with periods of remission.
Case report: a sixteen-year-old patient with a history of systemic juvenile arthritis is presented, with a clinical picture of 72 hours of evolution characterized by intermittent fever, cough, pyodermitis, generalized rash and arthritis. During the clinical evolution, it was considered base-disease activation versus sepsis supported on clinical criteria, which was later corroborated by laboratory findings. The patient underwent treatment with broad-spectrum antibiotics, non-steroidal anti-inflammatory drugs, steroids at a rate of 1mg / kg / day, without improvement. Subsequently, the treatment with pulses of methylprednisolone and immunomodulator-therapy was initiated. Finally, a macrophage activation syndrome was diagnosed, supported on clinical laboratory parameters. Patient’s evolvement was slowly and died in the Intensive Care Unit, 22 days after the admission.
Conclusions: the macrophage activation syndrome is one of the secondary forms within the group of hemophagocytic lymphohistiocytosis. The factors that in this context have been associated with its onset are very diverse. Clinically, these processes are characterized by prolonged fever that does not disappear despite antibiotic-therapy. Typically, arthritis is usually absent. The first line of treatment is corticosteroids at high doses. |
| first_indexed | 2024-03-12T09:20:41Z |
| format | Article |
| id | doaj.art-dd5ee252c1324658baa37383b8944b95 |
| institution | Directory Open Access Journal |
| issn | 1990-7990 |
| language | English |
| last_indexed | 2024-03-12T09:20:41Z |
| publishDate | 2019-01-01 |
| publisher | Universidad de Ciencias Médicas de Pinar del Río |
| record_format | Article |
| series | Universidad Médica Pinareña |
| spelling | doaj.art-dd5ee252c1324658baa37383b8944b952023-09-02T14:36:16ZengUniversidad de Ciencias Médicas de Pinar del RíoUniversidad Médica Pinareña1990-79902019-01-01151155162330Macrophage activation syndrome in a pediatric patient suffering from Still's diseaseLaura Elena Blanco de la Portilla0Raúl Basanta Ampudia1María del Carmen Blanco de la Portilla2Universidad de Ciencias Médicas de Pinar del Río. Facultad de Ciencias Médicas "Dr. Ernesto Che Guevara de la Serna"Universidad de Ciencias Médicas de Pinar del Río. Facultad de Ciencias Médicas "Dr. Ernesto Che Guevara de la Serna"Universidad de Ciencias Médicas de Pinar del Río. Facultad de Ciencias Médicas "Dr. Ernesto Che Guevara de la Serna"Introduction: Still's disease in childhood constitutes an inflammatory disorder, of autoimmune origin, which is framed within the group of juvenile idiopathic arthritis, probably being the most peculiar entity of the group. It usually occurs in the form of repeated outbreaks of activity, combined with periods of remission. Case report: a sixteen-year-old patient with a history of systemic juvenile arthritis is presented, with a clinical picture of 72 hours of evolution characterized by intermittent fever, cough, pyodermitis, generalized rash and arthritis. During the clinical evolution, it was considered base-disease activation versus sepsis supported on clinical criteria, which was later corroborated by laboratory findings. The patient underwent treatment with broad-spectrum antibiotics, non-steroidal anti-inflammatory drugs, steroids at a rate of 1mg / kg / day, without improvement. Subsequently, the treatment with pulses of methylprednisolone and immunomodulator-therapy was initiated. Finally, a macrophage activation syndrome was diagnosed, supported on clinical laboratory parameters. Patient’s evolvement was slowly and died in the Intensive Care Unit, 22 days after the admission. Conclusions: the macrophage activation syndrome is one of the secondary forms within the group of hemophagocytic lymphohistiocytosis. The factors that in this context have been associated with its onset are very diverse. Clinically, these processes are characterized by prolonged fever that does not disappear despite antibiotic-therapy. Typically, arthritis is usually absent. The first line of treatment is corticosteroids at high doses.http://www.revgaleno.sld.cu/index.php/ump/article/view/330síndrome de activación macrofágicamacrófagosartritisartritis juvenilartritis reumatoidemacrophage activation syndromemacrophagesarthritisarthritis, juvenilearthritis, rheumatoid |
| spellingShingle | Laura Elena Blanco de la Portilla Raúl Basanta Ampudia María del Carmen Blanco de la Portilla Macrophage activation syndrome in a pediatric patient suffering from Still's disease Universidad Médica Pinareña síndrome de activación macrofágica macrófagos artritis artritis juvenil artritis reumatoide macrophage activation syndrome macrophages arthritis arthritis, juvenile arthritis, rheumatoid |
| title | Macrophage activation syndrome in a pediatric patient suffering from Still's disease |
| title_full | Macrophage activation syndrome in a pediatric patient suffering from Still's disease |
| title_fullStr | Macrophage activation syndrome in a pediatric patient suffering from Still's disease |
| title_full_unstemmed | Macrophage activation syndrome in a pediatric patient suffering from Still's disease |
| title_short | Macrophage activation syndrome in a pediatric patient suffering from Still's disease |
| title_sort | macrophage activation syndrome in a pediatric patient suffering from still s disease |
| topic | síndrome de activación macrofágica macrófagos artritis artritis juvenil artritis reumatoide macrophage activation syndrome macrophages arthritis arthritis, juvenile arthritis, rheumatoid |
| url | http://www.revgaleno.sld.cu/index.php/ump/article/view/330 |
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