Síndrome de fiebre periódica, estomatitis aftosa, faringitis y adenitis / Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

Introducción: El síndrome de fiebre periódica, estomatitis aftosa, faringitis y adenitis es un cuadro relativamente frecuente de curso autolimitado y buen pronóstico. Es la más común de las enfermedades autoinflamatorias que presentan fiebre recurrente y su etiología y fisiopatogenia permanecen inciertas. Objetivo: Describir el caso clínico de un niño que consultó por fiebre recurrente y faringitis y se arribó al diagnóstico de síndrome de fiebre periódica, estomatitis aftosa, faringitis y adenitis. Presentación del caso: Se trata de un paciente de 5 años que consultó por un episodio febril acompañado de faringitis y adenitis, con antecedente de múltiples episodios previos. Conclusiones: El síndrome fiebre periódica, estomatitis aftosa, faringitis y adenitis, es una entidad de curso benigno y autolimitado que constituye la causa más común de fiebre recurrente con un gran impacto en la vida de los pacientes. La fisiopatogenia permanece incierta. Es destacable la sospecha clínica para arribar al diagnóstico ya que no existen en la actualidad exámenes complementarios específicos(AU)

Introduction: Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a relatively common picture of self-limiting course and good prognosis. It is the most common of self-inflammatory diseases with recurrent fever and its etiology and physiopathology remain uncertain. Objective: Describe the clinical case of a boy who was checked due recurrent fever and pharyngitis and being diagnosed with Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Case presentation: This is a 5-year-old patient who was consulted due to a febrile episode accompanied by pharyngitis and adenitis, with a history of multiple previous episodes. Conclusions: Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a benign, self-limiting course entity that is the most common cause of recurrent fever with a large impact on patients' lives. Its physiopathology remains uncertain. Clinical suspicion for diagnosis is noteworthy as there are currently no specific complementary test for it(AU)

Caracterización de pacientes con fiebre de origen desconocido recurrente / Characterizing patients with recurrent fever of unknown origin

Introducción: La fiebre de origen desconocido es un síndrome que genera un gran desafío para el pensamiento clínico; el patrón recurrente es el subtipo más retador y de difícil diagnóstico etiológico. Objetivo: Caracterizar los pacientes con fiebre de origen desconocido con patrón recurrente. Método: Se realizó un estudio descriptivo de corte transversal en el periodo de enero de 2008 a diciembre de 2015. La muestra quedó constituida por 48 pacientes, los cuales fueron hospitalizados y se les realizó una evaluación clínica en el Hospital Clínico Quirúrgico Hermanos Amejeiras. Para el análisis de los datos se utilizaron medidas de resumen para las variables cualitativas y para las cuantitativas, media y desviación estándar. Resultados: La edad media fue 41,3 años, predominaron los hombres (62,5 por ciento), la duración media de la fiebre fue de 497 días y las pistas clínicas más importantes fueron los hallazgos de adenopatías (43,8 por ciento) y hepatomegalia (22,9 por ciento). Los casos sin diagnóstico fueron 45,8 por ciento. Conclusiones: La fiebre de origen desconocido con patrón recurrente es de difícil diagnóstico. A pesar de una evaluación clínica minuciosa, la mayoría de los pacientes quedan sin diagnóstico(AU)

Introduction: Fever of unknown origin is a syndrome that generates great challenge for clinical thought; the recurrent pattern is the most challenging subtype and with difficult etiological diagnosis. Objective: To describe patients with fever of unknown origin with recurring pattern. Method: A descriptive cross-sectional study was carried out from January 2008 to December 2015. Forty-eight patients formed the sample. They were hospitalized and underwent clinical evaluation at Hermanos Amejeiras Surgical Clinical Hospital. For data analysis, summary measures were used for qualitative variables and mean and standard deviation variables were used for quantitative. Results: The mean age was 41.3 years, men predominated (62.5 percent), the mean duration of fever was 497 days, and the most important clinical signs were the findings of lymphadenopathy (43.8 percent) and hepatomegaly. (22.9 percent). Undiagnosed cases were 45.8 percent. Conclusions: Fever of unknown origin with recurring pattern is difficult to diagnose. Despite careful clinical evaluation, most patients remain undiagnosed(AU)

Síndrome PFAPA (febre periódica, aftas orais, faringite e adenite cervical) em crianças e adultos / PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome in children and adults

A síndrome PFAPA (febre periódica, aftas orais, faringite e adenite cervical) é a forma mais comum das doenças autoinflamatórias. Trata-se de uma doença rara, não genética, bimodal, usualmente com início dos sintomas antes dos 5 anos de vida, que também pode prolongar-se ou iniciar-se na vida adulta. A prevalência é variável em todo o mundo, e não há dados estatísticos da doença no Brasil. Diversos pontos da doença são discutíveis, como os critérios diagnósticos, as avaliações laboratoriais, a ausência de mutações genéticas específicas e a abordagem terapêutica e prognóstica. Este trabalho tem como objetivo rever, de forma narrativa e crítica, aspectos relacionados ao diagnóstico e tratamento da síndrome PFAPA em adultos e crianças. Foram feitas buscas nas redes de dados PubMed, Bireme e Cochrane, utilizando o acrônimo PFAPA sem auxílio de qualquer filtro. Quanto ao diagnóstico, os critérios pediátricos foram adaptados para os adultos, com análises variadas e divergentes. Há a necessidade pujante de desenvolver um consenso com diretrizes de diagnóstico de pacientes em populações bem caracterizadas para melhor diagnosticar a doença. Já em relação ao tratamento, a cirurgia com remoção das tonsilas é considerada curativa, com necessidade de se manter acompanhamento após o procedimento para confirmação da remissão da doença. As drogas poupadoras de corticoide ainda são incertas, e o uso de imunobiológicos deve ser reservado somente para pacientes refratários ao tratamento cirúrgico e com grande impacto na qualidade de vida.

PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome is the most common form of autoinflammatory disease. It is a rare, non-genetic, bi-modal disease, usually with onset of symptoms before the age of 5 years, which may also be prolonged or start in adult life. The prevalence varies worldwide and there are no specific statistical data in Brazil. Several aspects of the disease are debatable, such as diagnostic criteria, laboratory evaluations, absence of specific genetic mutations, and therapeutic and prognostic approach. This paper aims to review in a narrative and critical manner aspects related to diagnosis and treatment of PFAPA syndrome in adults and children. PubMed, Bireme and Cochrane databases were searched using the acronym PFAPA without any filter. Regarding diagnosis, pediatric criteria were adapted for use in adults, with varied and divergent analyses. There is a strong need to develop consensus diagnostic guidelines for patients in well-characterized populations to better diagnose the disease. Regarding treatment, tonsillectomy is considered curative, requiring follow-up after the procedure to confirm remission of the disease. Corticosteroid-sparing agents remain uncertain, and the use of immunobiological agents should be reserved only for patients who are refractory to surgical treatment and experience a great impact on quality of life.

<b>A Case of Recurrent Fever Successfully Treated with Yokukansan</b> / 日本東洋医学雑誌

The patient was a 14 year-old female. She had been hospitalized repeatedly since infancy for suspected tonsillitis. During this time, she experienced high fever for about a week once a month. In her school years, she often had a recurrent fever with cervical adenitis, with only a mild inflammatory reaction. We diagnosed her symptom as one of a periodic fever syndrome, triggered by the frequent administered antipyretic drugs for fevers of unknown origin (FUO). We also suspected that there was liver tension, based on her abdominal and back examination, and we prescribed yokukansan. After a 3-month course of yokukansan, she no longer experienced these fevers. Most case reports of FUO describe the use of bupleurum root drugs and tonic formulas as treatment. Although yokukansan has traditionally been used for FUO in older texts, to our knowledge, there are no reports on such use clinically. In the present patient's case, the yokukansan may have reduced or eliminated some type of trigger for the fevers, or it may have affected the regulation of cytokines.

Fevers and the Rheumatologist.

Fevers in children are mainly due to infection, malignancy or inflammatory conditions. Rheumatologists have an important role in the care of inflammatory conditions, many of which are associated with fevers. Seven conditions, the hereditary recurrent fever syndromes, have been defined with the presenting symptom of recurring fever, and for which mutation of a single gene has been defined: Chronic infantile neurological articular syndrome (CINCA), Familial cold autoinflammatory syndrome (FACS), Familial Mediterranean fever (FMF), hyperimmunoglobulinemia D (HIDS), Muckle-Wells syndrome (MWS), Pyogenic sterile arthritis and Pyoderma gangrenosum (PAPA) and Tumour necrosis factor receptorassociated periodic syndrome (TRAPS) . These conditions will be discussed in detail in regard to how they fit into the wider picture of pediatric rheumatological conditions, how the diagnoses may be established and the current recommended treatments for each condition.