Síndrome ASIA: prótesis mamarias y enfermedad de Still / ASIA syndrome: breast implant and Still's disease

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.

Adult Onset Stills Disease: A Case Report.

Adult onset still’s disease (AOSD) is a rare inflammatory disorder of unknown etiology. Because of lack of a defined diagnostic test the diagnosis of AOSD can only be made after excluding infectious, malignant, and autoimmune diseases. A 21-year-old female patient was admitted in our hospital with intermittent high-grade fever, polyarthritis and rash. On further history, examination and laboratory investigation she was found to be fulfilling all the Yamaguchi criteria for AOSD. All other causes consisting of acute or chronic infections, hematological malignancies, and other rheumatic disorders were excluded by laboratory investigations. Patient was treated as AOSD with non-steroidal anti-inflammatory drugs and steroids and is completely free of symptoms.

Revisando doença de Still do adulto / Reviewing adult-onset Still's disease

A doença de Still do adulto (DSA) é uma doença inflamatória sistêmica rara, de etiologia desconhecida, caracterizada por febre associada a rash maculoso cor de salmão durante os picos febris, podendo apresentar odinofagia, artralgia/artrite, além de hepatoesplenomegalia e linfadenopatia. Laboratorialmente não possui nenhum exame que seja específico desta enfermidade, apresentando elevação dos níveis de PCR, VHS e ferritina, além de leucocitose e elevação de enzimas de dano hepático. Trata-se de um diagnóstico de exclusão, e deve ser lembrado nos casos de febre de origem indeterminada (FOI), propiciando um diagnóstico precoce e evitando gastos desnecessários com exames subsidiários e internações hospitalares prolongadas.

Adult-onset Still's disease is a rare inflammatory systemic condition of unknown aetiology. The main clinical feature is fever accompained by salmon-pink maculous rash that appears along with fever spikes. Other symptoms are odynophagia, arthralgia or arthritis, enlargement of the spleen and liver, and lymphadenopathy. Although there are no specific laboratorial tests to confirm the condition, C-reactive protein, VHS, ferritin, white blood cells count and liver enzymes are usually elevated. Diagnosis is established by exclusion and must be considered in patients presenting with fever of obscure origin. Early identification reduces costs by avoiding unnecessary tests and long hospital admission.

Doença de Still do adulto: uma causa pouco comum de febre de origem indeterminada / An uncommon cause of fever of unknown origin

Os autores ilustram a dificuldade para se estabelecer o diagnóstico em um paciente com febre de origem indeterminada. Relatam um caso de doença de Still do adulto, diagnosticada após intensa investigaçäo hospitalar. Concluem que a doença de Still do adulto, embora contribua com uma parcela relativamente baixa dentro das causas de febre de origem indeterminada, deve ser lembrada, pois o seu reconhecimento precoce permite poupar o paciente de uma investigaçäo invasiva e dispendiosa, da mesma forma que possibilita a oferta de um tratamento efetivo.