Apresentação grave de doença de still do adulto

Abstract

Doença de Still do Adulto (DAS), uma variante da artrite reumatóide juvenil, é uma síndrome caracterizada por artralgia, febre elevada, rash cutâneo, linfadenomegalia e hepatoesplenomegalia. O diagnóstico é sempre de exclusão e baseado em critérios clínicos bem definidos, fazendo parte do diagnóstico diferencial infecções, malignidades e doenças imunológicas. O curso da doença varia entre formas leves e bem agressivas, havendo relatos de alguns casos de DSA evoluindo com choque. Paciente branca, feminina, quarenta e nove anos procura o hospital com queixa de febre, náusea e dores pelo corpo. O quadro com início há três semanas é caracterizado por indisposição, artrite simétrica de interfalangianas proximais e metacarpofalangianas, mialgia difusa, vômitos e febre com calafrios. Na primeira semana, havia dor de garganta, com remissão espontânea. Há dois meses notou surgimento de rash maculopapular em tronco, extremidades e face. Os exames revelavam anemia normocítica, plaquetopenia e aumento de formas jovens de leucócitos com descrição de granulações grosseiras de neutrófilos sem alteração na leucometria global. As provas bioquímicas revelavam hepatite e miosite e alterações inflamatórias inespecíficas. Foram iniciados oxacilina e gentamicina, devido à hipótese de endocardite infecciosa. Uma das cinco amostras de hemocultura foi positiva para Staphylococcus aureus. No ecocardiograma transesofágico não foram visualizadas vegetações ou lesões valvares. No quinto dia de antibioticoterapia, apresentou febre, associada a piora do rash e náusea. Identificada esplenomegalia no exame físico e linfonodomegalia cervical posterior esquerda Em três dias, apresentou hipotensão grave, insuficiência renal aguda (IRA), coagulação intravascular disseminada (CIVD) e acidose metabólica, preenchendo critérios para sepse grave e disfunção de múltiplos órgãos e sistemas (DMOS), sendo tratada com volume, drogas vasoativas, glicocorticóide em altas doses e proteína C ativada (drotrecogina). Foram iniciados cefepime e vancomicina empiricamente. Cerca de uma semana depois, após melhora inicial, a paciente desenvolveu nova piora clínica. Prostração, febre, tosse seca e rash, desta vez associados a petéquias em membros inferiores e leucopenia (1590 leucócitos, sendo 650 neutrófilos) surgiram e, como já havia dez dias de curso de cefepime e vancomicina, foi iniciado novo esquema empírico para sepse hospitalar de origem desconhecida com meropenem e fluconazol. Glicocorticóide foi reinstituído como parte da abordagem de choque séptico e foi repetida a ecografia abdominal que não mostrou alterações evolutivas. Diante da evolução, decidiu-se pela terapia imunossupressora com doses elevadas de glicocorticóide, sem suspensão dos antibióticos, sendo seu desfecho favorável. Apenas alguns casos de choque distributivo foram descritos no contexto de DSA e este seria o primeiro relato de um paciente sem o diagnóstico estabelecido se apresentando com SRIS, choque e DMOS como manifestações iniciais da doença.

Adult-onset Still disease (AOSD), the adult variant of the systemic form of juvenile arthritis, is a rheumatic syndrome characterized by arthralgia, high fever, evanescent skin rash, lymphadenopathy, and hepatosplenomegaly. Diagnosis is one of exclusion and strictly based upon clinical criteria, with differential diagnoses including infection, malignancy, and immunologic disorders. The clinical spectrum of the disease ranges from mild to aggressive, and a few cases of diagnosed AOSD evolving with shock have been previously reported. We present the case of a forty-nine year old white woman with a three-month fever and malaise. She had polyarthritis of hands, facial and thoracic skin rash, high spiking fever, nausea, myalgia and sore throat. In the recent history, she had a non-intentional weight loss of seven percent. She had been taking a sulfonylurea for diabetes for the past two years. On the admission, her physical examination only revealed a cutaneous maculopapular rash over the face, trunk, arms and thighs. Laboratory showed mild normochromic anemia, thrombocytopenia and left shift in the white blood cell count with neutrophil granules. There was mild elevation of liver transaminases and muscle enzymes with non-specific inflammatory alterations as well. Splenomegaly was evidenced by abdominal ultrasound. A large serological panel and chest xray were normal. On the third day she had higher fever. One out of 5 samples of blood cultures was positive for Staphylococcus aureus and antibiotics were administered. Considering infective endocarditis a possible diagnosis, the patient was started on oxacyllin and gentamicin. Transesophageal echocardiogram was unremarkable. The abdominal computed tomography confirmed splenomegaly and showed neither collections nor lymphadenopathy. As the source of the fever remained unidentified, a gallium scanning was performed and no abnormalities were found. For the first time, enlarged cervical lymph nodes and splenomegaly were clinically detected. Lymph node biopsy was performed. In 3 days dramatic worsening ensued with multiple organ disfuntion (severe hypotension, renal and haematological compromise). The patient was admitted to the intensive care unit (ICU) and treated for septic shock with vasoactive drug, corticosteroid, large spectrum antibiotics and drotrecogin. Within six days she recovered and was discharged from ICU. Lymph node biopsy showed reactional inflammatory infiltrate, negative for fungus and mycobateria. The tuberculin test reading was masked by the rash. Meanwhile the corticosteroid was withdrawn, spiking fever and maculopapular rash returned along with leucopenia and petechiae. Because AOSD became the main diagnosis, corticosteroid was restarted, this time with higher doses. A serum ferritin level was extremely high (>2000 ng/ mL), reinforcing the diagnosis. The patient gradually improved and was discharged asymptomatic with oral prednisone. Due to its inespecific presentation and its prevalence, the diagnosis of AOSD is often one of exclusion Our patient presented with a subacute febrile illness and, at the time shock and multiple organ dysfunction ensued, the diagnosis had not been established yet. In this setting adding large spectrum antimicrobial therapy to the supportive care instituted seemed mandatory. The initial clinical recovery was attributed to the antimicrobial therapy. On the fourth day of therapy, when the corticosteroid was discontinued, the patient worsened. At that time, fungal or resistant bacteria infection was suspected, the antimicrobial schema was changed and antifungal drugs added. Considering the continuously negative results of cultures and other screening exams for infection, the possibility of shock not related to sepsis emerged. In such context, the response to corticosteroid therapy and the full fitting diagnostic criteria for AOSD made it the presumptive diagnosis. Therefore, our patient had a true systemic inflammatory response syndrome (SIRS) with shock and multiple organ disfunction syndrome due to AOSD. Only a few cases of sepsislike shock were previously described during AOSD. To our best knowledge, this is the first report on a patient with AOSD, without a previous diagnosis of the disease, presenting with SIRS, shock and multiple organ dysfunction.