Abstract Objectives: To evaluate the inconsistency between clinical diagnosis of death and autopsy findings in adolescents with chronic diseases. Methods: A cross-sectional study including a sample of adolescents’ autopsies who died in a pediatric and adolescent tertiary hospital over 18 consecutive years. During this period, there were n = 2912 deaths, and n = 581/2912(20%) occurred in adolescents. Of these, n = 85/581(15%) underwent autopsies and were analyzed. Further results were divided into two groups: Goldman classes I or II (high disagreement between main clinical diagnosis of death and anatomopathological findings, n = 26) and Goldman classes III, IV or V (low or no disagreement between these two parameters, n = 59). Results: Median age at death (13.5 [10‒19] vs. 13 [10‒19] years, p = 0.495) and disease duration (22 [0‒164] vs. 20 [0‒200] months, p = 0.931), and frequencies for males (58% vs. 44%, p = 0.247) were similar between class I/II vs. class III/IV/V. The frequency of pneumonia (73% vs. 48%, p = 0.029), pulmonary abscess (12% vs. 0%, p = 0.026), as well as isolation of yeast (27% vs. 5%, p = 0.008), and virus (15% vs. 2%, p = 0.029) identified in the autopsy, were significantly higher in adolescents with Goldman class I/II compared to those with Goldman class III/IV/V. In contrast, cerebral edema was significantly lower in adolescents of the first group (4% vs. 25%, p = 0.018). Conclusion: This study showed that 30% of the adolescents with chronic diseases had major discrepancies between clinical diagnosis of death and autopsy findings. Pneumonia, pulmonary abscess, as well as isolation of yeast and virus were more frequently identified at autopsy findings in the groups with major discrepancies. Objectives Methods crosssectional cross sectional 1 years period 291 deaths 581/291220% 581291220 581/2912 20% 581 581/2912(20% 85/58115% 8558115 85/581 15% 85 15 85/581(15% analyzed high 26 III low parameters 59. 59 . 59) Results 13.5 135 5 (13. 10‒19 1019 10 19 [10‒19 vs 0.495 0495 0 495 22 (2 0‒164 0164 164 [0‒164 2 0‒200 0200 200 [0‒200 months 0.931, 0931 0.931 , 931 0.931) 58% 58 (58 44 44% 0.247 0247 247 IIIIVV III/IV/V 73% 73 (73 48 48% 0.029, 0029 0.029 029 12% 12 (12 0% 0.026, 0026 0.026 026 0.026) 27% 27 (27 5% 0.008, 0008 0.008 008 0.008) (15 2% contrast 4% 4 (4 25 25% 0.018. 0018 0.018 018 0.018) Conclusion 30 Pneumonia 29 291220 581/291220 58129122 5812912 581/291 581/2912(20 58115 85/58115 855811 85581 85/58 8 85/581(15 13. (13 10‒1 101 [10‒1 0.49 049 49 ( 0‒16 016 16 [0‒16 0‒20 020 [0‒20 093 0.93 93 (5 0.24 024 24 7 (7 002 0.02 02 (1 000 0.00 00 001 0.01 01 3 29122 581/29122 581291 581/29 581/2912(2 5811 85/5811 8558 85/5 85/581(1 10‒ [10‒ 0.4 04 0‒1 [0‒1 0‒2 [0‒2 09 0.9 9 0.2 0.0 58129 581/2 581/2912( 855 85/ 85/581( [10 0. 0‒ [0‒ 5812 581/ [1 [0 [

Resumo A piomiosite é uma infecção piogênica da musculatura esquelética, decorrente da disseminação hematogênica e geralmente acompanhada de formação de abscesso localizado. Esta infecção da musculatura é raramente descrita em adultos com lúpus eritematoso sistêmico (LES) e, até onde se sabe, ainda não o foi em pacientes com LES juvenil (LESJ). De nossos 289 pacientes com LESJ, uma apresentou piomiosite. Diagnosticada com LESJ aos 10 anos de idade e após seis anos de tratamento com prednisona, azatioprina e hidroxicloroquina, a paciente foi hospitalizada em razão de um histórico de 30 dias de dor insidiosa na coxa esquerda, sem relato algum de trauma aparente ou febre. O exame físico mostrou músculos sensíveis e com endurecimento lenhoso. Os exames laboratoriais revelaram anemia, aumento de reagentes de fase aguda e enzimas musculares normais. A tomografia computadorizada da coxa esquerda mostrou coleção no terço médio do vasto intermédio, sugerindo estágio purulento de piomiosite. Iniciou-se tratamento com antibiótico de largo espectro, que levou à resolução clínica completa. Em suma, descreveu-se o primeiro caso de piomiosite em pacientes com LESJ encontrado neste serviço. Este relato reforça que a presença de dor muscular localizada em pacientes imunocomprometidos, ainda que sem aumento de enzimas musculares, deve sugerir o diagnóstico de piomiosite. Recomenda-se tratamento imediato com antibióticos.

Abstract Pyomyositis is a pyogenic infection of skeletal muscle that arises from hematogenous spread and usually presents with localized abscess. This muscle infection has been rarely reported in adult-onset systemic lupus erythematous and, to the best of our knowledge, has not been diagnosed in pediatric lupus population. Among our childhood-onset systemic lupus erythematous population, including 289 patients, one presented pyomyositis. This patient was diagnosed with childhood-onset systemic lupus erythematous at the age of 10 years-old. After six years, while being treated with prednisone, azathioprine and hydroxychloroquine, she was hospitalized due to a 30-day history of insidious pain in the left thigh and no apparent trauma or fever were reported. Her physical examination showed muscle tenderness and woody induration. Laboratory tests revealed anemia, increased acute phase reactants and normal muscle enzymes. Computer tomography of the left thigh showed collection on the middle third of the vastus intermedius, suggesting purulent stage of pyomyositis. Treatment with broad-spectrum antibiotic was initiated, leading to a complete clinical resolution. In conclusion, we described the first case of pyomyositis during childhood in pediatric lupus population. This report reinforces that the presence of localized muscle pain in immunocompromised patients, even without elevation of muscle enzymes, should raise the suspicion of pyomyositis. A prompt antibiotic therapy is strongly recommended.

A doença de Kikuchi-Fujimoto (DKF) é uma linfadenite necrosante histiocítica autolimitante de origem desconhecida. É digno de nota que a DKF era apenas pouco frequentemente comunicada em pacientes com lúpus eritematoso sistêmico (LES), com rara ocorrência em pacientes com LES juvenil (LESJ). Até onde vai nosso conhecimento, ainda não foi estudada a prevalência de DKF na população pediátrica lúpica. Assim, em um período de 29 anos consecutivos, 5.682 pacientes foram acompanhados em nossa instituição e 289 (5%) satisfaziam os critérios de classificação do American College of Rheumatology para LES; um sofria DKF isolado (0,03%) e apenas um padecia de DKF associada a diagnósticos de LESJ; este caso foi descrito no presente artigo. Uma jovem com 12 anos de idade apresentava-se com febre alta, fadiga e linfadenopatia cervical e axilar. Os anticorpos antinucleares (ANA) estavam negativos, com imunologia positiva para IgM e IgG antivírus do herpes simples tipos 1 e 2. As imagens obtidas por tomografia por emissão de pósitrons com flúor-18-fluoro-desoxi-glicose/tomografia computadorizada (PET/TC) demonstraram linfadenopatia difusa. A biópsia dos linfonodos axilares demonstrou linfadenite necrosante com presença de histiócitos, sem doença linfoproliferativa, compatível com DKF. Transcorridos 30 dias, a paciente apresentou regressão espontânea, não havendo necessidade de tratamento. Nove meses depois, a paciente exibia erupção malar, fotossensibilidade, úlceras orais, linfopenia e ANA 1:320 (padrão nuclear homogêneo). Nessa ocasião, a aplicação do Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) (Índice de Atividade de Doença/LES 2000) teve um escore igual a 10, e a jovem foi tratada com prednisona (1,0 mg/kg/dia) e hidroxicloroquina, demonstrando melhora progressiva dos sinais e sintomas. Em conclusão, DKF é doença benigna e rara em nossa população lúpica pediátrica. Também queremos enfatizar a relevância do diagnóstico de doenças autoimunes durante o acompanhamento de pacientes com DKF.

Kikuchi-Fujimoto disease (KFD) is a self-limiting histiocytic necrotizing lymphadenitis of unknown origin. Of note, KFD was infrequently reported in adult systemic lupus erythematosus (SLE), with rare occurrence in childhood-SLE (C-SLE) patients. To our knowledge, the prevalence of KFD in the paediatric lupus population was not studied. Therefore, in a period of 29 consecutive years, 5,682 patients were followed at our institution and 289 (5%) met the American College of Rheumatology classification criteria for SLE, one had isolated KFD (0.03) and only one had KFD associated to C-SLE diagnoses, which case was reported herein. A 12 year-old female patient had high fever, fatigue and cervical and axillary lymphadenopathy. The antinuclear antibodies (ANA) were negative, with positive IgM and IgG herpes simplex virus type 1 and type 2 serologies. Fluorine-18-fluoro-deoxy-glucose positron emission tomography/computed tomography (PET/CT) imaging demonstrated diffuse lymphadenopathy. The axillary lymph node biopsy showed necrotizing lymphadenitis with histiocytes, without lymphoproliferative disease, compatible with KFD. After 30 days, she presented spontaneous regression and no therapy was required. Nine months later, she developed malar rash, photosensitivity, oral ulcers, lymphopenia and ANA 1:320 (homogeneous nuclear pattern). At that moment the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 10 and she was treated with prednisone (1.0 mg/kg/day) and hidroxychloroquine showing progressive improvement of hers signs and symptoms. In conclusion, KFD is a benign and rare disease in our paediatric lupus population. We also would like to reinforce the relevance of autoimmune diseases diagnosis during the follow-up of patients with KFD.

Cryptococose é uma infecção fúngica causada pelo Cryptococcus neoformans, geralmente associada com imunodeficiências e drogas imunossupressoras, e foi raramente descrita em pacientes com lúpus eritematoso sistêmico (LES), particularmente em LES juvenil (LESJ). De janeiro de 1983 a Junho de 2011, 5,604 pacientes foram seguidos em nosso Hospital Universitário e 283 (5%) casos preencheram critérios de classificação diagnóstica do Colégio Americano de Reumatologia para LESJ. Apenas um (0.35%) destes apresentou meningite criptocócica. Esta paciente teve diagnostico de lúpus aos 10 anos de idade. Aos 15 anos, ela apresentou cefaleia, náuseas e vômitos durante 5 dias, sem febre, após viagem a região de cavernas. Neste momento, ela estava em uso de prednisona 10 mg/dia, azatioprina e hidroxicloroquina. Foi realizada punção lombar e a tintura da Índia foi positiva para cryptococo, a cultura do liquido cerebroespinhal também foi positiva para Cryptococcus neoformans e a pesquisa de antígeno cryptocócico sérico foi positiva em título de 1:280. Azatioprina foi suspensa e anfotericina B liposomal (3 mg/Kg/dia) foi iniciada. No entanto, quatro dias após ela desenvolveu amaurose e coma. A tomografia computadorizada de crânio demonstrou áreas isquêmicas e nódulos sugestivos de infecção fúngica. Após quatro dias, ela desenvolveu sepse grave e vancomicina e meropenem foram iniciados, entretanto foi a óbito devido choque séptico. Portanto, meningite cryptocócica foi uma rara e grave infecção oportunista em uma população de lúpus juvenil. Este estudo reforça a importância do diagnóstico precoce e da pronta introdução de agentes antifúngicos, principalmente em pacientes com história de contato com excrementos de pássaros.

Cryptococcosis is a fungal infection caused by Cryptococcus neoformans, generally associated with immunodeficiency and immunosuppressive agents, and it is rarely reported in systemic lupus erythematosus (SLE), particularly in juvenile SLE (JSLE). From January 1983 to June 2011, 5,604 patients were followed at our University Hospital and 283 (5%) of them met the American College of Rheumatology (ACR) classification criteria for SLE. Only one (0.35%) of our JSLE patients had cryptococcal meningitis and is described in this report. A 10-year old girl was diagnosed with JSLE. By the age of 15 years, she presented persistent headaches, nausea and vomiting for a 5 day period without fever, after a cave-exploring trip. At that moment, she was under 10 mg/day of prednisone, azathioprine and hydroxychloroquine. A lumbar puncture was performed and India ink preparation was positive for cryptococcosis, cerebrospinal fluid culture yielded Cryptococcus neoformans and serum cryptococcal antigen titer was 1:128. Azathioprine was suspended, and liposomal amphotericin B was introduced. Despite of treatment, after four days she developed amaurosis and fell into a coma. A computer tomography of the brain showed diffuse ischemic areas and nodules suggesting fungal infection. Four days later, she developed severe sepsis and vancomycin and meropenem were prescribed, nevertheless she died due to septic shock. In conclusion, cryptococcal meningitis is a rare and severe opportunistic infection in juvenile lupus population. This study reinforces the importance of an early diagnosis and prompt introduction of antifungal agents, especially in patients with history of contact with bird droppings.