Severe still disease in adults

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

A population-based study of tuberculosis incidence among rheumatic disease patients under anti-tnf treatment

Introduction Tuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis. The advent of immunobiologic therapy with TNF inhibitors agents, has been associated with a significant increase in incident cases of tuberculosis in this population. Objective To estimate the incidence of tuberculosis in patients receiving TNF inhibitors therapy for rheumatic diseases. As secondary objectives, we sought to evaluate mortality and the clinical impact of screening for latent tuberculosis infection. Methods This retrospective study included patients with rheumatic diseases of Public Health System from the Brazilian state, a high TB incidence area, who received prescriptions of TNF inhibitors agents between 2006 and 2016. Results A total of 5853 rheumatic disease patients were included. Patients were predominantly women (68.7%) aged 49.5 (± 14.7) years old. Forty-three cases of TB were found (2.86 cases per 1000 person-years; 18 times higher than in the general population). Adalimumab and certolizumab users presented a higher risk for TB development compared to etanercept users (RR: 3.11, 95%CI 1.16–8.35; 7.47, 95%CI 1.39–40.0, respectively). In a subgroup of patients, screening for latent tuberculosis infection was performed in 86% of patients, and 30.2% had a positive tuberculin skin test. Despite latent TB treatment, TB was diagnosed in 2 out of 74 (2.7%) patients. Overall, TB diagnosis did not increase mortality. Conclusion In this population-based study of rheumatic disease patients from a high incident area, TNF inhibitor exposure was associated with an 18-time increased TB incidence. Adalimumab and certolizumab were associated with greater and earlier TB diagnosis compared to etanercept

Ultrasonographic and resistance index evaluation of nails in psoriatic arthritis, psoriasis, and control groups : a cross-sectional study

Background: Nail psoriasis occurs frequently in patients with psoriatic disease, it can lead to functional impairment, pain, discomfort, decreased quality of life and can also be a predictor for the development of arthritis. Early recognition of this condition can provide early and effective treatment and prevent structural impairment. This study aims to identify nail ultrasonographic characteristics in three groups: psoriasis (PsO), psoriatic arthritis (PsA) and controls patients, to determine if the ultrasonography (US) can identify early signs of nail psoriatic impairment or local inflammation. We conducted nail US to determine nail matrix resistance index (NMRI), nail bed resistance index (NBRI), and power Doppler (PD) and grayscale (GS) parameters in these 3 groups. Methods: Single-center, cross-sectional study. GS, PD, and spectral doppler images of bilateral 2nd and 3rd fingernails were acquired from 35 PsO, 31 PsA, and 35 controls patients. An US equipment with an 18 MHz linear transducer for GS and 8.0 MHz for PD was used. PD, NMRI, NBRI, nail plate thickness (NPT), nail bed thickness (NBT), nail matrix thickness (NMT), and morphostructural characteristics of the trilaminar structure (TS) were evaluated in saved images, blind. Results: Mean NMRI and NBRI did not differ between groups. Linear regression analysis detected no relationships between PsO or PsA and NMRI or NBRI. Nail PD grade did not differ between groups. Type I and IV TS changes were more frequent in PsO; types II and III changes were more frequent in PsA (p < 0.001). NPT was greater in PsA and PsO groups than controls: PsA 0.73 ± 0.14 mm, PsO 0.72 ± 0.15 mm, Controls 0.67 ± 0.10 mm (p = 0.001). Conclusion: Echographic TS characteristics of the nail plate and NPT evaluated by GS are useful and can distinguish PsO and PsA nails from controls. NMRI, NBRI, and US nail microcirculation parameters could not distinguish psoriatic nails

Flow cytometry evaluation of CD14/CD16 monocyte subpopulations in systemic sclerosis patients : a cross sectional controlled study

Background: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by vasculopathy and fibrosis, which can be subclassified into diffuse cutaneous (dSSc) and limited cutaneous (lSSc) subtypes. Previous studies suggest that an increase in monocytes can be a hallmark of various inflammatory diseases, including SSc. Our aim was to evaluate circulating blood monocyte subpopulations (classical, intermediate and non-classical) of SSc patients and their possible association with disease manifestations. Methods: Fifty consecutive patients fulfilling the 2013 ACR/EULAR classification criteria for SSc were included in a cross-sectional study. Monocyte subpopulations were identified based on their expression of CD64, CD14 and CD16, evaluated by flow cytometry, and were correlated with the clinical characteristics of the patients; furthermore, the expression of HLA-DR, CD163, CD169 and CD206 in the monocytes was studied. Thirty-eight age- and sex-matched healthy individuals were recruited as a control group. Results: SSc patients had an increased number of circulating peripheral blood monocytes with an activated phenotypic profile compared to healthy subjects. Absolute counts of CD16+ (intermediary and non-classical) monocyte subpopulations were higher in SSc patients. There was no association between monocyte subpopulations and the clinical manifestations evaluated. Conclusion: We identified higher counts of all monocyte subpopulations in SSc patients compared to the control group. There was no association between monocyte subpopulations and major fibrotic manifestations. CD169 was shown to be more representative in dSSc, being a promising marker for differentiating disease subtypes

Fears and beliefs of people living with rheumatoid arthritis : a systematic literature review

Objective: To assess the main fears and beliefs of people with rheumatoid arthritis (RA) and their effect on treatment outcomes; Methods: A systematic literature review was conducted in Pubmed/Medline; original articles published up to May 2017, reporting fears and/or beliefs of adult patients with RA were analyzed. Fears and beliefs were collected by two independent researchers and grouped into categories. Results: Among 474 references identified, 84 were analyzed, corresponding to 24,336 RA patients. Fears were reported in 38.4% of the articles (N = 32/84): most studies described fears related to pharmacological therapy (50.0%, N = 16/32) and fear of disability (28.1%, N = 9/32). Beliefs were reported in 88.0% of articles (N = 74/84) and were found to moderate the patient-perceived impact of RA in 44.6% (N = 33/74), mainly the emotional impact (18.9%, N = 14/74); measures of function, quality of life, fatigue and pain were also found to be affected by patients’ beliefs in 8.1% (N = 6/74), 6.8% (N = 5/74), 2.7% (N = 2/74) and 2.7% (N = 2/74) of the articles, respectively. Beliefs about therapy were linked to adherence in 17.6% of articles (N = 13/74) and beliefs about cause of RA predicted coping patterns in 12.2% of publications (N = 9/74). Only 9.5% (N = 8/84) of articles reported fears and/or beliefs of patients living outside Europe and North America: there was only one work which recruited patients in Latin America and no article included patients from Africa. Conclusion: In RA, patients’ beliefs are linked to impact of disease and non-adherence. Further research is needed on fears/ beliefs of patients living outside Europe and North America

The effects of resistance training with blood flow restriction on muscle strength, muscle hypertrophy and functionality in patients with osteoarthritis and rheumatoid arthritis : a systematic review with meta-analysis

Introduction Rheumatoid arthritis(RA) and osteoarthritis(OA) patients showed systemic manifestations that may lead to a reduction in muscle strength, muscle mass and, consequently, to a reduction in functionality. On the other hand, moderate intensity resistance training(MIRT) and high intensity resistance training(HIRT) are able to improve muscle strength and muscle mass in RA and OA without affecting the disease course. However, due to the articular manifestations caused by these diseases, these patients may present intolerance to MIRT or HIRT. Thus, the low intensity resistance training combined with blood flow restriction (LIRTBFR) may be a new training strategy for these populations. Objective To perform a systematic review with meta-analysis to verify the effects of LIRTBFR on muscle strength, muscle mass and functionality in RA and OA patients. Materials and methods A systematic review with meta-analysis of randomized clinical trials(RCTs), published in English, between 1957–2021, was conducted using MEDLINE(PubMed), Embase and Cochrane Library. The methodological quality was assessed using Physiotherapy Evidence Database scale. The risk of bias was assessed using RoB2.0. Mean difference(MD) or standardized mean difference(SMD) and 95% confidence intervals(CI) were pooled using a random- effects model. A P<0.05 was considered statistically significant. Results Five RCTs were included. We found no significant differences in the effects between LIRTBFR, MIRT and HIRT on muscle strength, which was assessed by tests of quadriceps strength(SMD = -0.01[-0.57, 0.54], P = 0.96; I2 = 58%) and functionality measured by tests with patterns similar to walking(SMD = -0.04[-0.39, 0.31], P = 0.82; I2 = 0%). Compared to HIRT, muscle mass gain after LIRTBFR was reported to be similar. When comparing LIRTBFR with low intensity resistance training without blood flow restriction(LIRT), the effect LIRTBFR was reported to be higher on muscle strength, which was evaluated by the knee extension test. Conclusion LIRTBFR appears to be a promising strategy for gains in muscle strength, muscle mass and functionality in a predominant sample of RA and OA women

Meningite tuberculosa : avaliação da reação em cadeia da polimerase como ferramenta diagnóstica : um estudo piloto

Meningitis is a severe and potentially fatal form of tuberculosis. The diagnostic workup involves detection of acidfast bacilli in the cerebrospinal fluid by microscopy or culture. However the difficulty in detecting the organism poses a challenge to diagnosis. Use of polymerase chain reaction (PCR) in the diagnostic approach to Mycobacterium tuberculosis (MTB) meningitis has been reported as a fast and accurate method, with several commercial kits available. As an alternative, some institutions have been developing inexpensive in-house assays. In our institution, we use an inhouse PCR for tuberculosis. The performance of our PCR for the diagnosis of MTB meningitis was analyzed in 148 consecutive patients, using MTB culture as the gold standard. Sensitivity of cerebrospinal fluid PCR for the diagnosis of MTB meningitis was 50%, specificity was 98.6%, and concordance with culture was 96% (kappa = 0.52). The performance of our PCR is similar to that obtained with the available commercial kits.Meningite é uma forma grave e potencialmente fatal de tuberculose. O diagnóstico envolve a detecção de bacilos álcool-ácido resistentes no líquido cefalorraquidiano por microscopia ou cultura. Entretanto, a dificuldade de detectar o organismo representa um desafio ao diagnóstico. O uso da reação em cadeia da polimerase (PCR) na abordagem diagnóstica de meningite causada por Mycobacterium tuberculosis (MTB) tem sido relatado como um método rápido e preciso, com diversos kits comerciais disponíveis. Como alternativa, algumas instituições vêm desenvolvendo testes in house com baixo custo. Em nossa instituição, usamos PCR in house para tuberculose. O desempenho de nossa PCR para o diagnóstico de meningite causada por MTB foi analisado em 148 pacientes consecutivos, usando a cultura do MBT como padrão-ouro. A sensibilidade da PCR no líquido cefalorraquidiano para o diagnóstico de meningite causada por MTB foi de 50%, especificidade de 98,6% e concordância coma cultura de 96% (kappa = 0,52). O desempenho de nossa PCR é semelhante ao obtido com os kits comerciais disponíveis

Morphological parameters in quadriceps muscle were associated with clinical features and muscle strength of women with rheumatoid arthritis : a cross-sectional study

Background: Rheumatoid arthritis (RA) is an autoimmune, inflammatory and chronic disease that may lead to loss of muscle mass, muscle strength and decreased functionality. Our objectives are to assess the quadriceps muscle morphology by ultrasound (MU) and verify its associations with clinical features, muscle strength and physical function in RA patients. Methods: In this cross-sectional study, RA women ( 18 years) were included. Morphological parameters in quadriceps muscle consisted of the muscle thickness and pennation angle of rectus femoris (RF), vastus intermedius (VI) and vastus lateralis (VL). RA activity was measured by a 28-joint disease activity score (DAS28), muscle strength by handgrip and chair stand tests, and physical function by health assessment questionnaire (HAQ), timed-up-and-go (TUG) test and short physical performance battery (SPPB). Results: Fifty-five patients were included (age: 56.73 9.46 years; DAS28: 3.08 1.29). Muscle thickness in RF, VI and VL were negatively associated with age (RF, p < 0.001; VI, p = 0.013; VL, p = 0.002) and disease duration (RF, p < 0.001; VI, p = 0.005; VL, p = 0.001), and were positively associated with handgrip strength (RF, p = 0.015; VI, p = 0.022; VL, p = 0.013). In addition, decreased muscle thickness in VI (p = 0.035) and a smaller pennation angle in RF (p = 0.030) were associated with higher DAS-28 scores. Conclusion: Quadriceps muscle morphology by ultrasound appears to be affected by age, disease duration, disease activity and muscle strength in patients with RA. MU can be a useful method to evaluate the impact of the disease on skeletal muscle