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1.
Joint Bone Spine ; 87(6): 538-547, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32758534

RESUMO

Septic arthritis (SA) in an adult native joint is a rare condition but a diagnostic emergency due to the morbidity and mortality and the functional risk related to structural damage. Current management varies and the recommendations available are dated. The French Rheumatology Society (SFR) Bone and Joint Infection Working Group, together with the French Language Infectious Diseases Society (SPILF) and the French Orthopaedic and Trauma Surgery Society (SOFCOT) have worked according to the HAS methodology to devise clinical practice recommendations to diagnose and treat SA in an adult native joint. One new focus is on the importance of microbiological documentation (blood cultures and joint aspiration) before starting antibiotic treatment, looking for differential diagnoses (microcrystal detection), the relevance of a joint ultrasound to guide aspiration, and the indication to perform a reference X-ray. A cardiac ultrasound is indicated only in cases of SA involving Staphylococcus aureus, oral streptococci, Streptococcus gallolyticus or Enterococcus faecalis, or when infective endocarditis is clinically suspected. Regarding treatment, we stress the importance of medical and surgical collaboration. Antibiotic therapies (drugs and durations) are presented in the form of didactic tables according to the main bacteria in question (staphylococci, streptococci and gram-negative rods). Probabilistic antibiotic therapy should only be used for patients with serious symptoms. Lastly, non-drug treatments such as joint drainage and early physical therapy are the subject of specific recommendations.


Assuntos
Artrite Infecciosa , Infecções Estafilocócicas , Adulto , Antibacterianos/uso terapêutico , Artrite Infecciosa/tratamento farmacológico , Artrite Infecciosa/terapia , Humanos , Idioma , Infecções Estafilocócicas/diagnóstico , Infecções Estafilocócicas/tratamento farmacológico , Staphylococcus aureus
2.
SICOT J ; 5: 34, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31560341

RESUMO

We report the case of a 30-year-old woman with histologically proven monostotic fibrous dysplasia of C2 revealed by a pathological fracture of the odontoid process. Radiological investigations showed a ground-glass mineralization of the vertebral body, a centimetric lytic area with poorly defined margins involving the inferior part of the vertebral body and inferior endplate and a fracture through an osteolytic area in the base of the odontoid process. Owing to the vertebral instability, a surgical procedure combining C0-C5 fixation and posterior bone grafting was performed. The surgical biopsy was inconclusive and pathological confirmation was finally obtained through a percutaneous needle biopsy under fluoroscopic guidance. At 26-month follow-up, the patient still experienced mild persistent cervical posterior neck pain and stiffness possibly related to a C5-6 laxity below the intervertebral fixation. This case combines three radiological findings, which are unusual in fibrous dysplasia: monostotic presentation involving the spine, some aggressive radiographic features, and a pathological fracture.

3.
Joint Bone Spine ; 86(3): 369-372, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30735807

RESUMO

OBJECTIVE: Prosthetic joint infection (PJI) is a serious complication of joint replacement surgery. The major pharmacological and surgical treatments required by PJI increase the risk of peri-operative complications in elderly patients. The increase in life expectancy combined with procedural advances make these treatments possible even in the oldest patients. Here, our objective was to compare the characteristics and outcomes of curative PJI treatment in patients < 80 years vs. ≥ 80 years. METHODS: A prospective single-center design was used to compare the characteristics and outcomes of curative treatment for hip or knee PJI in patients < 80 years and ≥ 80 years admitted in 2004-2014. RESULTS: Of 765 patients admitted for PJI, 590 were < 80 years and 124 were ≥ 80 years. Medical history and comorbidities were similar in the two groups. The older group had a significantly higher proportion of patients with American Society of Anesthesiologists Scores ≥ 3 and with streptococcal infection (20% vs. 13%, P < 0.05). After complete surgical excision and prolonged antibiotic therapy, the only event whose frequency differed significantly between the two groups was PJI-related death, which was more common in the older patients (6.5% vs. 0.8%, P < 0.05). The 2-year survival rate after one-stage exchange arthroplasty was > 90% in the ≥80 year group. CONCLUSION: Patients aged 80 years or older are eligible for the same curative pharmacological and surgical PJI treatments used in their younger counterparts. Before surgery, the risk/benefit ratio of the major surgical procedure required to treat PJI must be assessed on a case-by-case basis.


Assuntos
Antibacterianos/uso terapêutico , Artroplastia de Quadril/efeitos adversos , Artroplastia do Joelho/efeitos adversos , Infecções Relacionadas à Prótese/tratamento farmacológico , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Artroplastia de Quadril/métodos , Artroplastia do Joelho/métodos , Estudos de Coortes , Remoção de Dispositivo/métodos , Feminino , França , Avaliação Geriátrica , Prótese de Quadril/efeitos adversos , Humanos , Prótese do Joelho/efeitos adversos , Masculino , Prognóstico , Estudos Prospectivos , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/cirurgia , Reoperação/métodos , Medição de Risco , Resultado do Tratamento
4.
Joint Bone Spine ; 86(2): 239-243, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30266444

RESUMO

OBJECTIVE: Tuberculous prosthetic joint infection (PJI) is uncommon and often diagnosed late. The objective here is to describe the management of tuberculous PJI at an osteoarticular infection referral center. METHODS: A single-center retrospective study of patients managed between 1987 and 2016 was performed. RESULTS: We identified 9 patients with a median age of 80 years. The hip was involved in all 9 patients. A known history of tuberculosis was noted in 2 patients and tuberculosis was present at other sites in 4 patients (lung, n = 3; urinary tract and scrotum, n = 1; and spine, n = 1). The diagnosis was established by routine intra-operative microbiological sampling, during (n = 4) or at a distance from (n = 5) hip arthroplasty. In the 8 patients with available follow-up data, mean antibiotic therapy duration was 16 months (range, 12-18 months). None of the 4 patients in whom the infection was diagnosed during arthroplasty required surgical revision because of the infection. Of the other 5 patients, 3 were managed by exchange arthroplasty and 1 by excision of the hip without subsequent prosthesis implantation; the remaining patient did not undergo revision surgery. The infection was eradicated in all 9 patients, after 15 months to 10 years. CONCLUSION: Tuberculous PJI is uncommon. The prognosis is good with prolonged antibiotic therapy, although the optimal duration remains unclear. The surgical strategy should be discussed on a case-by-case basis. The prosthesis can be retained if the tuberculous infection is an unexpected finding during arthroplasty.


Assuntos
Antituberculosos/administração & dosagem , Prótese de Quadril/efeitos adversos , Mycobacterium tuberculosis/isolamento & purificação , Infecções Relacionadas à Prótese/microbiologia , Reoperação/estatística & dados numéricos , Idoso , Remoção de Dispositivo/métodos , Feminino , Seguimentos , França , Hospitais Universitários , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/epidemiologia , Infecções Relacionadas à Prótese/terapia , Estudos Retrospectivos , Medição de Risco , Estudos de Amostragem , Resultado do Tratamento , Tuberculose Osteoarticular/diagnóstico , Tuberculose Osteoarticular/terapia
5.
J Bone Jt Infect ; 3(5): 241-244, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30533344

RESUMO

Introduction: Prosthetic joint infections (PJIs) can be acquired hematogenously from a distant site or device. Notably, 30%-40% of patients with PJIs have Staphylococcus aureus bacteremia. No case reports or series of PJIs acquired from totally implantable venous-access device (TIVAD) infection or colonization have been published. This study was undertaken to describe epidemiological, clinical, microbiological and radiological characteristics of such PJIs, their treatments and outcomes. Methods: This retrospective study included all patients, identified in a prospective French Bone-and-Joint Infections Referral Center cohort treated between 2004 and 2017, with PJI secondary to TIVAD infection, with the same microbiologically documented microorganism isolated from both. Results: We describe six consecutive hematogenous PJIs (4 women, 2 men; median age: 66.5 years) acquired from TIVAD primary infections. The main infection risk factors were malignancy (n=5) and prior septic arthritis (n=2). Four participants' TIVADs were implanted for chemotherapy, preceding the prosthesis for one patient. The median TIVAD-implantation-to-symptom-onset interval was 12 months. Microorganisms were Staphylococcus epidermidis (n=4), Staphylococcus capitis (n=1) and Staphylococcus aureus (n=1). All TIVADs were removed. Five participants received curative treatment, with a median of 12 weeks of antibiotics. After median follow-up of 42 months, none have relapsed. Conclusions: When PJI occurs in a patient with a TIVAD, the latter must be tested as a potential source of the prosthesis infection. Conversely, PJIs must sought in all patients with bacteremia.

6.
Orphanet J Rare Dis ; 13(1): 127, 2018 07 31.
Artigo em Inglês | MEDLINE | ID: mdl-30064518

RESUMO

BACKGROUND: Fabry disease (OMIM #301500) is an X-linked disorder caused by alpha-galactosidase A deficiency with two major clinical phenotypes: classic and non-classic of different prognosis. From 2001, enzyme replacement therapies (ERT) have been available. We aimed to determine the epidemiology and the functional characteristics of anti-drug antibodies. Patients from the French multicenter cohort FFABRY (n = 103 patients, 53 males) were prospectively screened for total anti-agalsidase IgG and IgG subclasses with a home-made enzyme-linked immunosorbent assay (ELISA), enzyme-inhibition assessed with neutralization assays and lysoGb3 plasma levels, and compared for clinical outcomes. RESULTS: Among the patients exposed to agalsidase, 40% of men (n = 18/45) and 8% of women (n = 2/25) had antibodies with a complete cross-reactivity towards both ERTs. Antibodies developed preferentially in men with non-missense GLA mutations (relative risk 2.88, p = 0.006) and classic phenotype (58.6% (17/29) vs 6.7% (1/16), p = 0.0005). Specific anti-agalsidase IgG1 were the most frequently observed (16/18 men), but the highest concentrations were observed for IgG4 (median 1.89 µg/ml, interquartile range (IQR) [0.41-12.24]). In the men exposed to agalsidase, inhibition was correlated with the total IgG titer (r = 0.67, p < 0.0001), especially IgG4 (r = 0.75, p = 0.0005) and IgG2 (r = 0.72, p = 0.001). Inhibition was confirmed intracellularly in Fabry patient leucocytes cultured with IgG-positive versus negative serum (median: 42.0 vs 75.6%, p = 0.04), which was correlated with IgG2 (r = 0.67, p = 0.017, n = 12) and IgG4 levels (r = 0.59, p = 0.041, n = 12). Plasma LysoGb3 levels were correlated with total IgG (r = 0.66, p = 0.001), IgG2 (r = 0.72, p = 0.004), IgG4 (r = 0.58, p = 0.03) and IgG1 (r = 0.55, p = 0.04) titers. Within the classic group, no clinical difference was observed but lysoGb3 levels were higher in antibody-positive patients (median 33.2 ng/ml [IQR 20.6-55.6] vs 12.5 [10.1-24.0], p = 0.005). CONCLUSION: Anti-agalsidase antibodies preferentially develop in the severe classic Fabry phenotype. They are frequently associated with enzyme inhibition and higher lysoGb3 levels. As such, they could be considered as a hallmark of severity associated with the classic phenotype. The distinction of the clinical phenotypes should now be mandatory in studies dealing with Fabry disease and its current and future therapies.


Assuntos
Anticorpos/sangue , Doença de Fabry/imunologia , Doença de Fabry/patologia , alfa-Galactosidase/antagonistas & inibidores , Adulto , Terapia de Reposição de Enzimas , Ensaio de Imunoadsorção Enzimática , Doença de Fabry/sangue , Doença de Fabry/tratamento farmacológico , Feminino , Glicolipídeos/sangue , Humanos , Imunoglobulina G/sangue , Doenças por Armazenamento dos Lisossomos/sangue , Doenças por Armazenamento dos Lisossomos/tratamento farmacológico , Doenças por Armazenamento dos Lisossomos/imunologia , Doenças por Armazenamento dos Lisossomos/patologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Esfingolipídeos/sangue , alfa-Galactosidase/imunologia
7.
J Rheumatol ; 44(7): 1011-1017, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28412700

RESUMO

OBJECTIVE: Spondyloarthritis (SpA) and Takayasu arteritis (TA) are 2 chronic inflammatory diseases; their coexistence in a single patient is uncommon. The aims of our study were to describe clinical features of patients having SpA associated with TA and to identify some characteristics of the types of patients with SpA associated with TA. We also analyzed treatments used in this context. METHODS: This French multicenter retrospective survey called for observations on behalf of the Club Rhumatismes et Inflammations, with a standardized questionnaire established by the investigators. RESULTS: We included 14 patients (women: 10/14; median age at SpA diagnosis: 43.5 yrs, ranging from 19 to 63). Subtypes of SpA were ankylosing spondylitis (n = 11), psoriatic arthritis (n = 2), and synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome (n = 1). HLA-B27 was positive in 3 cases, negative in 9, and unknown in 2. SpA was diagnosed before TA in 13 cases. Imaging findings compatible with the diagnosis of TA were found with computed tomography (11/14) and/or Doppler ultrasound (10/14). Laboratory tests showed increased acute-phase reactants in all cases (C-reactive protein ≥ 25 mg/l in 71% of the cases). All patients except 1 received corticosteroids and 7 were treated with anti-tumor necrosis factor (anti-TNF). CONCLUSION: Association of SpA and TA is rare but probably not coincidental. Peripheral pulse palpation and vascular auscultation should be systematic and are the first indicators of TA in patients with SpA. Moreover, increased acute-phase reactants during SpA followup should lead to search for TA. Finally, there are therapeutic implications because anti-TNF are efficient in SpA and might be efficient in TA.


Assuntos
Antirreumáticos/uso terapêutico , Espondilartrite/complicações , Arterite de Takayasu/complicações , Proteínas de Fase Aguda/análise , Corticosteroides/uso terapêutico , Adulto , Idoso , Produtos Biológicos/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Espondilartrite/diagnóstico , Espondilartrite/tratamento farmacológico , Avaliação de Sintomas , Arterite de Takayasu/diagnóstico , Arterite de Takayasu/tratamento farmacológico , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Ultrassonografia Doppler , Adulto Jovem
8.
Medicine (Baltimore) ; 95(40): e4996, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27749559

RESUMO

The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD.Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy. Six patients (4M/2F) required a pacemaker (PM) for sinus node dysfunction (n = 4) or atrioventricular disease (n = 2). One female patient required an internal cardioverter-defibrillator (ICD) to prevent sudden cardiac death because of nonsustained ventricular tachycardia (nSVT). One female patient required PM-ICD for sinus node dysfunction and nSVT. One patient underwent CD implantation before the diagnosis of FD. The annual rate of CD implantation was estimated at 1.90 per 100 person years. On univariate analysis at the end of the follow-up period, the factors associated with ACAs requiring CD implantation were as follows: delayed diagnosis of FD, delayed initiation of enzyme replacement therapy, age at the last follow-up visit, and severe multiorgan phenotype (hypertrophic cardiomyopathy, chronic kidney disease, and/or sensorineural hearing loss). On multivariate analysis, age at diagnosis of FD and age at the last follow-up visit were independently associated with an increased risk of ACAs requiring CD (P < 0.05).Considering the high frequency of ACAs requiring CD implantation and the risk of sudden death in patients with FD, regular monitoring is mandatory, especially in patients with a late diagnosis of FD and/or with a severe phenotype. Regular Holter ECGs, therapeutic education of patients, and deliverance of an emergency card including a phenotype summary are crucial in the care of FD patients.Available guidelines for device therapy and the efficacy of enzyme replacement therapy for arrhythmias or conduction abnormalities are discussed.


Assuntos
Arritmias Cardíacas/epidemiologia , Arritmias Cardíacas/terapia , Doença de Fabry/epidemiologia , Adolescente , Adulto , Idoso , Criança , Desfibriladores Implantáveis , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Marca-Passo Artificial , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
9.
JAMA ; 316(11): 1172-1180, 2016 Sep 20.
Artigo em Inglês | MEDLINE | ID: mdl-27654603

RESUMO

IMPORTANCE: One-third of patients with rheumatoid arthritis show inadequate response to tumor necrosis factor α (TNF-α) inhibitors; little guidance on choosing the next treatment exists. OBJECTIVE: To compare the efficacy of a non-TNF-targeted biologic (non-TNF) vs a second anti-TNF drug for patients with insufficient response to a TNF inhibitor. DESIGN, SETTING, AND PARTICIPANTS: A total of 300 patients (conducted between 2009-2012) with rheumatoid arthritis, with persistent disease activity (disease activity score in 28 joints-erythrocyte sedimentation rate [DAS28-ESR] ≥ 3.2 [range, 0-9.3]) and an insufficient response to anti-TNF therapy were included in a 52-week multicenter, pragmatic, open-label randomized clinical trial. The final follow-up date was in August 2013. INTERVENTIONS: Patients were randomly assigned (1:1) to receive a non-TNF-targeted biologic agent or an anti-TNF that differed from their previous treatment. The choice of the biologic prescribed within each randomized group was left to the treating clinician. MAIN OUTCOMES AND MEASURES: The primary outcome was the proportion of patients with good or moderate response according to the European League Against Rheumatism (EULAR) scale at week 24. Secondary outcomes included the EULAR response at weeks 12 and 52; at weeks 12, 24, and 52; DAS28ESR, low disease activity (DAS28 ≤3.2), remission (DAS28 ≤2.6); serious adverse events; and serious infections. RESULTS: Of the 300 randomized patients (243 [83.2%] women; mean [SD] age, 57.1 [12.2] years; baseline DAS28-ESR, 5.1 [1.1]), 269 (89.7%) completed the study. At week 24, 101 of 146 patients (69%) in the non-TNF group and 76 (52%) in the second anti-TNF group achieved a good or moderate EULAR response (OR, 2.06; 95% CI, 1.27-3.37; P = .004, with imputation of missing data; absolute difference, 17.2%; 95% CI, 6.2% to 28.2%). The DAS28-ESR was lower in the non-TNF group than in the second anti-TNF group (mean difference adjusted for baseline differences, -0.43; 95% CI, -0.72 to -0.14; P = .004). At weeks 24 and 52, more patients in the non-TNF group vs the second anti-TNF group showed low disease activity (45% vs 28% at week 24; OR, 2.09; 95% CI, 1.27 to 3.43; P = .004 and 41% vs 23% at week 52; OR, 2.26; 95% CI, 1.33 to 3.86; P = .003). CONCLUSIONS AND RELEVANCE: Among patients with rheumatoid arthritis previously treated with anti-TNF drugs but with inadequate primary response, a non-TNF biologic agent was more effective in achieving a good or moderate disease activity response at 24 weeks than was the second anti-TNF medication. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT01000441.

10.
Joint Bone Spine ; 83(5): 559-62, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-27020953

RESUMO

OBJECTIVES: To describe the occurrence in prosthetic joints of crystal-induced arthritis (CIA) defined as the deposition within the synovial membrane and/or joint cavity of calcium pyrophosphate dehydrate (CPPD) (chondrocalcinosis), sodium urate (gout), or hydroxyapatite. METHODS: We retrospectively reviewed the 7 cases of prosthetic-joint CIA seen between 1993 and 2013 at a medical-surgical center specialized in the management of osteoarticular infections. RESULTS: The 4 females and 3 males ranged in age from 67 to 79 years. Acute CIA occurred at the knee in 6 patients (5 with total knee arthroplasty and 1 with unicompartmental knee arthroplasty) and at the hip in 1 patient (with total hip arthroplasty). Time from arthroplasty to CIA varied from 7 days to 9 years. An abrupt onset was a consistent feature, with pain, complete loss of function, and local evidence of inflammation. A single patient had a fever and 6 patients had laboratory evidence of systemic inflammation. Joint aspiration showed hemarthrosis in 3 patients and inflammatory joint fluid with 20,000 to 79,000neutrophils/mm(3) in 6 patients. Joint fluid cultures were negative in 6 patients. CPPD crystals were evidenced in 5 patients, including 1 who also had hydroxyapatite crystals detected by electron microscopy after alizarin red staining. Monosodium urate crystals were found in 1 patient. The remaining patient had both CPPD crystals and positive cultures for Campylobacter fetus. In 5 patients, treatment with colchicine or a nonsteroidal antiinflammatory drug ensured prompt control of the symptoms and systemic inflammation. The patient with total hip arthroplasty underwent joint aspiration for hemarthrosis. In 1 patient, an intraarticular injection of triamcinolone hexacetonide improved the symptoms and systemic inflammation. The patient with Campylobacter fetus infection was treated with antibiotics, excision of the abscess, and synovectomy. CONCLUSION: CIA may occur after arthroplasty, within synovial membrane remains or neosynovium developed around the prosthetic joint. CIA is a manifestation of a metabolic disease that persists and can reactivate after surgery. Routine testing for crystals is rarely performed in patients with sterile arthritis of a prosthetic joint, and crystals are difficult to detect in joints with hemarthrosis; consequently, the frequency of prosthetic-joint CIA may be underestimated. Although rare, CIA should be considered routinely when symptoms suggesting septic arthritis develop in a prosthetic joint, in order to avoid unnecessary prolonged antibiotic therapy and, in some cases, surgery. The treatment is usually simple.


Assuntos
Artroplastia de Quadril/efeitos adversos , Artroplastia do Joelho/efeitos adversos , Artropatias por Cristais/etiologia , Articulação do Quadril , Prótese Articular/efeitos adversos , Articulação do Joelho , Idoso , Idoso de 80 Anos ou mais , Artropatias por Cristais/diagnóstico , Feminino , Humanos , Masculino , Estudos Retrospectivos
11.
Rheumatology (Oxford) ; 55(2): 291-300, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26350487

RESUMO

OBJECTIVE: We describe myelodysplastic syndrome (MDS)-associated systemic inflammatory and autoimmune diseases (SIADs), their treatments and outcomes and the impact of SIADs on overall survival in a French multicentre retrospective study. METHODS: In this study, 123 patients with MDS and SIADs were analysed. RESULTS: Mean age was 70 years (s.d. 13) and the male:female ratio was 2. The SIADs were systemic vasculitis in 39 (32%) cases, CTD in 31 (25%) cases, inflammatory arthritis in 28 (23%) cases, a neutrophilic disorder in 12 (10%) cases and unclassified in 13 cases (11%). The SIADs fulfilled the usual classification criteria in 75 (66%) cases, while complete criteria were not reached in 21 (19%) cases. A significant association was shown between chronic myelomonocytic leukaemia (CMML) and systemic vasculitis (P = 0.0024). One hundred and eighteen (96%) SIAD patients were treated (91% with steroids), with an 83% response to first-line treatment, including 80% for steroids alone. A second-line treatment for SIADs was required for steroid dependence or relapse in 48% of cases. The effect of MDS treatment on SIADs could be assessed in 11 patients treated with azacytidine and SIAD response was achieved in 9/11 (80%) and 6/11 (55%) patients at 3 and 6 months, respectively. Compared with 665 MDS/CMML patients without SIADs, MDS/CMML patients with SIADs were younger (P < 0.01), male (P = 0.03), less often had refractory anaemia with ring sideroblasts (P < 0.01), more often had a poor karyotype (16% vs 11%, P = 0.04) and less frequently belonged to low and intermediate-1 International Prognostic Scoring System categories, but no survival difference was seen between patients with MDS-associated SIADs and without SIADs (P = 0.5). CONCLUSION: The spectrum of SIADs associated to MDS is heterogeneous, steroid sensitive, but often steroid dependent.


Assuntos
Autoimunidade/imunologia , Azacitidina/uso terapêutico , Glucocorticoides/uso terapêutico , Inflamação/imunologia , Leucemia Mielomonocítica Crônica/imunologia , Síndromes Mielodisplásicas/imunologia , Idoso , Antimetabólitos Antineoplásicos/uso terapêutico , Quimioterapia Combinada , Feminino , Seguimentos , França , Humanos , Inflamação/tratamento farmacológico , Inflamação/etiologia , Leucemia Mielomonocítica Crônica/complicações , Leucemia Mielomonocítica Crônica/tratamento farmacológico , Masculino , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/tratamento farmacológico , Prognóstico , Estudos Retrospectivos
12.
Joint Bone Spine ; 83(4): 421-6, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-26697993

RESUMO

OBJECTIVES: Fabry disease is a rare X-linked metabolic disorder characterized by a deficiency in the enzyme alpha-galactosidase A. Both males and females can be affected. The main presenting symptom is pain in the extremities, whereas at a more advanced stage, the manifestations include hypertrophic cardiomyopathy, cardiac dysrhythmia, proteinuria, chronic kidney dysfunction, stroke, and hearing loss. When not diagnosed and treated, Fabry disease causes early death. No studies specifically designed to describe the musculoskeletal manifestations of Fabry disease are available. METHODS: We conducted a single-center retrospective study of patients receiving follow-up at a Fabry disease referral center. We described the musculoskeletal manifestations and analyzed the differential diagnoses. RESULTS: Our study included 40 patients belonging to 20 families, including 25 females with a mean age of 44.2 years (range, 20-76 years) and 15 males with a mean age of 40.1 years (range, 16-61 years). Mean age at the diagnosis of Fabry disease was 37.2 years (range, 7-71 years) in the females and 26.9 years (range, 9-51 years) in the males. Specific enzyme replacement therapy was given to 10 (40%) females and 12 (80%) males. Musculoskeletal manifestations were as follows: past or present pain in the extremities (13 females and 10 males), combined in some patients with vasomotor disorders in the extremities and telangiectasia; exercise intolerance (12 females and 12 males); osteoporotic fractures (2 brothers aged 45 and 44 years, respectively); osteoporosis (3 females, aged 57, 63, and 75 years, respectively), which contributed to death in the oldest patient; osteopenia (2 females aged 38 and 47 years, respectively; and 1 male aged 43 years); Charcot foot and lymphedema with serious infectious complications (4 males older than 40 years), with avascular osteonecrosis of the lower limbs in 2 cases; toe amputations (3 cases); bilateral lower-limb amputation (1 case); abnormally slender lower limbs (5 females and 8 males); acute gout (3 males with severe chronic kidney failure); and carpal tunnel syndrome (1 female and 1 male, both younger than 40 years). Mistaken diagnoses that were made at an early stage, contributing to delay the identification of Fabry disease, included rheumatic fever (2 females and 2 males), growing pains (2 males), pain with paralysis (1 female), chilblains of the lower limbs (1 female), and erythermalgia (1 female). In adulthood, the following mistaken diagnoses were made: Sjögren's syndrome and/or sicca syndrome (6 females), systemic sclerosis (1 male), dysautonomia (1 female), and familial Mediterranean fever (1 female). CONCLUSION: The diagnosis of Fabry disease is usually delayed, due to confusion with more common disorders. Musculoskeletal manifestations may constitute the presenting symptoms. Past or present pain in the extremities is typical. Osteoporosis may develop early and become severe. Together with the family history, the presence of musculoskeletal manifestations can lead to the correct diagnosis by prompting alpha-galactosidase assays in males and genetic testing in females. Fabry disease is often responsible for musculoskeletal manifestations, of which the most common are pain in the extremities and osteoporosis. These manifestations can be inaugural and lead to diagnostic wanderings. They require specific treatment strategies.


Assuntos
Doença de Fabry/diagnóstico , Doença de Fabry/epidemiologia , Doenças Musculoesqueléticas/diagnóstico , Doenças Musculoesqueléticas/epidemiologia , Adulto , Distribuição por Idade , Idoso , Artralgia/diagnóstico por imagem , Artralgia/epidemiologia , Artrite Gotosa/diagnóstico , Artrite Gotosa/epidemiologia , Estudos de Coortes , Comorbidade , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Osteoartrite/diagnóstico por imagem , Osteoartrite/epidemiologia , Osteoporose/diagnóstico por imagem , Osteoporose/epidemiologia , Fraturas por Osteoporose/diagnóstico por imagem , Fraturas por Osteoporose/epidemiologia , Prognóstico , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Distribuição por Sexo , Taxa de Sobrevida , Centros de Atenção Terciária , Adulto Jovem
13.
Int J Infect Dis ; 29: 184-9, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25447723

RESUMO

OBJECTIVES: To describe elderly patients treated with prolonged suppressive antibiotic therapy for a prosthetic joint infection (PJI) in cases where the infected prosthesis could not be removed. METHODS: All patients aged ≥80 years with a documented PJI and treated with prolonged suppressive antibiotic therapy for more than 6 months were included retrospectively in this study. The following events were noted: failure including persisting infection, relapse, new infection, treatment discontinuation due to severe adverse events, and related death, and also unrelated death. RESULTS: Thirty-eight patients with a median age of 84 years (80-95 years) were included; there were 24 hip infections, 13 knee infections, and one shoulder infection. The main causative organisms were Staphylococcus aureus (39%) and Streptococcus agalactiae (16%). The most commonly prescribed antibiotics as prolonged suppressive therapy were penicillins. The median follow-up duration was 24 months; 60% of the patients were event-free at 24 months and were still on prolonged suppressive antibiotic therapy. Fifteen events (six failures and nine unrelated deaths) were observed. Hypoalbuminaemia, the presence of a sinus tract, and a staphylococcal PJI were associated with an increased risk of an event. CONCLUSIONS: Prolonged suppressive antibiotic therapy is an alternative therapy in elderly patients with PJI when surgery is contraindicated and when the bacteria are susceptible to well-tolerated oral antimicrobial therapy such as beta-lactams.


Assuntos
Prótese Articular/efeitos adversos , Infecções Relacionadas à Prótese/tratamento farmacológico , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Feminino , Humanos , Prótese Articular/microbiologia , Masculino , Infecções Relacionadas à Prótese/microbiologia , Infecções Relacionadas à Prótese/cirurgia , Recidiva , Estudos Retrospectivos , Staphylococcus aureus/isolamento & purificação , Resultado do Tratamento
14.
Medicine (Baltimore) ; 93(1): 1-10, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24378738

RESUMO

We describe the characteristics and outcome of inflammatory arthritis in patients with myelodysplastic syndrome (MDS) in a French multicenter retrospective study. Twenty-two patients with MDS (median age, 77.5 yr [interquartile range, 69-81]; 10 women) were included. Inflammatory arthritis presented as polyarthritis in 17 cases (77%) and with symmetric involvement in 15 cases (68%). At diagnosis, the median disease activity score 28 based on C-reactive protein (DAS28-CRP) was 4.5 [2-6.5]. Two patients had anti-citrullinated protein antibodies (ACPAs), and 1 had radiologic erosions. The median time between the diagnoses of arthritis and MDS was 10 months [6-42], with a median articular symptom duration of 3 months [2-8]. The diagnosis of both diseases was concomitant in 6 cases (27%); arthritis preceded MDS in 12 cases (55%), and occurred after MDS in 4 (18%). While the number of swollen and tender joints significantly decreased during follow-up, as did the median DAS28-CRP (from 4.3 [3.8-4.6] at baseline to 2.9 [1.75-3.3]; p < 0.05), CRP remained elevated (CRP >20 mg/L) in 8 patients (42%). Nevertheless, radiographic progression and new ACPA positivity were not observed during a median follow-up of 29 months [9-76]. While most of the patients were treated with steroids (n = 16) for arthritis, additional treatment was administered in only 4 patients (hydroxychloroquine, n = 2; sulfasalazine [Salazopyrin] and etanercept, n = 1, respectively). Eleven patients died during follow-up from acute myeloid leukemia (n = 5); infections (n = 3); or cerebral bleeding, cardiorespiratory failure, or undetermined cause (n = 1, respectively). Inflammatory arthritis associated with MDS can have various presentations and is often seronegative and nonerosive. Steroids alone are the most common treatment in MDS-associated arthritis, but that treatment is insufficient to control arthritis. Steroid-sparing strategies need to be identified.


Assuntos
Artrite/complicações , Síndromes Mielodisplásicas/complicações , Idoso , Idoso de 80 Anos ou mais , Artrite/tratamento farmacológico , Artrite/epidemiologia , Feminino , França/epidemiologia , Glucocorticoides/uso terapêutico , Humanos , Masculino , Síndromes Mielodisplásicas/epidemiologia , Polimialgia Reumática/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento
15.
Rheumatology (Oxford) ; 53(3): 532-9, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24282319

RESUMO

OBJECTIVES: Rituximab has been shown to induce remission of ANCA-associated vasculitides (AAVs). Our study was undertaken to describe AAV clinical responses to rituximab used for remission-induction and/or maintenance therapy, assess rituximab's safety profile and evaluate French clinical practices. METHODS: This retrospective study concerned AAV patients who had received one or more rituximab infusion between 2002 and January 2011 and had follow-up lasting ≥12 months. RESULTS: Eighty patients were included, most with refractory or relapsing AAV: 70 (88%) with granulomatosis with polyangiitis (GPA), 9 (11%) with microscopic polyangiitis and 1 (1%) with eosinophilic GPA. Rituximab was the first agent used to induce remission in 73 patients. The two most commonly administered regimens were an infusion of 375 mg/m(2)/week for 4 weeks (54 patients) and an infusion of 1 g every 2 weeks for a month (16 patients). Rituximab was first prescribed to maintain remission in seven patients. Respective 1-, 2-, and 3-year relapse-free survival rates after the first infusion were 80% (95% CI 72, 89), 63% (51, 77) and 52% (39, 70). Relapse-free survival was longer for patients receiving rituximab maintenance therapy (P = 0.002). Among 22 (28%) rituximab-treated patients experiencing severe adverse events, 12 (15%) had infectious complications leading to 4 (5%) deaths. Only 15 (19%) patients had received anti-pneumococcal vaccine before rituximab. CONCLUSION: Rituximab was able to induce AAV remission and seemed to maintain remission better than other agents, but caution is needed concerning its safety, especially regarding bacterial infections, in this population.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Anticorpos Monoclonais Murinos/uso terapêutico , Antirreumáticos/uso terapêutico , Gerenciamento Clínico , Adulto , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/mortalidade , Anticorpos Monoclonais Murinos/efeitos adversos , Antirreumáticos/efeitos adversos , Infecções Bacterianas/epidemiologia , Feminino , França/epidemiologia , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Indução de Remissão , Estudos Retrospectivos , Fatores de Risco , Rituximab , Taxa de Sobrevida , Resultado do Tratamento
16.
Joint Bone Spine ; 79(4): 409-11, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-22405857

RESUMO

A patient was diagnosed with discitis and sacroiliitis due to Mycobacterium xenopi. He had a history of percutaneous nucleotomy performed 15 years earlier (in 1992) at the Clinique du Sport, Paris, France, during an outbreak of nosocomial M. xenopi infection at that institution. In 1997, magnetic resonance imaging performed as part of the routine follow-up program for patients who had surgery at the Clinique du Sport during the outbreak was not interpreted as indicating discitis; this assessment was confirmed by our review of the images. Bone and joint infections due to atypical mycobacteria are rare and can develop very slowly. To our knowledge, this is the first reported case of M. xenopi discitis with secondary extension to the sacroiliac joint in an immunocompetent patient.


Assuntos
Infecção Hospitalar/diagnóstico , Discite/diagnóstico , Infecções por Mycobacterium não Tuberculosas/diagnóstico , Mycobacterium xenopi , Sacroileíte/diagnóstico , Infecção da Ferida Cirúrgica/microbiologia , Antibióticos Antituberculose/uso terapêutico , Antituberculosos/uso terapêutico , Claritromicina/uso terapêutico , Infecção Hospitalar/tratamento farmacológico , Infecção Hospitalar/microbiologia , Discite/tratamento farmacológico , Discite/microbiologia , Surtos de Doenças , Discotomia Percutânea/efeitos adversos , Quimioterapia Combinada , Etambutol/uso terapêutico , Humanos , Imunocompetência , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Infecções por Mycobacterium não Tuberculosas/tratamento farmacológico , Infecções por Mycobacterium não Tuberculosas/microbiologia , Rifampina/uso terapêutico , Sacroileíte/tratamento farmacológico , Sacroileíte/microbiologia , Resultado do Tratamento
18.
Joint Bone Spine ; 78(1): 75-9, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20800526

RESUMO

OBJECTIVE: Osteomyelitis is rare in adults and typically occurs in patients with risk factors such as sickle cell disease or immune deficiency. Cases in immunocompetent adults without sickle cell disease are extremely rare. The objective of this work was to describe the epidemiological, clinical, laboratory, and radiological features and the management of long-bone osteomyelitis in immunocompetent adults without sickle cell disease. METHODS: We conducted a retrospective descriptive study of all immunocompetent adults without sickle cell disease who were admitted to our center between November 2002 and November 2008 for long-bone osteomyelitis. In all patients, the clinical symptoms started in adulthood, in the absence of a childhood history of osteomyelitis. RESULTS: We identified six patients meeting our inclusion criteria over the 6-year study period. The causative microorganism was methicillin-susceptible Staphylococcus aureus in four patients and Salmonella in two patients (wild-type S. typhi and S. enterica, respectively). In each patient, there was a single focus of osteomyelitis and a single causative microorganism. The symptoms developed insidiously and lacked specificity. At presentation, the patients had moderate pain with or without a swelling. There was no fever initially in five patients, three of whom had major diagnostic delays as a result. Treatment associated antibiotics and surgery in all patients and the initial outcome was consistently favorable (median follow-up: 15 months; range: 8-72). CONCLUSION: Osteomyelitis can occur even in immunocompetent adults. The protracted course and atypical presentation of osteomyelitis in immunocompetent adults may lead to major diagnostic delays.


Assuntos
Imunocompetência , Osteomielite , Infecções por Salmonella , Salmonella typhi , Infecções Estafilocócicas , Feminino , Humanos , Incidência , Masculino , Osteomielite/diagnóstico , Osteomielite/epidemiologia , Osteomielite/imunologia , Estudos Retrospectivos , Infecções por Salmonella/diagnóstico , Infecções por Salmonella/epidemiologia , Infecções por Salmonella/imunologia , Salmonella enterica , Infecções Estafilocócicas/diagnóstico , Infecções Estafilocócicas/epidemiologia , Infecções Estafilocócicas/imunologia , Adulto Jovem
19.
Joint Bone Spine ; 76(5): 491-6, 2009 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-19525137

RESUMO

BACKGROUND: Outcome of streptococcal prosthetic hip infection is often thought to be better than that caused by other pathogens. That supposition was not confirmed in our experience with group B streptococcal prosthetic joint infection. OBJECTIVE: We compared outcomes of group B streptococcal and other-pathogen prosthetic hip infections. METHODS: One hundred and thirty nine patients, 24 with group B streptococcal and 115 other-pathogen prosthetic hip infections, were included. The primary outcome was the time from surgical treatment to treatment failure, defined as relapse, infection- or treatment-related death. Secondary outcomes were the times from surgical treatment to relapse or any event (event-free survival). The cumulative incidence estimator was used to model primary and secondary outcomes. Multivariable regression analysis was used to determine a set of independent predictors of treatment failure. RESULTS: With a median follow-up of 22 months, treatment failed more frequently in patients with group B streptococcal prosthetic hip infections (hazard ratio, 4.88 [95% CI, 1.4-17], P=.012). Multivariable analysis retained the American Society of Anesthesiologist score and group B streptococcal infection as independent risk factors of treatment failure; event-free survival was lower for these patients (hazard ratio, 2.64 [95% CI, 1.2-6], P=.02). CONCLUSION: Despite high antibiotic susceptibility, outcomes of group B streptococcal and other-pathogen prosthetic hip infection differ.


Assuntos
Antibacterianos/uso terapêutico , Infecções Bacterianas/epidemiologia , Prótese de Quadril/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Infecções Estreptocócicas/epidemiologia , Streptococcus agalactiae , Idoso , Infecções Bacterianas/tratamento farmacológico , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Complicações Pós-Operatórias/tratamento farmacológico , Complicações Pós-Operatórias/microbiologia , Modelos de Riscos Proporcionais , Falha de Prótese , Análise de Regressão , Reoperação/estatística & dados numéricos , Infecções Estreptocócicas/tratamento farmacológico , Fatores de Tempo , Falha de Tratamento , Resultado do Tratamento
20.
Presse Med ; 38(11): 1577-84, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19525087

RESUMO

OBJECTIVE: To describe the epidemiological, clinical, and laboratory characteristics of patients with group B streptococcal (GBS) prosthetic joint infections, their diagnoses, treatment, and long-term outcomes. METHODS: We conducted a retrospective cohort study including all patients hospitalized from January 1994 through May 2006 for a GBS prosthetic joint infection. RESULTS: The study included 30 patients, aged 35-87 (median 74) years with prosthetic hip (24) or knee (6) infections, 20 with at least one underlying disease. The route of infection was presumed to be hematogenous in 27 patients, and a portal of entry was identified in 9 (genitourinary tract 4, skin 2, gastrointestinal tract 2, oropharynx 1). All patients underwent surgery (6 debridement-synovectomy, 9 1-stage exchange arthroplasty, 8 2-stage exchange arthroplasty, 6 hip resection arthroplasty, and 1 knee arthrodesis) and received prolonged intravenous antibiotics. Four patients relapsed. One patient developed 2 other infections on her knee prosthesis. Two deaths were infection-related, and one was treatment-related. Nineteen patients followed for >/=2 years were cured. One patient was lost to follow-up and 3 died of causes unrelated to infection or treatment within 2 years. CONCLUSION: GBS prosthetic joint infections are mostly acute hematogenous infections that require prompt management for satisfactory outcome. Despite high antibiotic susceptibility, treatment failure is frequent because of the severity of the infection and patients' advanced age, underlying diseases, and relapses.


Assuntos
Prótese Articular/efeitos adversos , Infecções Relacionadas à Prótese/etiologia , Infecções Estreptocócicas/etiologia , Streptococcus agalactiae , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infecções Relacionadas à Prótese/diagnóstico , Infecções Relacionadas à Prótese/terapia , Estudos Retrospectivos , Infecções Estreptocócicas/diagnóstico , Infecções Estreptocócicas/terapia
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