2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc. METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed. RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud's phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found. CONCLUSION: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.

Work disability in fibromyalgia and other soft tissue disorders: analysis of preventive benefits in Brazil from 2006 to 2015.

BACKGROUND: Fibromyalgia is a common chronic disease characterized by persistent diffuse pain, fatigue, sleep disorders and functional symptoms. The disease can have negative consequences in personal and social life, in addition to significant public health expenses caused by treatment and work leave. The purpose of this article is to evaluate the number of social security benefits granted due to incapacity for work in Brazil in patients with ICD M79 and variants in the period 2006-2015. There has been no previous study with data referring to work withdrawals caused by fibromyalgia in Brazil. METHODS: Data for this study were obtained through an official Social Security platform. The disability and retirement benefits were analyzed. RESULTS: A total of 95,882 social security disability benefits were granted to ICD M79 and variants in the period from 2006 to 2015. Regarding gender, 69,420 benefits (72.3%) were granted to women and 26,562 (27.7%) to men. Regarding the types of benefits, we found 93,556 (97.5%) temporary withdrawals from work and 2426 (2.5%) permanent withdrawals. When comparing the initial and final years, we observed a significant reduction in the number of awards: 15,562 in 2006 to 6163 in 2015. CONCLUSION: Fibromyalgia was an important cause of withdrawal due to incapacity for work in Brazil, with consequent public health expenditure. These data may serve as a basis for new studies and can alert professionals of the need for adequate management of fibromyalgia to reduce work withdrawal and its consequences.

Capilaroscopia periungueal: relevância para a prática reumatológica / Nailfold capillaroscopy: relevance to the practice of rheumatology

A capilaroscopia periungueal é um método simples, de baixo custo, e de extrema relevância na avaliação de pacientes com fenômeno de Raynaud ou portadores de doenças do espectro da esclerose sistêmica (ES). Além de sua importância para o diagnóstico precoce da ES, constitui instrumento útil na identificação de pacientes esclerodérmicos com risco elevado para o desenvolvimento de complicações vasculares, viscerais e de óbito. A inclusão da capilaroscopia nos novos critérios para classificação da ES do Colégio Americano de Reumatologia (ACR) e da Liga Europeia Contra o Reumatismo (Eular) dá novo impulso para a utilização e disseminação do método. No presente artigo, pretendemos apresentar uma revisão didática, não sistemática, sobre o tema, com ênfase nos avanços recentemente descritos.

Nailfold capillaroscopy is a simple, low-cost method, that is extremely important in the evaluation of patients with Raynaud's phenomenon and of patients with systemic sclerosis (SSc) spectrum diseases. Besides its importance for the early diagnosis of SSc, nailfold capillaroscopy is a useful tool to identify scleroderma patients with high risk for development of vascular and visceral complications and death. The inclusion of capillaroscopy in the new classification criteria for SSc of the American College of Rheumatology (ACR) and European League Against Rheumatism (Eular) gives a new impetus to the use and dissemination of the method. In this paper, we present a didactic, non-systematic review on the subject, with emphasis on advances recently described.

[Nailfold capillaroscopy: relevance to the practice of rheumatology]. / Capilaroscopia periungueal: relevância para a prática reumatológica.

Nailfold capillaroscopy is a simple, low-cost method, that is extremely important in the evaluation of patients with Raynaud's phenomenon and of patients with systemic sclerosis (SSc) spectrum diseases. Besides its importance for the early diagnosis of SSc, nailfold capillaroscopy is a useful tool to identify scleroderma patients with high risk for development of vascular and visceral complications and death. The inclusion of capillaroscopy in the new classification criteria for SSc of the American College of Rheumatology (ACR) and European League Against Rheumatism (Eular) gives a new impetus to the use and dissemination of the method. In this paper, we present a didactic, non-systematic review on the subject, with emphasis on advances recently described.

Síndrome de Felty e Calazar: um desafio para o reumatologista / Felty's syndrome and Kala-azar: a challenge for the rheumatologist

Os autores relatam o caso de uma paciente com artrite reumatoide que evoluiu com grave neutropenia e esplenomegalia, sendo firmado o diagnóstico de Síndrome de Felty, que posteriormente desenvolveu Calazar. Ambas têm apresentação clínica e laboratorial semelhantes, tornando o diagnóstico diferencial difícil. O relato deste caso objetiva chamar a atenção para o reconhecimento da infecção por leishmaniose visceral em pacientes portadores de doenças reumáticas, assim como a possibilidade de um paciente com Calazar mimetizar um quadro de doença reumática sistêmica.

Case report of a patient with rheumatoid arthritis who developed severe neutropenia, splenomegaly and was diagnosed with Felty's syndrome. The patient later developed Kala-azar. Both diseases have similar clinical and laboratory presentation, making the differential diagnosis difficult. The present case report aims at drawing attention to the identification of visceral Leishmaniasis infection in patients with rheumatic diseases, as well as possibility of a patient with Kala-azar mimicking a set of symptoms of systemic rheumatic disease.

Felty's syndrome and Kala-azar: a challenge for the rheumatologist.

Case report of a patient with rheumatoid arthritis who developed severe neutropenia, splenomegaly and was diagnosed with Felty's syndrome. The patient later developed Kala-azar. Both diseases have similar clinical and laboratory presentation, making the differential diagnosis difficult. The present case report aims at drawing attention to the identification of visceral Leishmaniasis infection in patients with rheumatic diseases, as well as possibility of a patient with Kala-azar mimicking a set of symptoms of systemic rheumatic disease.

Sensorineural dysacusis in patients with systemic lupus erythematosus.

The objective of this study was to establish the frequency of involvement of the auditory apparatus in 45 female patients with systemic lupus erythematosus (SLE) submitted to general clinical and laboratory assessments, and tone and vocal audiometry accompanied by a questionnaire evaluating auditory symptoms. The control group consisted of 45 healthy women, matched by age. Auditory symptoms were present in 25 (55.5%) patients, with a diagnosis of sensorineural hearing loss in seven (15.6%) patients. A significant correlation with hypoacusis (P < 0.001), ear fullness (P = 0.012) and tinnitus (P = 0.017) was observed in patients with hearing loss. None of the clinical or laboratory parameters showed an association with sensorineural hearing loss. In the control group, three women (6.7%) presented audiometric alterations, including two with altered tympanometry results and one with mild sensorineural hearing loss. In conclusion, an adequate investigation of auditory symptoms is important during the follow-up of patients with SLE, since manifestations of the auditory apparatus and sensorineural hearing loss can affect a significant proportion of patients.

Avaliação da produção de espécies reativas de oxigênio por granulócitos de sangue periférico de pacientes com fibromialgia primária / Assessment of reactive oxygen species production by peripheral blood granulocytes from primary fibromyalgia patients

Obejetivo: avaliar anormalidades bioquímicas que possam estar relcionadas à fibromialgia, mais especificamente o metabolismo oxidante de granulócitos de pacientes com fibromialgia primária (FMP). Métodos: foram selecionados 10 pacientes (9 mulheres e 1 homem) portadores de FMP, com idade entre 20 e 40 anos, de acordo com os critérios do Colégio Americano de Reumatologia, que não apresentavam qualquer outra doença de curso agudo ou crônico e estavam sem utilizar medicamentos no mínimo durante duas semanas. Controles normais (n = 19) pareados por sexo e idade foram selecionados. Os granulócitos de pacientes e controles foram separados do sangue periférico e utilizados como célula de trabalho. A produção de espécies reativas de oxigênio (ERO) foi determinada por meio de ensaio de quimioluminescência dependente de luminol. Resultados: demonstramos, pela primeira vez, uma produção significantemente elevada de ERO (3,2 vezes maior) em granulócitos de sangue periférico de pacientes com FMP (< 0,05). Conclusões: estes resultados demonstraram um comportamento anormal dos granulócitos de pacientes com FMP, em que uma produção aumentada de espécies reativas do oxigênio foi observada. Estudos futuros serão necessários para melhor esclarecer estes resultados, delineando uma nova perspectiva na compreensão e abordagem destes pacientes

Sildenafil no tratamento da hipertensão pulmonar associada a lúpus eritematoso sistêmico e síndrome antifosfolipídio / Sildenafil for treatment of pulmonary hypertension in association with systemic lupus erythematosus and anti-phospholipid syndrome

Hipertensão pulmonar grave é uma doença debilitante, com expectativa de vida reduzida, que acomete adultos jovens. Complicações pleuropulmonares no lúpus eritematoso sistêmico ocorrem em 50 por cento a 70 por cento dos pacientes. A hipertensão pulmonar grave no lúpus eritematoso sistêmico é rara e tem prognóstico reservado. Descreve-se, pela primeira vez, um paciente com hipertensão pulmonar grave associada a lúpus eritematoso sistêmico e síndrome antifosfolipídio secundário que apresentou boa resposta ao uso do sildenafil oral, após falha do tratamento convencional com corticosteróides, ciclofosfamida, warfarin e diltiazem.

[The implantation of VDD pacemakers with a single electrode--a comparative study. The experience of the last 5 years]. / Implantação de pacemakers VDD com um único eléctrodo--estudo comparativo. Experiência dos últimos cinco anos.

OBJECTIVE: To compare the epidemiological characteristics and immediate results of all first single lead VDD pacemaker (PM) implantations with those of an equal number of dual chamber DDD PM, implanted during a 5-year period in a tertiary-care hospital. POPULATION AND METHODS: A total of 41 patients (pts) (25 males, mean age of 69.0 +/- 11.8 years) underwent a VDD PM implantation, from 30-11-92 to 15-9-97. This group was compared with an equal number of patients (28 males, mean age of 69.9 +/- 7.31 years) with a DDD PM implanted in the same period, selected by a criterion of immediate temporal proximity of procedure. For each patient we collected the clinical and electrocardiographic (ECG) indications for PM implantation, parameters of atrial (AS) and ventricular (VS) sensing and ventricular pacing (VP), X-ray exposure time (XRT) and complications. RESULTS: In the VDD group, 46.3% of the patients had syncope, 51.2% had complete AV block on the ECG, and 14.6% were PM-dependent. Analyzed procedure-related parameters were as follows: P-wave amplitude: 2.1 +/- 0.6 V; AS threshold: 1.2 +/- 0.7 V; R-wave amplitude: 9.1 +/- 3.3 V; VS threshold: 7.0 +/- 2.0 V; VP thresholds: 0.68 +/- 0.24 mA, 0.43 +/- 0.12 V (for a spike duration of 0.5 ms); ventricular impedance: 644.9 +/- 132.0 ohm; XRT; 7' 43" +/- 8' 23". There were two minor complications, for an incidence of 4.9% (one local hematoma and a vagal reaction). In the DDD group the clinical and ECG characteristics were similar, but there was a 22.0% prevalence of sinus-node dysfunction, VS 0% in the VDD group). The P-wave amplitude and AS threshold were significantly (p < 0.005) better (2.8 +/- 0.9 V and 2.8 +/- 0.9 V respectively). The other parameters were similar to those of the VDD group. CONCLUSIONS: The immediate results of VDD PM implantation are good and comparable with those of DDD PM, although with worse acute AS parameters.

Polineuropatia periférica no lúpus eritematoso sistêmico: terapêutica com "pulses" de metilprednisolona e ciclofosfamida / Peripheral polyneuropathy in systemic lupus erythematosus: methylprednisolone and cyclophosphamide pulse therapy

Os autores relatam um caso de polineuropatia sensitivomotora em uma paciente de 17 anos com lúpus eritematoso sistêmico (LES) e altos títulos de anticorpos anticardiolipina IgG. A terapêutica com metilprednisolona e ciclofosfamida resultou em significativa melhora clínica e reduçäo dos títulos de anticorpos

[Permanent pacing in childhood: a review of 11 years of experience]. / Pacing permanente em idades pediátricas: revisão de uma experiência de 11 anos.

STUDY OBJECTIVE: To review our eleven year experience in the implantation and follow-up of permanent Pacemakers in the paediatric age group. DESIGN: Retrospective study. SETTING: Children submitted to permanent cardiac Pacing implantation and accompanied in the Pacing Center of the Hospital de Santa Maria. PATIENTS: Children from both sexes, aged from neonate to 14 years old, with brady-dysrhythmia and indication for permanent cardiac Pacing implantation. MATERIAL AND METHODS: From November 1980 to September 1991, 16 children had permanent Pacemaker implantation. We describe the clinical and electrocardiographic characteristics of the population, mode of Pacing used, technical data from the implantation and evolution. RESULTS: One children died due to associated cardiac defect not related to the Pacemaker. The other 15 children remain in follow-up with normofunctioning Pacemakers and free of symptoms. We had to perform 11 reinterventions in 8 children due to generator or electrode problems (28,2 months reintervention interval). CONCLUSIONS: Improvements in Pacemaker technology and a careful technique of implantation can significantly reduce the morbidity associated to permanent pacemaker implantation in this age group.

[The use of Holter electrocardiography in permanent pacemaker users]. / Emprego da electrocardiografia de Holter em portadores de pacemaker permanente.

The authors selected 37 permanent pacemaker patients followed for eleven successive months, with symptomatology eventually related to the pacing system. An Holter ambulatory monitorization was performed to all of them during 24 hours. Twenty one had single chamber systems and the others double chamber. Twelve malfunctions were found in ten patients. Six due to "undersensing", 4 related to "oversensing", and 2 from loss of capture. Two patients had tachycardia pacemaker mediated. An inadequate mode of pacing was found in other two cases. Fifteen rhythm disturbances not related to the pacemaker systems were registered in thirteen patients: supra-ventricular in 9; ventricular greater than or equal to 3 degree of lown in 5; and 2nd degree A.V.B. Mobitz type I in one case. Four patients were submitted to surgical intervention in order to modify the mode of pacing. It was also necessary to reprogram ten patients. The Holter Ambulatory Electrocardiography revealed to be an important diagnostic method for the detection of intermittent malfunction of pacing systems. The occasional changes of cardiac rhythm not linked with pacing were also revealed.