Systemic lupus erythematosus and Raynaud's phenomenon

Abstract: BACKGROUND: Patients with systemic lupus erythematosus seem to belong to different serological and clinical subgroups of the disease. Genetic background can cause the appearance of these subgroups. OBJECTIVE: To determine whether Brazilian patients who have systemic lupus erythematosus and Raynaud's phenomenon differ from those who do not. METHODS: Retrospective analysis of 373 medical records of systemic lupus erythematosus patients studied for demographic, clinical and serological data. A comparative analysis was performed of individuals with and without RP. RESULTS: There was a positive association between Raynaud's phenomenon and age at diagnosis (p=0.02), presence of anti-Sm (p=0.01) antibodies and anti-RNP (p<0.0001). Furthermore, a negative association was found between Raynaud's phenomenon and hemolysis (p=0.01), serositis (p=0.01), glomerulonephritis (p=0.0004) and IgM aCL (p=0.004) antibodies. CONCLUSION: Raynaud's phenomenon patients appear to belong to a systemic lupus erythematosus subset with a spectrum of clinical manifestations located in a more benign pole of the disease.

La capilaroscopia en reumatología: conceptos básicos de una valiosa técnica de imágenes / The capillaroscopy in rheumatology: basics of a valuable imaging technique

The capillaroscopy is an easy and non invasive tool that allows an accurate study of the microcirculation. It has gained over the years a space in the rheumatology community, being currently a fundamental method for the diagnosis and management of connective tissue disease (CTD), in particular systemic sclerosis. Currently it is possible to demonstrate a correlation between the microvascular alterations and the diagnosis and prognosis of the ETC. In this “point of view” work we provide a practical description of the utility of capillaroscopy in the ETC describing also the characteristic pathologic findings and mentioning technical details for a correct execution of the examination...

La capilaroscopia es una técnica sencilla y no invasiva que permite un correcto y minucioso estudio de los vasos de la microcirculación. Ésta ha ido ganando a lo largo de los últimos años un espacio en la comunidad reumatológica hasta el punto de ser, actualmente, considerada un método fundamental para el diagnóstico y manejo de las enfermedades del tejido conectivo (ETC), en particular la esclerosis sistémica. Actualmente es posible establecer y demostrar una correlación entre las alteraciones capilaroscópicas y el diagnóstico y pronóstico de las ETC. En este trabajo de “punto de vista” se provee una descripción práctica de la utilidad de la capilaroscopia en las ETC, describiendo los hallazgos patológicos característicos y mencionando detalles técnicos para una correcta ejecución del examen...

N-acetilcisteína oral no tratamento do fenômeno de Raynaud secundário à esclerose sistêmica: ensaio clínico randomizado, placebo-controlado e duplo-cego / Oral N-acetylcysteine in the treatment of Raynaud's phenomenon secondary to systemic sclerosis: A randomized, double-blind, placebo-controlled clinical trial

Objetivo Avaliar a segurança e a eficácia da N-acetilcisteína (NAC) por via oral sobre o fluxo sanguíneo da microcirculação digital em pacientes com fenômeno de Raynaud (FRy) secundário à esclerose sistêmica (ES). Métodos Este foi um estudo randomizado, duplo-cego e placebo-controlado, no qual 42 pacientes com ES receberam NAC oral na dose de 600 mg, três vezes ao dia (21 pacientes, idade média 45,6±9,5 anos) ou placebo (21 pacientes, idade média 45,0±12,7 anos) durante quatro semanas. O desfecho primário do estudo foi: melhora no fluxo sanguíneo da microcirculação cutânea antes e após estímulo frio avaliado pelo laser Doppler imaging (LDI) nas semanas 0 e 4. A frequência e a gravidade do FRy e o número de úlceras digitais também foram avaliados nas semanas 0 e 4. Os efeitos adversos foram registrados na quarta semana. Resultados Não houve mudança significativa no fluxo sanguíneo digital avaliado pelo LDI antes ou depois do estímulo frio após quatro semanas de NAC ou placebo. Ambos os grupos apresentaram melhora significativa na frequência e gravidade dos ataques de FRy, sem diferença entre os dois. O grupo placebo apresentou três úlceras digitais enquanto o grupo NAC não apresentou úlceras ao final do estudo. NAC foi bem tolerada e nenhum paciente descontinuou o tratamento. Conclusões NAC por via oral na dose de 1.800mg/dia não demonstrou efeito vasodilatador sobre a microcirculação das mãos após quatro semanas de tratamento em pacientes com FRy secundário à ES. .

Objective To evaluate the safety and efficacy of oral N-acetylcysteine (NAC) on digital microcirculation blood flow in patients with Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). Methods This was a randomized, double-blind, placebo-controlled trial in which 42 patients with SSc received oral NAC at a dose of 600mg tid (21 patients, mean age 45.6±9.5 years) or placebo (21 patients, mean age 45.0±12.7 years) for four weeks. The primary endpoint was the change in cutaneous microcirculation blood flow before and after cold stimulation measured by laser Doppler imaging (LDI) at weeks 0 and 4. The frequency and severity of RP and the number of digital ulcers were also measured at weeks 0 and 4. The adverse events were recorded in the fourth week. Results There was no significant change in digital blood flow assessed by LDI before or after cold stimulus after four weeks of NAC or placebo. Both groups showed significant improvement in the frequency and severity of RP attacks, with no difference between the two groups. At the end of the study, the placebo group had three digital ulcers, while the NAC group showed no ulcers. NAC was well tolerated and no patient discontinued the treatment. Conclusions NAC orally at a dose of 1800mg/day showed no vasodilator effect on hands’ microcirculation after four weeks of treatment in patients with RP secondary to SSc. .

Esclerose sistêmica e o fenômeno de Raynaud: manejo terapêutico / Systemic sclerosis and Raynaud's phenomenon: therapeutic management

O fenômeno de Raynaud (FRy) caracteriza-se por episódios reversíveis de vasoespasmos de extremidades, que ocorrem usualmente após estresse ou exposição ao frio. O FRy pode ser primário ou secundário a uma série de condições, principalmente a doenças do espectro da esclerose sistêmica (ES). Na ES, o FRy costuma ser mais grave, e lesões isquêmicas de extremidades são frequentes. Nos últimos anos, avanços no estudo da fisiopatologia do FRy e da doença vascular na ES propiciaram o surgimento de novas opções terapêuticas para esta manifestação. Os bloqueadores de canal de cálcio devem ser utilizados como tratamento de primeira escolha para o FRy. Novas drogas, como os inibidores da fosfodiesterase V e os prostanoides, podem ser utilizados em pacientes com FRy grave, e a bosentana (antagonista do receptor da endotelina-1) é indicada para a prevenção de úlceras digitais recorrentes.

Raynaud's phenomenon (RP) is characterized by episodic vasospasm of the extremities, usually in response to stress or cold exposure. It can be primary or secondary to several conditions, especially systemic sclerosis-related diseases. In systemic sclerosis (SSc), RP is usually more severe and digital ischemic lesions are a frequent problem. In recent years, advances in the understanding of the pathophysiology of RP and of SSc vasculopathy led to the development of new therapeutic options for this condition. Calcium-channel blockers are the first choice for the treatment of RP. New drugs including phosphodiesterase type V inhibitors and prostanoids can be used for severe RP, and bosentan (endothelin-1 receptor antagonist) for prevention of recurrent digital ulcers.

Esclerosis sistémica / Systemic sclerosis

La esclerosis sistémica (ES) es una enfermedad del tejido conectivo de etiología desconocida que se caracteriza por cambios fibróticos de la piel, tejido subcutáneo y visceral, anormalidades de la microvasculatura y desórdenes inmunes.

Síndrome de Raynaud secundario: disminución del flujo sanguíneo de la piel entre los episodios isquémicos y prolongación de la vasoconstricción con las maniobras respiratorias / Secondary Raynaud's syndrome: skin blood flow decrease between the ischemic episodes and prolongation of the vasoconstriction during the respiratory maneuvers

El síndrome de Raynaud se caracteriza por isquemia cutánea digital episódica, manifestada por palidez, cianosis y rubor de los dedos de manos y pies expuestos al frío o cuando el paciente está sujeto a un estrés emocional. No se conoce el mecanismo fisiopatológico exacto; la hipótesis más invocada es una alteración autonómica en la inervación simpática de los vasos sanguíneos de la piel. Objetivo: Estudiar los cambios de la frecuencia cardíaca y el flujo sanguíneo de la piel (FSP) en sujetos sanos y en pacientes con síndrome de Raynaud durante las siguientes condiciones: 1. respiración espontánea (RE), 2. respiración rítmica (RR), 3. inspiración profunda repentina (IPR), 4. maniobra de Valsalva (MV). Método: Se estudiaron 22 sujetos sanos y 22 pacientes con síndrome de Raynaud secundario. Las variables medidas fueron: 1. intervalo R-R; 2. amplitud de FSP; 3. porcentaje de disminución de FSP; 4. latencia de la máxima disminución del FSP. Resultados: Los pacientes con síndrome de Raynaud presentaron mayor taquicardia basal. La amplitud del FSP se encontró disminuido durante la respiración espontánea y durante las maniobras respiratorias (P < 0.001). La vasoconstricción estuvo prolongada ya que la latencia promedio de recuperación del FSP estuvo prolongada en IPR y MV en comparación de los sujetos controles. Conclusión: La amplitud del FSP basal está disminuida en los pacientes con síndrome de Raynaud, durante períodos asintomáticos; esto sugiere daño endotelial. También se encontró disminuido con las diversas maniobras respiratorias (RR, IPR y MV). Este cambio dinámico sugiere hiperactividad simpática hacia los vasos sanguíneos de la piel.

The Raynaud's syndrome is an episodic skin ischemia manifested by pallor, cyanosis and erythema of the fingers in response to cold or emotional stress. The exact pathophysiology is unknown but it has been hypothetised that may be due to an autonomic alteration in the sympathetic innervation of skin blood vessels. Objective: To study the changes of heart rate and skin bloodflow (SBF) in healthy subjects and in patients with secondary Raynaud's syndrome during different respiratory maneuvers: 1. spontaneous respiration; 2. rhythmic respirations (RR), 3. sudden inspirations (SI), and 4. Valsalva maneuver (VM). Methods: We studied 22 healthy subjects and 22 patients with secondary Raynaud's syndrome. The variables measured were: 1) RR intervals; 2) amplitudes of SBF; 3) percentage of decrease of SBF; 4) latency of the maximum decrease of SBF. Results: In all patients with secondary Raynaud's syndrome the SBF was decreased basally during spontaneous respirations and during all respiratory maneuvers (p < 0.001). The mean latency of recovery of the SBF was prolonged during sudden deep inspiration. The patients with Raynaud also had significant basal tachycardia at rest (p < 0.003). Conclusions: The basal skin blood flow during spontaneous respirations and in asymptomatic periods is decreased in patients with Raynaud's syndrome; this may be related to endothelial arterioral damage. The SBF was also significantly decreased dynamically during sudde3n inspirations (SI), rhythmic breathing (RR) and Valsalva maneuver (VM). This dynamic change suggests sympathetic hyperactivity.

Raynaud primario y secundario a síndromes esclerodermiformes: comunicación de 97 pacientes estudiados por capilaroscopía periungueal / Primary Raynaud and secondary to sclerodermoform syndromes: a communication of 97 patients studies with periungueal capilaroscopy

La detección temprana de síndromes esclerodermoformes es de gran relevancia para el pronóstico del paciente. Nuestro objetivo es determinar la utilidad de la capilaroscopía periungueal en el diagnóstico diferencial entre Raynaud primario y secundario a síndromes esclerodermiformes. Se estudian pacientes con Raynaud primario (21), Raynaud secundario a síndromes esclerodermia sistémica (57) y Raynaud secundario a síndromes de superposición con esclerodermia (19). El grupo control de sujetos sin Raynaud estudiados fue de 40 personas. Todos los grupos fueron estudiados por capilaroscopía periungueal de dedos de ambas manos por medio de microscopio esteroscópico de 40 aumentos. La especificidad del método para detección de patrón SD (esclerodermiforme) fue de 100 por ciento y la sensibilidad asciende 82,5 por ciento para esclerodermia sistémica y 57 por ciento para síndromes de superposición