Atención de la úlcera de pie como manifestación clínica de la esclerosis sistémica / Managing foot ulcers as a clinical manifestation of systemic sclerosis

Las úlceras pueden aparecer sobre protuberancias óseas, como las falanges proximales o los codos, pero es más probable que sean secundarias a la piel tensa y al trauma y no respondan a la terapia vasodilatadora. Todas las úlceras son muy dolorosas. El propósito de este trabajo es presentar aspectos relacionados con la atención de las úlceras digitales en la esclerodermia, según la experiencia de diferentes autores. En reumatología, se recomienda, en todo paciente con dolor reumático crónico, calcular la intensidad del dolor, tanto para la elección primera del tratamiento analgésico como para la medición de la respuesta. Y, para ello, se han propuesto varios métodos de cuantificación. El manejo de las úlceras digitales en la esclerodermia incluye la intervención no farmacológica, farmacológica y quirúrgica. El uso de nitratos, infusiones de acetilcisteína, el gel de vitamina E, entre otros métodos mejoran el tiempo de curación en pacientes con esclerodermia. La falta de terapias aprobadas por la Administración de Alimentos y Medicamentos de los Estados Unidos para las ulceraciones digitales garantiza un enfoque y una atención renovados para encontrar opciones de tratamiento significativas. Los médicos deberán emplear un enfoque terapéutico multifacético para optimizar el tratamiento de úlceras digitales existente y evitar la formación de nuevas lesiones para proporcionar a los pacientes la mejor calidad de vida(AU)

Ulcers can occur on bony bumps, such as the proximal phalanges or elbows, but are more likely to be secondary to tight skin and trauma and not responsive to vasodilator therapy. All ulcers are characteristically very painful. To present aspects related to the management of digital ulcers in scleroderma based on the experience of different authors. The management of digital ulcers in scleroderma includes non-pharmacological, pharmacological and surgical intervention. The use of nitrates, infusions of acetylcysteine, Vitamin E Gel, among other methods, improve the healing time in patients with scleroderma. The lack of US Food and Drug Administration approved therapies for digital ulcerations ensures a renewed focus and attention on finding meaningful treatment options. Clinicians should employ a multi-faceted therapeutic approach to optimize existing digital ulcers treatment and avoid new lesion formation to provide patients with the best quality of life(AU)

Endothelial lesion and complement activation in patients with Scleroderma Renal Crisis / Lesão endotelial e ativação do complemento em pacientes com crise renal esclerodérmica

Abstract In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation.

Resumo Nas revisões de biópsias renais, a crise renal esclerodérmica (CRE) é caracterizada por lesões endoteliais vasculares, depósitos de C4d em vasos peritubulares e lesões agudas e crônicas que coexistem na mesma biópsia. Os sinais clínicos de microangiopatia trombótica (MAT) são descritos na esclerose sistêmica (ES); no entanto, não foram relacionados às lesões agudas descritas nas biópsias renais. Relatamos um caso de CRE em um paciente com síndrome de superposição de esclerodermia-dermatomiosite, que também apresentou dados clínicos e histopatológicos de MAT. No exame de fundo do olho, foi encontrada uma retinopatia hipertensiva aguda grave. A biópsia renal mostrou lesão endotelial grave com alargamento das células mucoides ao nível da íntima, proliferação concêntrica focal na maioria das pequenas arteríolas e depósitos de C3, C4d e IgM ao longo das paredes dos capilares. O estudo genético do complemento mostrou apenas a presença de haplótipos de risco da proteína cofator de membrana (PCM), sem outros distúrbios genéticos do complemento. Entendemos que em um paciente com MAT e ES, o dano renal seria fundamentalmente endotelial e do tipo agudo; além disso, observaríamos evidências claras de ativação do complemento. Uma vez que novos estudos correlacionam dados clínico-analíticos com estudos anatomopatológicos, é provável que sejamos forçados a redefinir o conceito de CRE, enfocando a relação entre dano endotelial agudo e ativação do complemento.

Ventilation distribution as a contributor to the functional exercise capacity in patients with systemic sclerosis-associated interstitial lung disease without pulmonary hypertension

Phenotypic differences have been described between patients with systemic sclerosis (SSc)-associated interstitial lung disease (ILD) and SSc-associated pulmonary hypertension, including performance differences in the 6-min walk test (6MWT). Moreover, the correlations between the 6MWT and traditional pulmonary function tests (PFTs) are weak, indicating the need to search for new parameters that explain exercise performance. Thus, our objective was to evaluate the impact of ventilation distribution heterogeneity assessed by the nitrogen single-breath washout (N2SBW) test and peripheral muscle dysfunction on the exercise capacity in patients with SSc-ILD and limited involvement of the pulmonary parenchyma. In this cross-sectional study, 20 women with SSc-ILD and 20 matched controls underwent PFTs (including spirometry, diffusing capacity for carbon monoxide (DLco), and the N2SBW test) and performed the 6MWT and knee isometric dynamometry. The 6-min walking distance (6MWD, % predicted) was strongly correlated with the phase III slope of the single-breath nitrogen washout (phase III slopeN2SBW) (r=−0.753, P<0.0001) and reasonably correlated with the forced vital capacity (FVC) (r=0.466, P=0.008) and DLco (r=0.398, P=0.011). The peripheral oxygen saturation (SpO2) during exercise was not significantly correlated with any of the pulmonary or muscle function parameters. The phase III slopeN2SBW was the only predictive variable for the 6MWD, whereas quadriceps strength and FVC/DLco were predictive variables for SpO2. Ventilation distribution heterogeneity is one factor that contributes to a lower 6MWD in SSc-ILD patients. In addition, muscle dysfunction and abnormal lung diffusion at least partly explain the decreased SpO2 of these patients.

Fenómeno de Raynaud con lesiones isquémicas digitales: tratamiento con bloqueo de ganglio estrellado: presentación de un caso / Digital ischemic lesions associated to Raynaud's phenomenon treated with stellate ganglion block: case report

El fenómeno de Raynaud consiste en la aparición paroxística de espasmo vascular, que afecta a pequeñas arterias y arteriolas cutáneas, de las extremidades. Se manifiesta con palidez extrema, que puede ir seguida de cianosis y posterior rubicundez durante la fase hiperémica. Se acompaña generalmente de disestesias y, en forma ocasional, también de dolor. Generalmente, presentan escasa repercusión sistémica asociada. Los principales desencadenantes son el frío y el estrés emocional. El bloqueo del ganglio estrellado es utilizado frecuentemente para el diagnóstico y tratamiento de múltiples sindromes dolorosos crónicos. Con el bloqueo de la actividad simpática, se produce una vasodilatación de los vasos de la extremidad superior, cabeza y cuello, siendo éste el sustrato para su utilización en cuadros de insuficiencias vasculares de las zonas inervadas. Presentamos el caso de un bloqueo de ganglio estrellado en una paciente con lesiones isquémicas digitales secundarias a un fenómeno de Raynaud primario.

The sudden onset of vascular spasm, affecting small arteries and skin arterioles of limbs is known as Raynaud's phenomenon. it includes severe paleness sometimes followed by cyanosis and redness during the hyperemic phase. Usual findings are dysestesias and ocassionally pain. Normally it causes a small systemic impact. The most relevant triggering factors are cold and emotional stress. Stellate Ganglion Block is frequently used to diagnose and treat several chronic pain syndromes. The sympathetic activity block causes the vasodilation of the arms, head and neck, and this is the substratum for using it in vascular impairment syndrome. We present a stellate ganglion block in a patient with isquemic digital lesions secondary to Raynaud phenomenon.

Clinical manifestations and vascular events in patients with lupus erythematosus anticardiolipin antibodies and raynaud's phenomenon

BACKGROUND: Raynaud's phenomenon (RP) and anticardiolipin antibodies (ACL) are two common clinical manifestations in patients with systemic lupus erythematosus (SLE). RP may lead to digital or limb loss. ACL are associated to thrombotic episodes. It is not yet clear if there is an association between RP and the presence of ACL in patients with SLE. OBJECTIVES: To study if the presence of both RP and ACL in patients with SLE may be associated with certain clinical manifestations or thrombotic events compared to SLE patients without RP or ACL. METHODS: SLE patients from two lupus clinics were recruited. The patients were divided into 4 groups. Patients with RP and positive ACL (RP+ ACL+), patients with RP but negative ACL (RP+ ACL-), patients with negative RP and positive ACL (RP- ACL+), and patients that were negative for RP and ACL (RP- ACL-) used as the control group. Demographic data, diagnostic criteria, clinical manifestations, history of arterial thrombosis, venous thrombosis and abortions were recorded. A physical examination was done. Anticardiolipin antibodies IgG and IgM were done in the rheumatology laboratory at the University of Puerto Rico School of Medicine. Descriptive statistics as well as analysis of variances (ANOVA), and polytomous logistic regression were used. RESULTS: 236 patients with SLE were studied. There was a tendency toward an increase in arterial thrombosis (p-value = 0.094) and venous thrombosis (p-value = 0.067) in the group that were positive for RP and ACL (RP+ ACL+). Although it was not statistical significant, when polytomous logistic regression was used, both arterial and venous thrombosis had an increase in relative risk 3.21 for arterial and 3.11 for venous thrombosis. Abortions were not increased in any of the four groups. Clinical manifestations from SLE did not differ among the four groups. CONCLUSIONS: Patients with both RP and ACL seem to be at an increase risk for both arterial and venous thrombotic events; these patients may benefit from an antiplatelet medication to prevent these events to occur.

Esclerodermia mortal / Fatal scleroderma

Se presentan dos casos de esclerodermia con restricción pulmonar grave: un paciente masculino de 23 años con fiebre de 20 días de evolución asociada a disnea de medianos esfuerzos y enfermedad de Raynaud; y una paciente de 31 años con disnea de medianos esfuerzos y enfermedad de Raynaud. Ambos fallecieron por insuficiencia respiratoria aguda. La trascendencia de mostrar estos dos casos radica en la súbita presentación de la insuficiencia respiratoria en esclerodermia de reciente inicio

Manifestaçöes reumatológicas na acromegalia / Rheumatologic manifestations in acromegaly

Os autores relatam 13 casos de acromegalia, todos apresentando aumento das extremidades e alteraçöes nos níveis séricos do hormônio de crescimento, atendidos no Hospital de Clínicas-UFPR entre 1979 e 1986, sendo sete mulheres e seis homens, idade entre 16 e 68, tempo de doença variando de seis meses até 20 anos. Chamam a atençäo para as manifestaçöes osteoarticulares encontradas em 77% dos casos, sendo as mais freqüentes a dor articular aos movimentos (30,7%), parestesias de MMSS (23%) e de MMII (15,3%)

Esclerose sistêmica progressiva sem envolvimento cutâneo / Progressive systemic sclerosis without cutaneous involvement

Relatamos caso de paciente do sexo feminino, de 25 anos, com artralgias, fenômeno de Raynaud, hipertensäo pulmonar, sem espessamento de pele nem esclerodactília. O diagnóstico de esclerose sistêmica progressiva foi confirmado por achados laboratoriais: fator anti-núcleo padräo e a presença de anticorpo anti Scl 70