Position article and guidelines 2018 recommendations of the Brazilian Society of Rheumatology for the indication, interpretation and performance of nailfold capillaroscopy

Abstract Nailfold capillaroscopy (NFC) is a reproducible, simple, low-cost, and safe imaging technique used for morphological analysis of nail bed capillaries. It is considered to be extremely useful for the investigation of Raynaud's phenomenon and for the early diagnosis of systemic sclerosis (SSc). The capillaroscopic pattern typically associated with SSc, scleroderma ("SD") pattern, is characterized by dilated capillaries, microhemorrhages, avascular areas and/or capillary loss, and distortion of the capillary architecture. The aim of these recommendations is to provide orientation regarding the relevance of NFC, and to establish a consensus on the indications, nomenclature, the interpretation of NFC findings and the technical equipments that should be used. These recommendations were formulated based on a systematic literature review of studies included in the database MEDLINE (PubMed) without any time restriction.

Impact of Diltiazem Alone versus Diltiazem with Nitrate on Five-Year Clinical Outcomes in Patients with Significant Coronary Artery Spasm

PURPOSE: Calcium channel blockers diltiazem and nitrate have been used as selective coronary vasodilators for patients with significant coronary artery spasm (CAS). However, no study has compared the efficacy of diltiazem alone versus diltiazem with nitrate for long-term clinical outcomes in patients with CAS. MATERIALS AND METHODS: A total of 2741 consecutive patients without significant coronary artery disease with positive CAS by acetylcholine (Ach) provocation test between November 2004 and May 2014 were enrolled. Significant CAS was defined as a narrowing of >70% by incremental intracoronary injection of 20, 50, and 100 µg of Ach into the left coronary artery. Patients were assigned to either the diltiazem group (n=842) or the dual group (diltiazem with nitrate, n=1899) at physician discretion. To adjust for potential confounders, a propensity score matching (PSM) analysis was performed using the logistic regression model. After PSM analysis, two well-balanced groups (811 pairs, n=1622, C-statistic=0.708) were generated. RESULTS: At 5 years, there were similar incidences in primary endpoints, including mortality, myocardial infarction, revascularization, and recurrent angina requiring repeat coronary angiography between the two groups. Diltiazem alone was not an independent predictor for major adverse cardiovascular events or recurrent angina requiring repeat coronary angiography. CONCLUSION: Despite the expected improvement of endothelial function and the relief of CAS, the combination of diltiazem and nitrate treatment was not superior to diltiazem alone in reducing mortality and cardiovascular events up to 5 years in patients with significant CAS.

Suspected Microvascular Coronary Spasm Diagnosed by Spasm Provocation Test with High Dose of Acetylcholine.

A 71-year-old Japanese man was admitted to our hospital complaining of chest oppression on exercise, such as cycling uphill, which had continued for 9 years. We diagnosed him as having microvascular spasm according to the results of coronary angiography with an acetylcholine provocation test with a high dose (150g) of acetylcholine administrated at a time during 20 seconds. Electrocardiographic changes during the spasm provocation test resembled that during exercise tests performed upon admission and 9 years previously. He was treated with a calcium channel blocker and partial symptom relief was obtained.

Intraoperative "Kounis syndrome" that improved electrocardiography changes and hemodynamic situation afteradministering nitroglycerine / Síndrome de Kounis intraoperatória com melhoria das alterações eletrocardiográficas e da situação hemodinâmica após a administração de nitroglicerina / Síndrome de Kounis intraoperatorio con mejoría de las alteraciones electrocardiográficas y de la situación hemodinámica después de la administración de nitroglicerina

A 58-year-old female without cardiovascular risk factors, was going to be operated to repair the rotator cuff. Induction and interscalene brachial plexus block were uneventful, but after her placement for surgery the patient started with severe bronchospasm, hypotension, cutaneous allergic reaction and ST elevation on the electrocardiogram. An anaphylactic shock was suspected and treated but until the perfusion of nitroglycerina was started no electrocardiographic changes resolved. After necessary diagnostic test the final diagnosis was variant I of Kounis syndrome due to cefazolin and rocuronium. Ephinephrine is the cornerstone of treatment for anaphylaxis but should we use it if the anaphylactic reaction is also accompanied by myocardial ischemia? The answer is that we should not use it because myocardial ischemia in this syndrome is caused by vasospasm, so it would be more useful drugs such as nitroglycerin. But what if we do not know if it is a Kounis syndrome or not? In this article we report our experience that maybe could help you in a similar situation.

Paciente do sexo feminino, 58 anos, sem fator de risco cardiovascular, submetida a cirurgia para reparação do manguito rotador. A indução do bloqueio do plexo braquial interescalênico foi feita sem intercorrência, mas, após seu posicionamento para a cirurgia, a paciente apresentou broncoespasmo grave, hipotensão, reação alérgica cutânea e elevação do segmento ST ao eletrocardiograma. Houve suspeita de choque anafilático que foi tratado, mas até que a perfusão de nitroglicerina fosse iniciada não houve resolução das alterações eletrocardiográficas. Após teste diagnóstico necessário, o diagnóstico final foi de variante tipo I da síndrome de Kounis por causa de cefazolina e rocurônio. Epinefrina é a base sólida do tratamento para anafilaxia, mas devemos usá-la se a reação anafilática também for acompanhada de isquemia miocárdica? A resposta é que não devemos usá-la, porque a isquemia miocárdica nessa síndrome é causada por vasoespasmo; portanto, drogas como a nitroglicerina seriam mais úteis. Porém, e quando não sabemos se é ou não uma síndrome de Kounis? Neste artigo relatamos nossa experiência que, talvez, possa ajudar em uma situação similar.

Paciente del sexo femenino, 58 años de edad, sin factor de riesgo cardiovascular, sometida a cirugía para la reparación del manguito rotador. La inducción del bloqueo del plexo braquial interescalénico fue realizada sin intercurrencias, pero después de su posicionamiento para la cirugía, la paciente presentó broncoespasmo grave, hipotensión, reacción alérgica cutánea y elevación del segmento ST al electrocardiograma. Hubo sospecha de choque anafiláctico que fue tratado, pero hasta que la perfusión de nitroglicerina se iniciase no hubo resolución de las alteraciones electrocardiográficas. Después del test diagnóstico necesario, el diagnóstico final fue de variante tipo i del síndrome de Kounis debido a la cefazolina y al rocuronio. La epinefrina es la base sólida del tratamiento para la anafilaxia, pero ¿debemos usarla si la reacción anafiláctica también viene seguida de isquemia miocárdica? La respuesta es que no debemos usarla porque la isquemia miocárdica en ese síndrome está causada por el vasoespasmo; por tanto, fármacos como la nitroglicerina serían más útiles. Sin embargo, ¿y cuando no sabemos si es o no un síndrome de Kounis? En este artículo, relatamos nuestra experiencia que, tal vez, pueda ayudarle a usted a hacer frente a una situación similar.

Cefalalgia cardíaca: cuando la angina es embriagadora / Cardiac cephalalgia: when angina is heady

La angina cafalalgica cardíaca es un fenómeno poco frecuente como manifestación de un evento coronario agudo. Presentamos un caso típico de ésta manifestación que se asocia a la presencia de vasospasmo coronario y flujo coronario muy lento a nivel epicárdico y miocárdico. La presencia de palpitaciones durante la cefalea advirtió a la paciente del probable origen cardíaca de sus síntomas.

Headache angina or heart headache is a rare phenomenon as a manifestation of an acute coronary event. We report a typical case of this event that is associated with the presence of coronary vasospasm and slow coronary flow on myocardial and epicardial levels. The presence of palpitations during the headache warned the patient of probable cardiac origin of their symptoms.

Espasmo coronariano no pós-operatório de cirurgia de revacularização do miocárdio sem circulação extracorpórea: Diagnóstico e manejo / Coronary-artery spasm after coronary artery bypass graft surgery without extracorporeal circulation: Diagnostic and management

O espasmo de artérias coronárias no perioperatório de cirurgia de revascularização do miocárdio é uma complicação grave, com elevada mortalidade. Paciente de 51 anos submetido à cirurgia de revascularização do miocárdio sem circulação extracorpórea. Apresentou no 1ºdia de pós-operatório (PO) alteração enzimática e supradesnivelamento do segmento ST, evoluindo, em seguida, em fibrilação ventricular, com reanimação cardiopulmonar com sucesso. Cateterismo cardíaco demonstrou espasmo importante de todas as artérias coronárias e da anastomose entre artéria torácica interna esquerda com artéria interventricular anterior. Utilizados vasodilatadores intracoronarianos e intra-enxerto, com restabelecimento de seus calibres usuais, imediata melhora clínica e estabilidade hemodinâmica. Com evolução satisfatória, o paciente recebeu alta hospitalar no 13º PO.

Coronary artery spasm in perioperative of coronary artery bypass graft surgery is a serious complication, with high rate mortality. Patient 51 years-old submitted to coronary artery bypass graft surgery without Extracorporeal Circulation. The patient evolved in 1st post operative (PO) day with enzymatic alteration and ST-elevation, developing soon afterwards in ventricular fibrillation, defibrillation with success. Cardiac catheterization showed important spasm of all coronary arteries and anastomosis between the left internal thoracic artery and the left anterior interventricular artery. Intracoronary Vasodilators and intra-graft, with re-establishment of their usual and immediate calibers to improve clinic and Hemodynamic stability was used. Satisfactory evolution, discharged at 13rd PO day.

La seguridad del eco-stress farmacológico ergotaminérgico para valorar el espasmo coronario en el laboratorio de ecocardiografía: 13 años de experiencia sobre 478 estudios en 464 pacientes / Safety of ergotamine pharmacology stress echocoardiography for vasospasm testing at the echo laboratory

La seguridad del stress ergotaminérgico para desencadenar el vasoespasmo coronario realizado fuera de la sala de hemodinamia ha sido enérgicamente cuestionada. El objetivo de este trabajo fue evaluar el alcance de la prueba de stress con ergotamina/ergovasina efectuada en el laboratorio de ecocardiografía. Con este propósito, evaluamos los datos obtenidos prospectivamente en el laboratorio del Instituto de Fisiología Clínica de Pisa desde el 1 de enero de 1985 hasta el 1 de octubre de 1997, en 478 eco-stress realizados en 464 pacientes con ergotamina/ergovasina. Por selección, todos los pacientes tenían una historia de dolor precordial sugestiva de angor vasoespástico, estudios funcionales por ejercicio negativos o dudosos, sin infarto de miocardio previo, y una función ventricular izquierda normal o cercana a lo normal. La ergotamina/ergovasina fue administrada hasta un total de 0,35 mg de dosis acumulativa, bajo monitoreo continuo de 12 derivaciones de electrocardiograma y ecocardiografía. No hubo muertes, infartos de miocardio, fibrilaciones ventriculares ni bloqueos aurículo-ventriculares de tercer grado. Un paciente presentó una taquicardia ventricular no sostenida asociada con elevación del segmento ST transitoria durante 30 minutos después del estudio. Dos pacientes tuvieron un bloqueo aurículo ventricular de segundo grado asociado con alteraciones segmentarias de la contractilidad, prontamente reversibles con la administración de nitratos. Ocurrieron alteraciones transitorias de la contractilidad regional en 74 pacientes (15 por ciento) otros efectos colaterales independientes de la isquemia se presentaron en 13 pacientes (3 por ciento): hipotensión arterial en un paciente, hipertensión arterial en cinco, taquicardia ventricular no sostenida en dos y nauseas/vómitos en cinco. Se obtuvieron ecocardiogramas interpretables en todos los pacientes con una factibilidad de 97 por ciento. El eco-stress, tanto con ergotamina como con ergovasina, tecnicamente simple, altamente viable y puede ser realizado en forma muy segura en el laboratorio de ecocardiografía con pacientes correctamente seleccionados, en los cuales se sospecha el vasoespasmo coronario. Es a menudo la única manera de documentar el espasmo, que puede ser difícil de interpretar por otros métodos convencionales no invasivos, incluyendo la angiografía coronaria

hyperventilation challenge test for the diagnosis of coronary vasospasm in patients with angina-like chest pain

In the evaluation of patients with agina-like chest pain but with insignificant obstructive coronary artery disease, it is important to exclude myocardial ischemia from coronary artery vasospasm. Hyperventilation, like ergonovine and acetylcholine, can also induce myocardial ischemia and its clinical expressions by provoking coronary vasospasm. A possible mechanism for hyperventilation induction of coronary vasospasm is from promotion of an intracellular influx of calcium ions, induced by the alkalotic state, which then stimulates the vascular contractile process, leading to coronary vasospasm. Fifteen patients with anginal-like chest pain [at least one attack per day] were studied. A hyperventilatory ischemic electrocardiographic changes were reported in 11 patients [73.3%] while a hyperventilatory chest pain was reported in 9 patients [60%]. All the positive responses were reported in the posthyperventilation phase or phase II and ventricular ectopics were seen in one patient [6.6%] during the hyperventilation phase, but neither infarction nor arrest have been reported. The hyperventilation test is an alternative diagnostic tool in evaluating patients with angina-like chest pain of unknown etiology and is an effective alternative diagnostic tool for the diagnosis of active coronary vasospasm, especially when spontaneous electrocardiographic evidence of myocardial ischernia, either silent or with chest pain, is not obtained

Raynaud miocárdico en esclerosis sistémica / Myocardial raynaud in systemic sclerosis

En un estudio prospectivo evaluamos 28 pacientes con Esclerosis Sistémica con Ecocardiografía bidimensional y un test de frío. Durante el test de frío encontramos zonas de hipoquinesia (16 casos) o de aquinesia ventricular (3 casos), transitorias y reversibles luego de cesar el estímulo frío. Estos cambios de la función ventricular inducido por el test de frío, son compatibles con vasoespasmo coronario: "Raynaud miocárdico". Este fenómeno se demostró en 9 de los 10 casos con formas difusa de ES y en 9 de 18 con la variedad más localizada de Acroesclerosis (p < 0.05)

Espasmo arterial coronario: estudio de 66 pacientes / Coronary artery spasm: study of 66 patients

Estudiamos en forma prospectiva 66 pacientes, 27 mujeres y 39 hombres con arteriografía coronaria efectuada entre enero de 1983 y abril de 1985, todos ellos con angor típico, de reposo, o atípico. Ninguno tenía obstrucciones fijas significativas y por ello se estudiaron con maleato de ergonovina en busca de vasoespasticidad. La aplicación del fármaco por vía intracoronaria en 42 pacientes e intra aórtica en 24, permite concluir que la primera vía requiere menores dosis, con ellos las complicaciones son escasas, la efectividad de la prueba se mantiene y facilitó la aplicación inmediata de nitroglicerina intracoronaria en los 25 casos en los que la prueba fué positiva de acuerdo a nuestros criterios, por tener reducción del calibre mayor del 60% o bien entre el 30 y 60% de cualquier rama coronaria epicárdica con dolor precordial y/o desnivel del segmento ST en el electrocardiograma. A diferencia de otros estudios, el espasmo no fué privativo de pacientes con dolor precordial en reposo sino encontramos algunos con angor típico y aún con dolor precordial atípico. El electrocardiograma de reposo y de esfuerzo no fueron característicos de espasmo coronario. Las soluciones de ergonovina y nitroglicerina fueron preparadas durante el estudio sin observar complicaciones importantes relacionadas a ello. Discutimos las indicaciones de la prueba; la efectuamos cuando el dolor precordial que indica la arteriografía coronaria no sea justificado por las lesiones obstructivas poco significativas y el paciente tenga al menos 24 horas sin medicamentos vasoactivos