Durometry as an alternative tool to the modified Rodnan's skin score in the assessment of diffuse systemic sclerosis patients: a cross-sectional study.

BACKGROUND: The reproducibility and reliability of the modified Rodnan's Skin Score (mRSS) are debated due to investigator-related subjectivity. Here, we evaluate if durometry correlates with mRSS in patients with diffuse systemic sclerosis (SSc). METHODS: This cross-sectional study was conducted from December 2018 to June 2019, including 58 diffuse SSc patients. Two certified researchers, blind to each other's scores, performed the mRSS, followed by durometry at 17 predefined skin sites. For durometry and mRSS, individual scores per skin site were registered. Durometry and mRSS results measured by each researcher, as well as scores from different researchers, were compared. Skin thickness measurements from forearm skin biopsies were available in a subset of the patients, for comparisons. Statistical analyses included Cohen's Kappa Coefficient, Intraclass Correlation Coefficient, Kendall's Coefficient and Spearman's test. RESULTS: Mean (standard deviation, SD) patient age was 44.8 (12.9) years, and 88% were female. Inter-rater agreement varied from 0.88 to 0.99 (Intraclass correlation coefficient) for durometry, and 0.54 to 0.79 (Cohen's Kappa coefficient) for mRSS, according to the specific evaluated sites. When data were compared with skin thickness assessed in forearm biopsies, durometry correlated better with skin thickness than mRSS. CONCLUSION: Durometry may be considered as an alternative method to quantify skin involvement in patients with diffuse SSc. The strong inter-rater agreement suggests that the method may be useful for the assessment of patients by multiple researchers, as in clinical trials.

Complete Atrioventricular Block and Cardiopulmonary Involvement in Rapidly Progressive Systemic Sclerosis

Abstract The heart and lung are target organs in systemic sclerosis (SSc) and similar symptoms (dyspnea and cough) may make the differential diagnosis between the two lesions difficult. In addition, complete atrioventricular block (CAVB) is a rare complication of this disease. This case report is about a patient with SSc and pulmonary fibrosis who was admitted to the emergency room with CAVB, heart failure (HF) and progressive worsening of the underlying disease.

Systemic Sclerosis at an Intensive Care Unit: A Case Series and Literature Review.

INTRODUCTION: Systemic sclerosis (SSc) is a multisystemic autoimmune disease. Few studies have focused on the outcomes of SSC patients who require intensive care unit (ICU) admission, largely due to the absence of protocols for the optimal management of this disease during an ICU stay. OBJECTIVES: This study aimed to describe the outcomes of a series of SSc patients admitted to the ICU at a single center in Cali, Colombia. METHODS: Case series of SSc patients admitted to the ICU were reviewed. The main outcome was ICU mortality. Statistical analysis was performed with measures of central tendency and proportions. RESULTS: All the patients (n = 14) were female and either middle-aged or elderly; 9 (64%) were diagnosed with diffuse cutaneous sclerosis, and the remaining 5 patients with limited cutaneous sclerosis. Some were readmitted; therefore, the total number of ICU admissions was 21. The principal causes of ICU admissions were non-SSc-related causes (n = 15 [71.4%]). The respiratory system was the most involved on ICU admissions. The ICU mortality rate was 43% (n = 6). CONCLUSIONS: The severity of the disease at ICU admission and comorbidity are independently associated with ICU-related mortality. Furthermore, the optimal management of SSc patients includes accurate detection of SSc-associated organ involvement. More studies involving this category of patients are needed to establish the best effective protocols.

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.

Assessment of myocardial fibrosis and microvascular damage in systemic sclerosis by magnetic resonance imaging and coronary angiotomography.

OBJECTIVE: Cardiac involvement in SSc is characterized by myocardial fibrosis, arrhythmias and pericarditis. Prevalence studies have shown variable results. The objective of this study was to determine the prevalence of cardiac involvement in SSc patients using the non-invasive, highly sensitive diagnostic methods of cardiac MRI and coronary angiotomography. METHODS: We included 62 SSc patients and excluded those with heart disease prior to the onset of SSc, renal failure, diabetes mellitus, hyperlipidaemia, arterial hypertension, untreated thyroid disease, cor pulmonale, pregnancy or contraindications to performing cardiac MRI. All underwent clinical and laboratory evaluation, ECG, coronary angiotomography and cardiac MRI. RESULTS: The prevalence of myocardial fibrosis was 45% and was higher in dcSSc (59%) than in lcSSc patients (33%; P = 0.04). The mean left ventricular ejection fraction (LVEF) was lower in patients with myocardial fibrosis (56%) than in those without fibrosis (63%; P = 0.0009); myocardial fibrosis on MRI was more frequent in the basal-septal segments of the LV. Seventy-nine per cent of patients had subendocardial perfusion defects and these were associated with higher ultrasensitive serum CRP values. There was no association of myocardial fibrosis or microvascular damage with atherosclerosis. CONCLUSION: The prevalence of myocardial fibrosis on MRI attributable to SSc is 45%, is more frequent and severe in dcSSc patients, is associated with lower LVEF and affects mainly basal LV walls. Microvascular damage in SSc is common and is associated with elevated ultrasensitive CRP levels. Cardiac damage due to SSc is not associated with coronary artery disease.

Hallazgos dermatoscópicos en enfermedades del tejido conectivo en pacientes pediátricos / Dermoscopic findings in connective tissue diseases in pediatric patients

Introducción: La dermatoscopía de superficie ha mostrado utilidad en el examen de pacientes con enfermedades de tejido conectivo. Anormalidades de los vasos capilares del repliegue ungueal proximal se pueden presentar en etapas iniciales de dichas enfermedades y su hallazgo puede ayudar al diagnóstico temprano de éstas. Este estudio tuvo como objetivo describir los hallazgos capilaroscópicos del repliegue ungueal proximal en pacientes pediátricos con lupus eritematoso sistémico, dermatomiositis y esclerodermia. Materiales y métodos: Estudio observacional, prospectivo, transversal y descriptivo. Se incluyeron 53 pacientes de entre 2 y 18 años de edad. El repliegue ungueal proximal de todos ellos fue observado con dermatoscopio manual de luz polarizada. Resultados: Todos los pacientes con dermatomiositis y esclerodermia y 14 de 30 pacientes con lupus eritematoso sistémico mostraron hallazgos anormales en el repliegue ungueal proximal. Éstos consistieron en una desorganización de la distribución capilar normal, aumento de los lazos capilares, capilares ramificados, pérdida progresiva de capilares, megacapilares y microhemorragias. Discusión: Los hallazgos capilaroscópicos del repliegue ungueal proximal en pacientes pediátricos con lupus eritematoso sistémico, dermatomiositis y esclerodermia tienen utilidad para el diagnóstico.

Introduction: Superficial dermatoscopy has proved useful in the examination of connective tissue diseases. Anomalies in capillary vessels of the proximal nailfold may be present in early stages of these diseases, and this finding may aid in their early diagnosis. The objective of this study was to determine capillaroscopic findings of the proximal nailfold in pediatric patients with systemic lupus erythematosus, dermatomyositis and sclerodermia. Materials and methods: Observational, prospective, transversal and descriptive study. Fifty-three patients with an age range between 2 and 18 years were included. The proximal nailfold of each patient was observed with a manual polarized light dermatoscopeResults: All of the patients diagnosed with dermatomyositis and sclerodermia, and 14 of 30 patients with systemic lupus erythematosus showed significant signs in the proximal nailfold, which consisted of capillary distribution disorganization, branched capillaries, progressive loss of capillaries, megacapillaries and microhemorrages. Discussion: Capillaroscopic findings of the proximal nailfold in pediatric patients with systemic lupus erythematosus, sclerodermia and dermatomyositis are useful for diagnosis.

Cold stimulus fingertip lacticemy test--an effective method to monitor acute therapeutic intervention on primary Raynaud's phenomenon and systemic sclerosis.

OBJECTIVES: The recently developed cold stimulus fingertip lacticemy test (CS-FTL) provides biochemical assessment of peripheral perfusion in patients with Raynaud's phenomenon (RP). We evaluated how the CS-FTL test can assess the acute effect of nifedipine in microvascular dynamics on primary RP and RP secondary to SSc. METHODS: A double-blinded controlled trial with crossover design was performed in 20 primary RP and 20 SSc patients. Patients received one single sublingual placebo or 10 mg nifedipine capsule, with crossover after a 15-day washout period. FTL was determined in resting conditions (pre-CS-FTL) and 10 min after CS (post-CS-FTL), before and 1 h after drug administration. Percent variation in post- vs pre-CS-FTL was expressed as deltaCS-FTL. RESULTS: Before intervention, CS induced FTL decrease in primary RP (deltaCS-FTL = -21.3 +/- 13.0%) and FTL increase in SSc patients (deltaCS-FTL = +24.5 +/- 21.2%). Placebo had no effect on pre-CS-FTL, post-CS-FTL and deltaCS-FTL in primary RP and SSc. Nifedipine induced a significant decrease in pre-CS-FTL (1.94 +/- 0.45 vs 1.57 +/- 0.41 mg/dl; P = 0.005) and post-CS-FTL (1.53 +/- 0.35 vs 1.32 +/- 0.37 mg/dl; P = 0.004) in primary RP and a significant decrease in post-CS-FTL (3.18 +/- 1.43 vs 2.56 +/- 1.30 mg/dl; P = 0.028) and deltaCS-FTL (+15.9 +/- 24.7% vs -12.9 +/- 16.6%; P = 0.001) in SSc. CONCLUSIONS: The CS-FTL test was able to demonstrate and quantify a dual effect of nifedipine on the biochemical dimension of peripheral perfusion in primary RP and in SSc patients in which there was a significant improvement in tissue perfusion in resting conditions and after exposure to a CS. The CS-FTL test will enrich the armamentarium for investigation and clinical evaluation of conditions associated with RP.

Prognostic factors of low bone mineral density in systemic sclerosis.

OBJECTIVE: To analyse the results of bone densitometry in patients with systemic sclerosis (SSc), evaluating the prognostic factors of low bone mineral density (BMD) in fertile and postmenopausal patients, and comparing to a control healthy group. METHODS: Cross-sectional study analysing 61 female SSc patients, aged 25 to 51 years, who performed a bone densitometry using dual x-ray absorptiometry. BMD values (lumbar spine, femoral neck, Ward and trochanter) infertile and postmenopausal patients were compared according to SSc clinical variant (limited and diffuse), race, previous use of drugs (corticosteroids and cyclophosphamide) and bone mass index (BMI). These results were compared with 47 fertile and 60 postmenopausal healthy women; multivariate linear regression analysis was used to study the influence of the variables of interest in the BMD results. RESULTS: Twenty-seven SSc patients presented osteopenia and 14 densitometric osteoporosis. No statistical association was found between BMD values and SSc clinical variants, race and previous use of corticosteroids and cyclophosphamide, in the fertile and in the postmenopausal groups. Fertile SSc patients were paired by age and race with the control group, but BMI (p = 0.035) was significantly lower in the SSc group. BMD values of lumbar spine (p = 0.070, statistical trend), femoral neck (p = 0.003), Ward (p < 0.001) and trochanter (p = 0.003) were significantly lower in the SSc group. Postmenopausal SSc patients were paired by age and race with the control group, but BMI (p < 0.001) was also significantly lower in the SSc group. Age at menopause (p = 0.006) was also significantly lower and time from menopause (p < 0.001) was significantly higher in the SSc group. BMD values of femoral neck (p < 0.001), Ward (p < 0.001) and trochanter (p = 0.001) were significantly lower in the SSc group. Multivariate linear regression analysis showed that BMI was the main variable influencing BMD in the fertile and postmenopausal groups. CONCLUSION: In the present study, BMD results in fertile and postmenopausal SSc patients were independent of the SSc clinical variants, race and previous use of corticosteroids and cyclophosphamide. A low BMD in appendicular sites was observed infertile and postmenopausal SSc patients when compared to a control healthy group, associated to a low BMI.