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.

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

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.

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.

Nailfold videocapillaroscopy in healthy children and adolescents: description of normal patterns.

OBJECTIVES: to describe normal patterns of nailfold videocapillaroscopy (NVC) in healthy children and adolescents; to quantify the relationship between age and capillary dimensions, intercapillary distance and number of capillaries/mm; to evaluate the inter and intraobserver concordance. METHODS: Cross-sectional study including 100 healthy participants aged 5 to 18 years. Capillary dimensions (capillary loop length, capillary width and intercapillary distance) and number of capillaries/mm were evaluated in 900 capillaries using stereomicroscope under 100x magnification. Intra and inter observer agreements were tested. RESULTS: The capillary dimensions (mean ± SD) were: capillary loop length 278.6±60.3 µm, intercapillary distance 124.1±28.1 µm, capillary width 15.0±2.6 µm. Teenagers between 15 and 18 years had longer and more enlarged capillaries than the other age groups (p<0.001 and p=0.012 respectively). We also found a significant increase in the number of capillaries/mm with age (p<0.001). There was a positive correlation between age and number of capillaries/mm, capillary length, and capillary width (p<0.001, R=0.796; p<0.001, R=0.368; p=0.004, R=0.285, respectively). There was a good intra and interobserver concordance. Enlarged capillary and avascular areas were present in 11% and 10% of capillaries respectively. A weak negative correlation was found between the intercapillary distance and the number of capillaries/mm (p=0.05; R=-0.20). CONCLUSIONS: There is a wide variability in the capillary morphology among healthy individuals. There was a positive correlation between age and number of capillaries/mm, capillary length, and capillary width. In addition, NVC has been shown to be a reproducible method.

Reliability of widefield nailfold capillaroscopy and video capillaroscopy in the assessment of patients with Raynaud's phenomenon.

OBJECTIVE: To analyze the diagnostic performance and reliability of different parameters evaluated by widefield nailfold capillaroscopy (NFC) with those obtained by video capillaroscopy in patients with Raynaud's phenomenon (RP). METHODS: Two hundred fifty-two individuals were assessed, including 101 systemic sclerosis (SSc; scleroderma) patients,61 patients with undifferentiated connective tissue disease, 37 patients with primary RP, and 53 controls. Widefield NFC was performed using a stereomicroscope under 10­25 x magnification and direct measurement of all parameters. Video capillaroscopy was performed under 200 x magnification, with the acquirement of 32 images per individual (4 fields per finger in 8 fingers). The following parameters were analyzed in 8 fingers of the hands (excluding thumbs) by both methods: number of capillaries/mm, number of enlarged and giant capillaries, microhemorrhages, and avascular score.Intra- and interobserver reliability was evaluated by performing both examinations in 20 individuals on 2 different days and by 2 long-term experienced observers. RESULTS: There was a significant correlation (P < 0.000) between widefield NFC and video capillaroscopy in the comparison of all parameters. Kappa values and intraclass correlation coefficient analysis showed excellent intra- and interobserver reproducibility for all parameters evaluated by widefield NFC and video capillaroscopy. Bland-Altman analysis showed high agreement of all parameters evaluated in both methods. According to receiver operating characteristic curve analysis, both methods showed a similar performance in discriminating SSc patients from controls. CONCLUSION: Widefield NFC and video capillaroscopy are reliable and accurate methods and can be used equally for assessing peripheral microangiopathy in RP and SSc patients. Nonetheless, the high reliability obtained may not be similar for less experienced examiners.

Nailfold capillaroscopy abnormalities as predictors of mortality in patients with systemic sclerosis.

OBJECTIVES: Peripheral microangiopathy is a hallmark of systemic sclerosis (SSc) and can be early detected by nailfold capillaroscopy (NFC). This study aimed to examine whether more severe peripheral microangiopathy at NFC are predictive factor for death in SSc patients. METHODS: 135 SSc patients who performed NFC between June 2001 and July 2009 were included. The following NFC parameters were evaluated: number of capillary loops/mm, avascular score (scored from 0 to 3), and number of enlarged and giant capillary loops. Univariate and multivariate regression models were used to analyse the association of mortality with NFC and clinical parameters. RESULTS: At the time of the analysis (August 2010), 123 patients were alive, and 12 were dead. By univariate analysis, male gender, forced vital capacity <75% predicted, higher number of giant capillary loops, and an avascular score >1.5 on NFC were associated with a significantly increase risk of death. By multivariate analysis, an avascular score >1.5 was the only independent predictor of death (hazard ratio 2.265). Survival rates from diagnosis at 1, 5 and 10 years were lower in patients with avascular score >1.5 (97%, 86%, and 59%, respectively) compared with those with avascular score ≤1.5 (97%, 97%, and 91% respectively) (p=0.009 by log rank test). CONCLUSIONS: Avascular scores higher than 1.5 at NFC was an independent predictor of death in SSc, suggesting that NFC can be useful for predicting SSc outcome.