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Dermatomyositis (DM) is an immune mediated inflammatory disease classically associated with muscular and cutaneous involvement. Existing studies have suggested characteristic nailfold findings may be observed in DM, indicating a potential role for nailfold microscopic examination in the diagnosis of DM. To that end, we performed a systematic review of literature pertaining to nailfold microscopic, capillaroscopic, and dermoscopic findings observed in patients with DM, with a secondary review of the association of nailfold microscopic findings with myositis-specific antibody (MSA) and myositis-associated antibody (MAA) status. Thirty-seven papers, representing 346 patients, met inclusion criteria. The most prevalent nailfold findings were evidence of increased vascular diameter (64.5%, n  = 223) and decreased vascularity (57.5%, n  = 199). Scleroderma-spectrum pattern and microhemorrhage or hemorrhage were both observed in 156 (45.1%) patients. Fifty-one patients had nailfold capillaroscopic findings reported in direct association with antibody positivity and demonstrated a range of nailfold findings, preliminarily suggesting associations between antibody-status and capillaroscopic findings. The results of our study align with existing data indicating the scleroderma-spectrum pattern and the pattern’s composite features are prevalent in patients with DM. Our review further demonstrates a variety of nailfold capillaroscopic findings are found in patients positive for MSAs and MAAs.

A 51-year-old woman with a history of Raynaud phenomenon was referred to the rheumatology department due to edematous hands. Physical examination revealed puffy fingers, periungual erythema, dilated capillaries, and hemorrhages of the proximal nail folds. Giant capillaries were also observed using a dermascope. Serologic tests showed positive antinuclear antibody and anticentromere antibodies, leading to a diagnosis of limited cutaneous systemic sclerosis. The patient was treated with prednisone, mycophenolate mofetil, and beraprost sodium, resulting in improvement of symptoms. Nail fold capillary abnormalities and Raynaud phenomenon are characteristic of early systemic sclerosis. Close monitoring for complications such as interstitial lung disease and digital ulcers is important in systemic sclerosis. Alternative diagnoses include mixed connective tissue disease and dermatomyositis. This case highlights the importance of recognizing nail fold capillary abnormalities and early signs of systemic sclerosis.

Video nailfold capillaroscopy (NFC), considered as an extension of the widefield technique, allows a more accurate measuring and storing of capillary data and a better defining, analyzing, and quantifying of capillary abnormalities. Capillaroscopic study is often performed on the patients suspected of having microcirculation problems such as Raynaud’s phenomenon as the main indication for nailfold capillaroscopy. Capillaroscopic findings based on microcirculation studies can provide useful information in the fields of pathophysiology, differential diagnosis, and monitoring therapy. Nailfold capillaroscopy provides a vital assessment in clinical practices and research; for example, its reputation in the early diagnosis of systemic sclerosis is well established and it is also used as a classification criterion in this regard. This review focuses on the manner of performing video nailfold capillaroscopy and on a common approach for measuring capillary dimensions in fingers and toes.

Although nailfold capillaroscopy (NFC) appears to have a bright future in clinical practice, the lack of familiarity with the technique and how to interpret its outcomes is major barriers which have made nailfold capillaroscopy an underutilized method in standard clinical practice. Traditional methods for assessment and measurement of capillary patterns, density, and blood flow are falling behind and face some challenges. In fact, there have been calls for improvement, hence the recent publication of the standardization of NFC by the EULAR Study Group on Microcirculation in Rheumatic Diseases. Nailfold capillaroscopy has the advantage of being a non-invasive technique that provides a window into the digital microcirculation. This paved the way for a rapidly growing interest in using capillaroscopy parameters as outcome measures in research. In standard clinical practice, whilst its main application is in the identification of an underlying systemic sclerosis spectrum disorder in patients presenting with Raynaud’s phenomenon, its use has expanded to include other clinical features possibly suggestive of an underlying connective tissue disease. This article presents the challenges, provides tips, and highlights the exciting potential of nailfold capillaroscopy in standard practice.

Objective Assessment of associations of nailfold videocapillaroscopy (NVC) scleroderma patterns (‘early’, ‘active’ and ‘late’) with future severe clinical involvement in a systemic sclerosis (SSc) population. Methods Sixty-six consecutive patients with SSc according to the LeRoy and Medsger criteria underwent NVC assessment at baseline. Videocapillaroscopic images were classified into ‘normal’, ‘early’, ‘active’ or ‘late’ NVC pattern. Clinical evaluation was performed for nine organ systems (general, peripheral vascular, skin, joint, muscle, gastrointestinal tract, lung, heart and kidney) according to the disease severity scale of Medsger (DSS) at 18–24 months of follow-up. Severe clinical involvement was defined as category 2–4 per organ of the DSS. Results NVC patterns were significantly associated with future severe, peripheral vascular/lung involvement at 18–24 months. The OR rose steadily throughout the patterns. The OR for future severe peripheral disease based on simple/multiple (correcting for disease duration, subset and medication) logistic regression was 2.49/2.52 (95% CI 1.33 to 5.43, p=0.003/1.11 to 7.07, p=0.026) for early, 6.18/6.37 for active and 15.35/16.07 for late NVC scleroderma patterns versus the normal NVC pattern. The OR for future severe lung involvement based on simple/multiple regression was 2.54/2.33 (95% CI 1.40 to 5.22, p=0.001/1.13 to 5.52, p=0.021) for early, 6.43/5.44 for active and 16.30/12.68 for late NVC patterns. Conclusions This pilot study is the first demonstrating an association between baseline NVC patterns and future severe, peripheral vascular and lung involvement with stronger odds according to worsening scleroderma patterns. This may indicate a putative role of capillaroscopy as a biomarker.

Nailfold Capillaroscopy (NFC) is a simple, non-invasive diagnostic tool used to detect microvascular changes in nailfold. Chronic pathological changes associated with a wide range of systemic diseases, such as diabetes, cardiovascular disorders, and rheumatological conditions like systemic sclerosis, can manifest as observable microvascular changes in the terminal capillaries of nailfolds. The current gold standard relies on experts performing manual evaluations, which is an exhaustive time-intensive, and subjective process. In this study, we demonstrate the viability of a deep learning approach as an automated clinical screening tool. Our dataset consists of NFC images from a total of 225 participants, with normal images accounting for 6% of the dataset. This study introduces a robust framework utilizing cascade transfer learning based on EfficientNet-B0 to differentiate between normal and abnormal cases within NFC images. The results demonstrate that pre-trained EfficientNet-B0 on the ImageNet dataset, followed by transfer learning from domain-specific classes, significantly enhances the classifier’s performance in distinguishing between Normal and Abnormal classes. Our proposed model achieved superior performance, with accuracy, precision, recall, F1 score, and ROC_AUC of 1.00, significantly outperforming both models of single transfer learning on the pre-trained EfficientNet-B0 and cascade transfer learning on a convolutional neural network, which each attained an accuracy, precision, recall, and F1 score of 0.67 and a ROC_AUC of 0.83. The framework demonstrates the potential to facilitate early preventive measures and timely interventions that aim to improve healthcare delivery and patients’ quality of life.

Nailfold Capillaroscopy in Rheumatic DiseaseA 19-year-old man presented with a 3-year history of Raynaud’s phenomenon and a 1-year history of fatigue, rash on the face and hands, and pain in the finger joints.

Objective This study aimed to characterize nailfold videocapillaroscopy (NVC) features in patients with different subtypes of Idiopathic inflammatory myopathy (IIM) and to investigate the correlations between NVC findings, myositis-specific antibody (MSA) subtypes, disease activity, cytokine profiles, and interferon-stimulated gene (ISG) expression levels. Methods This cross-sectional observational single-center study included 55 IIM patients, categorized into MDA5 (+), anti-aminoacyl-tRNA-synthetase antibodies (ARS) (+), and MSA(-) groups based on their MSA profiles. Demographic data, laboratory tests, and NVC assessments were systematically collected and analyzed. The relative expression of type I ISGs in whole blood, as well as serum cytokine and chemokine profiles, were measured. Statistical analyses were performed to explore correlations between NVC scores and clinical parameters, including serum biomarkers. Results NVC abnormalities were observed in most IIM patients, with significant differences in NVC features among the MSA subgroups. The MDA5(+) group exhibited significantly higher scores for capillary dilation ( P  < 0.01), giant capillaries ( P  < 0.05), microhemorrhages ( P  < 0.01), and abnormal capillary morphology ( P  < 0.05) compared to the ARS (+) group. ISG expression and cytokine levels were upregulated in IIM patients, with active disease patients showing significantly higher levels of certain ISGs and cytokines compared to clinically stable patients. Notably, specific NVC score dimensions were positively correlated with the levels of certain ISGs and cytokines. For example, microhemorrhage, capillary dilation, and capillary density all had significantly positive correlations with MX1, IFI27, IP-10, RANTES, and GROα ( P  < 0.05). And giant capillary is also related to levels of IFI27, SDF-1α, IP-10, RANTES, and GROα ( P  < 0.05). Conclusion IIM patients exhibit distinct NVC abnormalities, which vary across different MSA subtypes. NVC findings have potential clinical value in screening disease activity and interferon pathway activation in IIM patients.

To systematically review the role of ultrasound (US) in the assessment of the joint-enthesial-nail apparatus in patients with psoriatic arthritis (PsA) or psoriasis (PSO) in terms of prevalence, diagnosis, prognosis, monitoring and treatment. A systematic literature review was conducted through medical databases (PubMed, Embase) and the grey literature up to February 2018. The main areas of application of nail US were first identified, allowing the development of research questions, which were rephrased following the PICOs methodology to develop inclusion criteria. Of the 585 studies produced by PubMed and Embase searches, 17 studies met the criteria for inclusion. Five additional studies were included: 1 from the hand search and 4 from the 2016–2017 ACR and EULAR congresses. The prevalence of nail plate changes varied from < 10 to 97%, for power Doppler signal from 20–30 to 96% and distal interphalangeal joint (DIJ) involvement from 8.9 to 100%. The performance of US nail/DIJ abnormalities in the diagnosis of PsA and PSO elementary lesions was analysed by five studies, with a wide heterogeneity. Reproducibility and reliability of US nil/DIJ were assessed by interclass correlation coefficient or Cohen’s k and their values ranged from 0.6 to 0.9. The value of US nail/DIJ in the monitoring of the lesions was analysed only by a single study. The analysis revealed applications for US nail/DIJ in PsA and PSO and highlights limitations. Validation is strongly needed to demonstrate its appropriateness in the clinical practice and to define its diagnostic and prognostic role.

To describe various demographic, clinical, laboratory, and capillaroscopic features of Egyptian patients with systemic sclerosis (SSc) and to explore the relation between various capillaroscopic features and internal organ involvement as well as other disease parameters. In this cross-sectional multi-centric prospective analysis, two hundred twenty-two adult patients with SSc were recruited. Data regarding general and rheumatological examination including the modified Rodnan skin score (MRSS), internal organ involvement and related imaging and laboratory investigations were collected. Both activity and severity indices were measured. Nail fold capillary microscopy (NFC) was performed for 144 patients. Out of 222 patients; 139 (62.6%) had limited type while 83 (37.4%) had diffuse type, middle aged females were predominant (91%). Peripheral vascular, pulmonary, gastrointestinal and general manifestations were the most frequently documented affection with high activity, severity profiles and bad prognostic features in more than half of the patients. MRSS was above 20 in 122 patients (55%), and 126 patients (56.8%) had pulmonary hypertension (SPAP) while 78.4% had ILD which showed a weak correlation to age and disease duration (r = 0.135, r = − 0.152 respectively). Significant weak correlations were found between disease duration and SPAP (r = 0.134), Medseger’s severity score (r = 0.189), peripheral vascular, skin, and GIT affection severity (r = 0.210, 0.135, and 0.196 respectively). Significant difference was observed between different NFC patterns and antibodies, severity scale, some disease manifestations i.e.: general, gastrointestinal, myopathy, and digital ulceration while no significance was found between NFC patterns and the type of SSc. This study provided a comprehensive clinical and laboratory characterization of a large cohort of Egyptian patients with SSc which reflects a more severe disease with unfavorable prognostic aspects compared to the available world-wide reports. Nail fold capillaroscopy showed many associations to different aspects of the disease especially in late and active capillaroscopic features, if introduced early, it may improve the disease outcome.