Background/Purpose: Juvenile dermatomyositis (JDM) is a rare systemic autoimmune disease characterized by vasculopathy and rash. The Bohan-Peter criteria for dermatomyositis are not ideal. There is no widely accepted test that has been consistently used in JDM to monitor disease activity. Abnormal nailfold capillaries, indicating presence of vasculopathy in JDM, occurs in approximately 35-91% of patients. Evidence suggests nailfold capillary density (NCD) may be an indicator of skin and muscle disease activity. Thus nailfold capillaroscopy (NFC), a sensitive, non-invasive approach to recognize early microvascular changes in JDM, could be used to monitor course of illness. The purpose of this survey is to assess current practices and barriers to the use of NFC by pediatric rheumatologists in connective tissue diseases including JDM.

Methods: An electronic survey was created to evaluate individual practices for the use of NFC and any barriers in using this technique. The survey was distributed to pediatric rheumatologist members of CARRA after IRB and CARRA approval via secure server to email addresses. Participation was voluntary, and responses were collected in a de-identified manner. Results were analyzed using descriptive statistics and chi-square tests.

Results: Survey response rate was 46%. 129 responders provided direct care to JDM patients and were included in the analysis. 72% of responders had significant experience with six or more years since completing training. 82% of responders reported “almost always” using NFC in the diagnosis of JDM as compared to 65% in MCTD, 78% in systemic sclerosis, 34% in SLE, and 79% in primary RP. 70% of responders “almost always” used NFC in monitoring of disease activity in JDM compared to 57% in systemic sclerosis. 60% of responders used an ophthalmoscope, 58% used a dermatoscope, 4% used a microscope, and 0% used nailfold videocapillaroscopy to obtain images. 95% of responders evaluated images of nailfolds purely by inspection without a quantitative mechanism for evaluation (such as measuring NCD). 93% of survey responders reported that using NFC influences their clinical diagnosis. No statistically significant difference was seen in the practice of using more advanced mechanisms of NFC (methods other than an otoscope or ophthalmoscope) with regards to experience of the responder. Barriers to use of NFC included cost of equipment (29%), time it takes to perform in clinic visits (26%), and inability to bill for procedure (20%). In the survey, responders commented on the need for standardization of technique and procedure in the analysis of NFC.

Conclusion: NFC is being used frequently in the surveyed population for diagnosis and monitoring of disease activity in JDM, MC­TD, systemic sclerosis, and primary RP. The ophthalmoscope and dermatoscope are the most frequently used methods, and use was not associated with experience of the physician. When evaluating nailfold images, the majority of pediatric rheumatologists use visual inspection rather than objective methods of quantification and analysis. There appears to be interest in the pediatric rheumatology community for the implementation of a standardized methodology for evaluation and interpretation of nailfold capillaropathy.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/nailing-down-nailfold-capillaroscopy-practices-a-survey-of-pediatric-rheumatologists-within-the-childhood-arthritis-and-rheumatology-research-alliance-carra-network/