PO:30:155 | The role of nailfold videocapillaroscopy in the assessment of juvenile connective tissue diseases

Marco Binda¹, Beatrice Moccaldi¹, Francesca Tirelli², Anna Cuberli¹, Andrea Benini¹, Meneghel Alessandra², Andrea Doria¹, Francesco Zulian², Elisabetta Zanatta¹ | ¹U.O.C. Reumatologia, Dipartimento di Medicina DIMED, Università degli Studi di Padova Padova, Italy; ²U.O.S.D. Reumatologia Pediatrica, Dipartimento per la Salute della Donna e del Bambino, Azienda Ospedaliera di Padova, Italy

Background. Nailfold videocapillaroscopy (NVC) represents a reliable, non-invasive tool for the diagnosis of several adult rheumatological diseases, and the assessment of peripheral microvascular abnormalities may serve as a potential biomarker of disease severity in these patients. Unfortunately, data on NVC findings and their clinical significance in paediatric patients are limited. We aimed to evaluate NVC findings in children with primary Raynaud’s phenomenon (PRP) and connective tissue diseases (CTDs). In juvenile CTDs, we aimed to assess potential associations between NVC features and clinical or organ involvement.

Materials and Methods. Paediatric patients who performed NVC for primary and secondary RP over the past five years were included in the study. Clinical and laboratory data were collected and nailfold capillaries images of the distal row of all fingers (excluding the thumb) of both hands were analysed by two independent observers. Six capillaroscopic abnormalities were evaluated: enlarged and giant capillaries (diameter > 20 micrometres and > 50 micrometres, respectively), microhaemorrhages, disarray, capillary ramifications and loss of capillaries (avascular score). For each field a semi-quantitative rating scale was adopted to score each capillary abnormality and an average score value was calculated. Qualitative variables were compared using Chi-squared test or Fisher’s exact test, while quantitative data were compared with Mann–Whitney U test, as appropriate. Per organ system, the association between capillaroscopic patterns and organ involvement was investigated by logistic regression analysis.

Results. A total of 800 NVC images from 50 subjects (30 females; mean age 16.4±4.0 years) were evaluated. Scleroderma pattern was significantly more frequent in juvenile systemic sclerosis (jSSc) compared to both juvenile dermatomyositis and PRP (p=0.003 and p<0.001, respectively). In terms of capillaroscopic alterations, differences were observed only in PRP vs. jSSc for reduction of capillary density (p<0.001) and presence of giants (p=0.01). Scleroderma pattern was associated with skin sclerosis (21/25 vs. 0/9; p<0.001), digital ulcers (8/25 vs. 0/9; p=0.07) and gastrointestinal involvement (17/25 vs. 1/9; p=0.006). Avascular score was higher in children with interstitial lung disease (ILD) than in those without (p<0.05). Patients with severe reduction of capillary density (4 or fewer per millimetre) were more likely to have ILD (5/10 vs. 4/13; p=0.02).

Conclusions. As in the adult population, NVC may help differentiate primary from secondary RP, supporting the early diagnosis of CTDs. The presence of a scleroderma pattern correlates with disease-specific features, while significant capillary loss is associated with ILD. Larger disease-specific cohort studies are needed to confirm these preliminary results.