Multidimensional assessment of Juvenile Sjögren disease

Abstract

Objectives: This study aims to provide a multidimensional assessment of juvenile Sjögren's disease (jSjD), focusing on diagnostic and management processes through various instrumental tests.

Methods: A cross-sectional and retrospective study was conducted on 21 jSjD patients diagnosed between June 2016 and September 2023. In addition, 37 healthy children were included only for comparison of nailfold videocapillaroscopy (NVC) findings. Clinical data, parotid gland ultrasound (US), NVC, salivary gland biopsy, and Schirmer test results were analyzed.

Results: Positive correlations were found between parotitis scores in parotid gland US and bizarre capillary, neoangiogenesis, and microhemorrhage scores in NVC (r = 0.35, 0.32, 0.52; p < 0.05). Diagnostic delay was associated with higher parotitis frequency and scores in US (p = 0.025) and increased dilated capillaries in NVC (p < 0.05). Clinically evident parotitis correlated with higher parotitis frequency in US (p = 0.009) and higher parotitis scores (r = 0.480, p = 0.051). Higher ESSDAI scores at diagnosis correlated with increased parotitis frequency and scores in US (p = 0.001), with higher scores in patients with high disease activity (p < 0.05). ENA-positive patients had higher parotitis scores in US (p = 0.022) and increased cross capillaries in NVC (p = 0.024). Capillary density was lower in the jSjD group compared to healthy controls (p = 0.021), with healthy children having higher median capillary density (p = 0.005). Dilated, bushy, bizarre capillaries, and microhemorrhages were significantly higher in the jSjD group (p < 0.001).

Conclusion: Early and accurate diagnosis is crucial for managing jSjD. The integration of US and NVC provides a comprehensive framework for detecting glandular and microvascular abnormalities, emphasizing the need for multidimensional approach. Key Points • This study provides a multidimensional and comprehensive assessment of Juvenile Sjögren's disease (jSjD) by combining various instrumental tests used in the diagnosis and monitoring of Sjögren's disease. • It is the first study in the literature to demonstrate the connection between glandular and microvascular abnormalities in jSjD patients. • The comparison of nailfold videocapillaroscopy findings in healthy children highlights the pronounced microvascular abnormalities present in jSjD patients. • The results emphasize the importance of early diagnosis and intervention, offering critical insights into the systemic effects and clinical progression of the disease.

Keywords: Juvenile Sjögren’s disease; Multidimensional approach; Nailfold videocapillaroscopy; Parotid gland ultrasound; Salivary gland biopsy; Schirmer test.

Fig. 1

Examples of nailfold videocapillaroscopy images of four patients with juvenile Sjögren's syndrome (magnification 200x); a. One bizarre capillary (arrow) indicating neoangiogenesis and a cross capillary (arrowhead), along with reduced capillary density (5 capillaries/mm) b. Reduced capillary density (5 capillaries/mm) c. One dilated capillary (arrow) and cross capillaries (arrowheads) with normal capillary density d. Reduced capillary density (4 capillaries/mm)

Fig. 2

Correlations between parotitis scores in parotid gland ultrasound and bizarre capillary scores, neoangiogenesis scores, and microhemorrhage scores in nailfold videocapillaroscopy