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Background: Microvascular damage and fibrosis are hallmarks of systemic sclerosis (SSc). The extent of skin fibrosis defines distinct clinical phenotypes: limited (lSSc) and diffuse (dSSc) cutaneous SSc. Although some studies have demonstrated an association between “late” scleroderma pattern (LSP) and loss of capillaries on nailfold videocapillaroscopy (NVC) with dSSc [1, 2, 3], a detailed analysis between the NVC findings and the extent of skin fibrosis in SSc is not yet clearly established.
Objectives: The aim of this study was to compare the NVC findings between lSSc and dSSc patients.
Methods: We recruited 157 patients at the Division of Rheumatology of the University of Genova: 127 with SSc and 30 with primary Raynaud phenomenon (pRP). The NVC pattern and the absolute number of capillaries (1 linear mm) of the first NVC performed were accessed in all patients, as well as the scores A (early NVC changes: capillary dilations, microhemorrhages and giant capillaries) and B (advanced NVC changes: capillary density, altered microvascular architecture and abnormal capillary shapes (angiogenesis)) in SSc pts. These scores are semiquantitative assessments that grade the extent of NVC changes from 0 to 3 (0: no changes; 1: ≤33% changes; 2: 33-66% changes; 3: ≥66% changes) [4].
Results: A statistically significant difference in the absolute number of capillaries between patients with pRP, lSSc and dSSc was found (F (2,154) =50.18,p<0.001). The post-hoc analysis demonstrated that the absolute capillary number was significantly higher in pRP patients (8.87±0.78,p<0.001) than in SSc patients. Though capillary loss was present in both SSc subgroups, it was significantly higher in patients with dSSc (4.89±1.53,p<0.001) compared to lSSc (6.18±1.75,p<0.001). Moreover, in dSSc patients, a significantly higher B score was observed (t (125) =-3.95,p<0.001), with not only a lower capillary density (H (1) =12.01,p<0.001), but also an altered capillary architecture (H (1) =13.96,p<0.001) and presence of abnormal shapes (H (1) =8.00,p=0.005) (Table 1). In addition, a statistically significant association was established between LSP and dSSc (χ 2 (1,126) =10.45,p=0.004), as well as between “early” scleroderma pattern (ESP) and lSSc (χ 2 (1,126) =6.56,p=0.010). The extent of skin fibrosis in SSc and A score showed no association.
Conclusion: The current study reinforces the importance of NVC in discriminating microvascular damage between lSSc and dSSc. Capillary loss is present in both clinical phenotypes, but is particularly higher in dSSc, despite a greater use of immunosuppressants. Furthermore, higher B scores seem correlated with dSSc with more abnormal shapes and altered capillary architecture detected in dSSc. Confirming this advanced NVC changes in dSSc, an association between LSP and dSSc was also documented. No differences regarding giants were found between lSSc and dSSc, with this being explained by the prevalence of ESP (presence of few giants) in lSSc and of LSP (absence of giants) in dSSc.
REFERENCES: [1] Cutolo M et al. Nat Rev Rheumatol 2021.
[2] Ingegnoli F et al . Microvasc Res. 2013.
[3] Sato LT et al . Acta Reumatol Port. 2009.
[4] Sulli A et al. Ann Rheum Dis 2008.
Descriptive and comparative statistics of the demographic, clinical, laboratorial and capillaroscopic variables in lSSc and dSSc; p-value <0.05 was considered significant.
| lSSc (N=100) | dSSc (N=27) | p-value | |
|---|---|---|---|
| AGE AT NVC (years) (M±SD) | 58.64±12.85 | 50.11±13.79 | 0.002 |
| SEX n (%) | 0.010 | ||
| Women | 91 (91.0) | 19 (70.4) | |
| Men | 9 (9.0) | 8 (29.6) | |
| DISEASE DURATION (years) (M±SD) | 9.23±10.45 | 4,93±7.58 | 0.024 |
| ANTIBODY PROFILE* n (%) | |||
| Anti-centromere | 57 (57.0) | 1 (3.7) | <0.001 |
| Anti-Scl70 | 24 (24.0) | 19 (70.4) | <0.001 |
| Only ANA positive | 13 (13.0) | 4 (14.8) | 0.510 |
| Other SSc-associated antibodies** | 9 (9.0) | 5 (18.5) | 0.146 |
| ANA negative | 1 (1.0) | 0 (0) | 0.787 |
| CLINICAL MANIFESTATIONS n (%) | |||
| RP | 98 (98.0) | 26 (96.3) | 0.515 |
| DUs | 19 (19.0) | 14 (51.9) | <0.001 |
| PAH | 6 (6.0) | 1 (3.7) | 0.539 |
| ILD | 26 (26.0) | 9 (33.3) | 0.449 |
| Esophageal involvement | 25 (25.0) | 13 (48.1) | 0.020 |
| MSK involvement | 8 (8.0) | 8 (29.6) | 0.006 |
| mRSS (M±SD) (n=83) | 4.69±4.36 | 17.16±13.08 | <0.001 |
| TREATMENT n (%) (n=81) | |||
| Immunosuppressors $ | 6 (9.7) | 8 (42.1) | 0.003 |
| Vasodilators # | 30 (48.4) | 7 (36.8) | 0.377 |
| NVC PATTERN n (%) | |||
| Normal | 2 (2.0) | 0 (0) | 0.619 |
| Non-Specific Alterations | 13 (13.0) | 4 (14.8) | 0.510 |
| Early Scleroderma | 32 (32.0) | 2 (7.4) | 0.010 |
| Active Scleroderma | 34 (34.0) | 11 (40.7) | 0.516 |
| Late Scleroderma | 7 (7.0) | 8 (29.6) | 0.004 |
| Scleroderma-Like | 12 (12.0) | 2 (7.4) | 0.391 |
| SCORE (M±SD) | |||
| Score A (early NVC changes ) | 4.04±1.25 | 4.04±1.58 | 0.496 |
| Capillary Dilations | 1.99±0.41 | 2.07±0.62 | 0.397 |
| Microhemorrhages | 0.91±0.57 | 0.81±0.74 | 0.296 |
| Giant Capillaries | 1.13±0.69 | 1.15±0.77 | 0.860 |
| Score B (late NVC changes ) | 2.38±2.29 | 4.37±2.45 | <0.001 |
| Capillary Density (1 linear mm) | 1.03±0.93 | 1.74±0.86 | <0.001 |
| Altered microvascular architecture | 0.74±0.81 | 1.48±0.89 | <0.001 |
| Abnormal shapes (angiogenesis) | 0.62±0.75 | 1.15±0.91 | 0.005 |
*Positivity for more than one antibody was observed. **Includes the following autoantibodies: anti-RNA polymerase III, anti-U3 RNP, anti-PmScl75, anti-PmScl100, anti-NOR90, anti-Th/To, anti-Ku and anti-SSA. $ Include methotrexate, azathioprine, cyclosporine A, mycophenolate mofetil, cyclophosphamide, and rituximab # Include pentoxifylline, calcium-channel blockers, phosphodiesterase 5 inhibitors, iloprost, bosentan and aminaftone (Italy). M: mean; SD: standard deviation; ANA: anti-nuclear antibodies; ENA: extractable nuclear antigen antibodies; RP: Raynaud phenomenon; DUs: digital ulcers; PAH: pulmonary arterial hypertension; ILD: interstitial lung disease; MSK: musculoskeletal;mRSS: modified Rodnan Skin Score.
Acknowledgements: NIL.
Disclosure of Interests: None declared.
© The Authors 2025. This abstract is an open access article published in Annals of Rheumatic Diseases under the CC BY-NC-ND license (