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Background: There is limited information on how the combination of antibodies other than anti-centromere (ACA) and limited cutaneous systemic sclerosis (lcSSc) will affect the distribution of future organ involvement and mortality. Objectives: We aimed to evaluate the prevalence and progression of the interstitial lung disease(ILD) and pulmonary hypertension(PH) in patients with lcSSc according to the presence of antibodies (ANA only vs anti-scl70 vs ACA). Methods: The medical records of 210 SSc patients who were regularly followed-up between 1988-2021 were screened, the data of 160 patients with limited cutaneous SSc (lcSSc) were extracted and 155 lcSSc patients (145 females, 93.5%) with existing autoantibodies were included into this retrospective analysis. Results: Mean age, duration of Raynaud's and non-Raynaud's were 54.2(±12.5), 15.7(±11.2) and 12.8(±7.5) years, in 155 lcSSc patients with a mean follow-up of 96.2(±68.8) months. ANA was detected in 49 (31.6%) (patterns of speckled in 42.9%, nucleolar in 10.2%, homogeneous in 16.3%, nucleolar + speckled or +homogenous in 18.4%, undefined in 12.3%), anti-Scl70 in 62(40%), and ACA in 44(28.4%). Twenty-five patients (16.1%) progressed to dcSSc during the follow-up in 33.4(±49.5) of months. LcSSc patients with anti-Scl70 more frequently progressed to dcSSc (22 vs 14.3 and 9.1% for ANA and ACA groups). ACA(+)'s less frequently received immune-suppressives (43.2 vs 89.8 and 85.5%, p=0.002). ACA(+) patients who progressed to dcSSc had more frequent initial and cumulative ILD when compared to those did not progressed (p= 0.036 and p=0.032). The frequency of initial and cumulative ILD or worsening ILD was highest in anti-Scl70(+)'s followed by ANA(+)'s and then ACA(+)'s (p=0.003 and p=0.005 or p=0.02, respectively) in lcSSc patients who were not progressed to dcSSc. Although not significant the progression of ILD took longer to occur in ACA(+)'s (med 118 vs 47 and 69 months). Cumulative PH groups were as follows; PAH in 4 patients with ACA(lcSSc), group 3-PH in 2 patients with ANA only (lcSSc), 5 patients with anti-scl70 (n=3, dcSSc) and 3 with ACA (lcSSc)(Table 1). ANA(+) 4 patients (at 36., 39.,48., and 120. months), anti-Scl70(+) one patient (at 235. month) and ACA(+) one patient (at 132.month) deceased during the follow-up period. Conclusion: LcSSc patients with anti-Scl70, tended to progress to dcSSc more frequently. Progression to dcSSc was associated with higher frequency of ILD in ACA(+) patients. Anti-Scl70 or ANA positivity was associated with a higher frequency ILD and progression compared to ACA positivity. Grup 1 PH was diagnosed only in ACA(+)'s, group3-PH was seen in all autoantibody groups. In lcSSc, mostly autoantibodies determine the course of the disease; the potential for progression to dcSSc and major organ involvement should be monitored in high risk patients.REFERENCES: NIL. Acknowledgements: NIL. Disclosure of Interests: Yasemin Yalçinkaya Boehringer Ingelheim, Melodi Gizem Can: None declared, Büşra Demir: None declared, Bahar Artim-Esen: None declared, Ahmet Gül: None declared, Murat Inanc Boehringer Ingelheim.
Yalçınkaya et al. (Sat,) studied this question.