AB0612 SHORT-TERM REVERSIBLE IMPROVEMENT IN EARLY-PHASE ELEMENTS OF NAILFOLD CAPILLARY ABNORMALITIES IN PATIENTS WITH SYSTEMIC SCLEROSIS BY INTRAVENOUS CYCLOPHOSPHAMIDE (IVCY)
Authors: T. Sugimoto, S. Hirata, H. Kohno, H. Watanabe, Y. Yoshida, S. Mokuda, E. Sugiyama
Journal: Annals of the Rheumatic Diseases (2020)

Background: Nailfold capillary abnormalities are one of representative signs in systemic sclerosis (SSc). However, previous reports about changes in nailfold capillary by immunosuppressive therapy have been limited. Especially, there have been no reports about short-term changes in nailfold capillary abnormalities. Objectives: To clarify whether intravenous cyclophosphamide (IVCY) treatment for SSc patients can improve nailfold capillary abnormalities in half a year. Methods: Among patients diagnosed as having SSc according to the 2013 ACR/EULAR classification criteria at our hospital from May 2018 to December 2019, those who treated with IVCY for interstitial lung disease (ILD) were consecutively registered. All patients received IVCY six times. Nailfold capillary abnormalities on eight fingers including both second to the fifth fingers were observed with a nailfold videocapillaroscopy (NVC). Each finger was evaluated for enlarged capillary, giant capillaries, hemorrhage, loss of capillary, disorganization of the vascular array, and capillary ramification. Quantitative scoring was performed on a scale of 0 to 3 in accordance with the ratio of each of them. NVC tests were evaluated before IVCY treatment intervention and after IVCY. In all cases, the evaluation of NVC after IVCY treatment was performed 6 months after the administration day. Skin changes were evaluated by modified Rodnan’s total skin thickness score (mRSS) at performing NVC. Anti-centromere antibodies, anti-Scl-70 antibodies, anti-RNA polymerase III, and anti-RNP antibodies were measured. Pulmonary function tests (PFTs) including forced vital capacity (FVC) and diffusing capacity of the lung carbon monoxide (DL CO ) were performed before and after IVCY. The statistical significance of the differences between means of two groups was evaluated by paired t-test. A p level of 0.05 or less was considered statistically significant. Results: Five patients were included. The mean age was 59 years and 4 patients were female (80%). High dose corticosteroids were used in 2 patients (40%). Anti-RNA polymerase III was positive in 2 patients (40%), anti-Scl-70 antibody was positive in 1 (20%), and negative test for any specific antibodies was in 2 (40%). Changes in NVC scores, which were total scores of 8 fingers, were as follows: Enlarged; 13.2±4.8 to 6.4±5.9 (p=0.018), Giant; 7.0±5.7 to 1.6±1.1 (p=0.0314), Hemorrhage; 8.4±6.2 to 3.2±2.3 (p=0.0274), Loss; 4.0±2.5 to 0.6±1.3 (p=0.0288), Disorganization; 0.6±0.9 to 1.0±1.0 (p=0.7065), Ramification; 0.6±0.9 to 0.8±1.8 (p=0.5730). (Table) After IVCY treatment, mRSS reduced in 4 cases (80%). Changes in mRSS scores were as follows: 18.8±8.3 to 12.4±13.3 (p=0.0677). The cases with improved mRSS and those with improved NVC findings were consistent. The mean FVC before and after IVCY was 2077 ml and 2062 ml, respectively. The mean DL CO before and after IVCY was 9.88 mL/min/mmHg and 9.58 mL/min/mmHg, respectively. Conclusion: Nailfold capillary abnormalities in patients with SSc could be improved in half a year with IVCY. Especially, early phase elements including enlargement, giant, and hemorrhage were specifically reversible. Table. No. (E ) (G ) (H ) (L ) (D ) (R ) mRSS 1 21→14 16→3 19→7 0→0 0→2 0→0 14→9 2 12→3 4→2 6→1 4→0 2→1 1→0 15→1 3 14→11 8→2 6→2 7→0 0→0 0→4 10→5 4 10→4 6→1 8→3 4→3 0→2 2→0 25→12 5 9→0 1→0 3→3 5→0 1→0 0→0 30→35 mean ± SD 13.2±4.8 7.0±5.7 8.4±6.2 4.0±2.5 0.6±0.9 0.6±0.9 18.8±8.3 6.4±5.9 1.6±1.1 3.2±2.3 0.6±1.3 1.0±1.0 0.8±1.8 12.4±13.3 p-value 0.018 0.0314 0.0274 0.0288 0.7065 0.5730 0.0677 E: enlarged, G: giant, H: hemorrhage, L: loss, D: disorganization, R: ramification. The table shows the total of eight points for each finding in the NVC test. The previously described values are before treatment and the later values are after treatment. Disclosure of Interests: Tomohiro Sugimoto: None declared, Shintaro Hirata Grant/research support from: Eli Lilly, Consultant of: Bristol-Myers Squibb, UCB, Paid instructor for: AbbVie, Eisai, Tanabe-Mitsubishi, Speakers bureau: AbbVie, Eisai, Tanabe-Mitsubishi, Astellas, Ayumi, Bristol-Myers Squibb, UCB, Chugai, Eli Lilly, Janssen, Kissei, Sanofi, Takeda, Hiroki Kohno: None declared, Hirofumi Watanabe: None declared, Yusuke Yoshida Grant/research support from: Astellas, Paid instructor for: Astellas, Tanabe Mitsubishi, Sanofi, Novartis, GlaxoSmithKline, Eli Lilly, Bristol-Myers Squibb, Chugai, Asahikasei, Eisai, Janssen, Speakers bureau: Astellas, Tanabe Mitsubishi, Sanofi, Novartis, GlaxoSmithKline, Eli Lilly, Bristol-Myers Squibb, Chugai, Asahikasei, Sho Mokuda: None declared, Eiji Sugiyama Grant/research support from: AbbVie, Astellas, Ayumi, Kissei, Pfizer, Sanofi, Takeda, Tanabe-Mitsubishi, Bristol-Myers Squibb, Chugai, Eisai, Eli Lilly, Speakers bureau: AbbVie, Astellas, Ayumi, Kissei, Pfizer, Sanofi, Takeda, Tanabe-Mitsubishi, Bristol-Myers Squibb, Chugai, Eisai, Eli Lilly, Actelion