BackgroundRecent studies show that Aminaphtone is effective in the treatment of Raynaud’s phenomenon (RP) symptoms in patients with systemic sclerosis (SSc), and an increase in peripheral blood perfusion was demonstrated by Laser speckle contrast analysis in treated patients (1,2). Unfortunately, the drug is only available in a few countries.ObjectivesTo evaluate long-term tolerability and safety of Aminaphtone in SSc patients with secondary RP.MethodsSeventy SSc patients (EULAR/ACR criteria) (mean disease duration 8±7 years, mean age 61±10 years) who started Aminaphtone treatment due to active RP were enrolled and followed for at least 4 years. Patients were also taking various concomitant treatments, including immunomodulators, cyclic intravenous iloprost, endothelin receptor antagonists and aspirin. None was taking sildenafil or selexipag. Survival of Aminaphtone in therapy was assessed along with possible drug-related side effect. The Raynaud condition score (RCS) to asses disease severity and blood examinations were routinely performed.ResultsThe mean follow-up of patients was 49±11 months. Aminaphtone was orally administered at 75 mg twice daily, as standard initial posology in our clinical practice. During the follow-up, six patients (8,6%) referred headache as side effect and had to reduce Aminaphtone posology to 75 mg per day, while maintaining clinical benefits. No other side effect related to the drug was observed during the follow-up. Seven patients increased the posology to 75 mg three times daily due to poor effectiveness, and further seven patients increased the posology to 75 mg three times daily only during the colder months of the year. Conversely, thirty-five patients reduced the dosage to 75 mg once daily only during the hottest months of the year, due to partial remission of the RP. During follow-up, blood tests did not reveal any significant alteration ascribable to Aminaphtone. A subjective improvement of Raynaud’s symptoms (assessed by the RCS) was already evident after 1-2 months of treatment in fifty-six patients (80%). Globally, the patients referred a sustained improvement followed by stabilization of Raynaud’s symptoms during the follow-up.ConclusionDuring an average observation period of four years, Aminaphtone showed a good tolerability and safety profile along with sustained efficacy in patients with SSc-related RP, without disabling or serious side effects. A randomized controlled trial for Aminaphtone use in the management of SSc-related RP is desirable to better assess the clinical efficacy of the drug over time.References[1]Parisi S, et al. Am J Int Med. 2015;3:204–209. 2. Ruaro B et al. 2019. Front Pharmacol 10:293.Disclosure of InterestsANDREA CERE: None declared, Emanuele Gotelli: None declared, Adriano Lercara: None declared, Carmen Pizzorni: None declared, Sabrina Paolino: None declared, Elisa Alessandri: None declared, Maurizio Cutolo Grant/research support from: Bristol Myers Squibb, Celgene, Pfizer, Boehringer Ingelheim, Alberto Sulli: None declared

BackgroundThe temporomandibular disorders (TMDs) encompass a heterogenous group of inflammatory and degenerative diseases which impair the masticatory function causing local pain and dysfunctional consequences of the temporomandibular joint (TMJ) [1].ObjectivesTo systematically review the literature concerning TMDs in immune-mediated rheumatic diseases (IMRDs) of the adult and synthetize their burden in multiple domains of clinical interest: patient-reported outcomes (PROs), frequencies of signs on physical examination, imaging features, histological findings, and risk factors for their development in patients with IMRDs.MethodsA literature search on PubMed Central, Embase and Cochrane Library databases was performed, until June 2022, for studies including TMJ outcomes in IMRDs patients compared with healthy controls, other rheumatic diseases or in the assessed IMRDs patients after follow-up and treatment.Among the IMRDs of the adult, original articles investigating TMJ involvement in inflammatory polyarthritides and/or autoimmune connective tissue diseases were considered.The TMJ outcomes used in clinical studies, the prevalence of TMDs in IMRDs and the risk factors for their development were qualitatively synthetized.The quality of the studies was scored using the Newcastle-Ottawa scale (NOS).ResultsOf the 3259 screened abstracts, 56 papers were included in the systematic review. All of them were evaluated as of fair quality, at least.Most of the papers (77%) investigated TMDs in rheumatoid arthritis (RA) with a prevalence of signs and symptoms varying from 8% to 70%(Table 1).The risk factors for TMDs development in RA were female sex, younger age, anti-citrulline peptide antibodies (ACPA) positivity, higher disease activity, cervical spine involvement, cardiovascular and neuropsychiatric comorbidities(Figure 1).Ten papers (18 %) evaluated TMDs in spondylarthritides (SpA) reporting a prevalence of symptoms and signs in 12%-80% of patients with higher TMDs prevalence in patients with radiographic spine involvement, skin psoriasis and HLADRB1*01 positivity.Among autoimmune connective tissue diseases (CTDs), systemic sclerosis (SSc) displayed the highest evidence of TMDs PROs and clinical findings (20-93%), followed by systemic lupus erythematosus (SLE) in 18-85%, mixed connective tissue disease (MCTD) in 31-63%, primary Sjögren’s syndrome (pSS) in 24-54% and idiopathic inflammatory myopathies (IIMs) in 4-26%.In SSc and SLE, TMDs were more frequent in patients with higher disease activity and duration, correlating with the extent of skin fibrosis in SSc and with renal involvement in SLE.ConclusionTMDs in IMRDs display a significant relevance in the rheumatological clinical practice even if they are often overlooked.This burden is epidemiologically important in terms of PROs and clinical findings which correlate with disease activity in RA, SpA, SSc and SLE.The early recognition and multidisciplinary management of TMDs is warranted and should be aimed at hindering the TMJ structural damage maximizing the quality of life of patients.Reference[1]Covert et al. Diagnostics 2021Table 1.Prevalence of TMJ findings across multiple clinical domains in different IMRDs.IMRD Domains of TMJ involvementRASpASScSLEpSSMCTDIIMsNumber of studies investigating TMJ involvement43/56(77%)10/56(18%)5/56(9%)4/56(7%)4/56(7%)3/56(5%)1/56(2%)Prevalence of TMJ PROs8-70%12-80%20-93%31-66%24-54%31%17-26%Prevalence of TMJ signs on physical examination(i.e., reduced mouth opening)30-54%17-68%44-71%41-85%24-44%50-63%4-13%Imaging findings on X-ray of TMJ50-66%30-38%NA22%NA19%NAImaging findings on computerized tomography of TMJ61-76%NANANANANANAImaging findings on magnetic resonance imaging of TMJ11-95%6-67%67-94%NANA13-93%NAHistological findings of TMJSynovitis and degenerative changesNANANANANANALegenda.NA: not assessed. See the text for the other abbreviations.Figure 1.Risk factors for TMJ involvement in RA, SpA, SSc and SLE (created with biorender.com)Acknowledgements:NIL.Disclosure of InterestsNone Declared.