However, if the patients have autoantibodies related to CTD, we usually consult the rheumatologists to determine whether the diagnosis of CTD can be fulfilled. phenotypic characteristics resemble those of RA-ILD and ACPA-positive ILD but not RA [8]. In RA-ILD or ILD associated with ACPA, unlike in most other CTDs, the UIP pattern is more commonly seen than NSIP (8,23). Additionally, no previous PF-4 reports showed a NSIP + OP pattern in RA-ILD including ILD associated with ACPA. Only 1 1 patient (case 7) showed a possible UIP pattern in our study. Taken together, the results of our patients and previous case reports suggested the possibility that ACPA-positive ASS favors a characteristic radiological ILD pattern of ASS rather than ILD associated with RA or ACPA. Table 2 Summary of case reviews of ILD associated with ACPA-positive ASS (10)63/femalePL-769.025++NFB PENSIP + OPN/APSL + CyAImproved (unknown)Park (11)56/femaleJo-1+3755++Mechanics handNSIP + OPN/APSL + CPAImproved (unknown)Watanabe (12)54/femaleEJ+N/A+??N/AFibrotic NSIPN/AN/A62/maleEJ+N/A+?Fingertip eczemaN/AN/AN/AN/ATomioka (13)53/femaleEJ 10082+??NSIP + OPFibrotic NSIPPSL + CyAImproved (5 years) Open in a separate window ACPA, anti-citrullinated peptide/protein antibody; ARS, aminoacyl tRNA synthetase; ASS, anti-synthetase syndrome; CK, creatine kinase; PF-4 CPA, cyclophosphamide; CyA, cyclosporine; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; N/A, not available; NFB, nail fold bleeding; NSIP, nonspecific interstitial pneumonia; PE, periungual erythema; PSL, prednisolone; OP, organizing pneumonia. The most common histological pattern of CTD-ILD is NSIP (24). In contrast, the proportion of UIP pattern was reported to be higher in ILD associated with RA or ACPA followed by NSIP (8,25). Although it was previously thought in the past that lymphoid follicles are commonly seen especially in ILD associated with RA or ACPA, lymphoid follicles were a remarkable finding in ILD associated with ASS as reported by Watanabe (13). Our previous pathological analysis of anti-EJ antibody associated ILD also showed lymphoid follicles in half of the cases (26). Our cases with available pathological findings showed NSIP with or without lymphoid follicles, and previous case reports also showed the same (reported that approximately 40% of ACPA-positive ASS patients were still under prednisolone treatment at PF-4 a dosage 10 mg/day (mean follow-up, 93.19 months) (28). None of our 3 patients (case 1C3) experienced a PF-4 relapse of arthralgia under prednisolone (10 mg/day) during the follow-up period. Although only a few cases were analyzed, combined therapy with tacrolimus as a calcineurin inhibitor might also be effective. The reported treatment response of ILD of most patients, whether those with NSIP (including NSIP + OP) in association with ASS or CTD (e.g., RA, PM/DM) was often good as in our patients (16,21,29). Recent guidelines recommend serologic evaluations such as rheumatoid element, ACPA, and anti-nuclear antibody in ILD, actually in the absence of signs or symptoms of CTD, provided that more specific antibodies such as anti-ARS antibodies are evaluated in select instances because of unclear diagnostic value of the additional evaluations and to improve cost effectiveness (15). However, anti-TNF providers for RA may even result in myositis and/or ILD in ASS and lead to refractory arthritis (27,30). In other words, RA therapy may lead to harmful events (including ILD) in individuals diagnosed as having RA when the individuals ILD is associated with ACPA-positive ASS. Consequently, we may perform serologic checks for anti-ARS antibodies in ILD individuals even if they are positive for ACPA. Particularly, special care should be taken when assessing individuals having a radiological ILD pattern of ASS rather than RA or ACPA, as explained above. Our PF-4 results need to be interpreted with extreme caution due to the following limitations. First, this was a retrospective, the sample size was small, and some medical and pathological findings were not available. Second, since ILD became the reason FRAP2 why our patient was diagnosed as ACPA-positive ASS, this study was viewed from respiratory physicians, not rheumatologists. However, if the individuals have autoantibodies related to CTD, we usually consult the rheumatologists to determine whether the analysis of CTD can be fulfilled. Third, there was selection bias because not all of the individuals with ILD were evaluated for.