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 Table of Contents  
Year : 2022  |  Volume : 17  |  Issue : 1  |  Page : 73-77

Antisynthetase syndrome with anti-oj antibody: A Case report and review of literature

1 Department of Rheumatology and Clinical Immunology, Star Hospitals, Hyderabad, Telangana, India
2 Department of Radiology and Imaging, Star Hospitals, Hyderabad, Telangana, India

Date of Submission09-Dec-2020
Date of Acceptance01-Jan-2021
Date of Web Publication15-Mar-2022

Correspondence Address:
Dr. Nayan Patel Sureja
Department of Rheumatology and Clinical Immunology, Star Hospitals, Banjara Hills, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_338_20

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Antisynthetase syndrome (ASS) is a rare autoimmune disease characterized by the presence of an anti-aminoacyl transfer RNA synthetase antibody with one or more clinical features. The prevalence of anti-OJ antibodies in ASS is 2%–5%. Information regarding the clinical presentation and evolution of these patients is limited. We report a case of ASS with anti-OJ antibody in a 45-year-old female from India, who presented with arthritis and interstitial lung disease (ILD). She was managed with corticosteroids and intravenous cyclophosphamide. We also described the clinical details of 69 previously reported ASS patients with anti-OJ antibody. Data from these patients including our patient showed that isolated ILD was the most common form of presentation (24%) followed by myositis with ILD (16%), isolated arthritis (15%), and isolated myositis (13%). The prevalence of arthritis, myositis, and ILD, at the disease onset, was 33%, 42%, and 70%, respectively, whereas the overall prevalence was 44%, 77%, and 83%, respectively.

Keywords: Antisynthetase syndrome, arthritis, interstitial lung disease, myositis

How to cite this article:
Sureja NP, Nandamuri S. Antisynthetase syndrome with anti-oj antibody: A Case report and review of literature. Indian J Rheumatol 2022;17:73-7

How to cite this URL:
Sureja NP, Nandamuri S. Antisynthetase syndrome with anti-oj antibody: A Case report and review of literature. Indian J Rheumatol [serial online] 2022 [cited 2022 Oct 1];17:73-7. Available from:

  Introduction Top

Antisynthetase syndrome (ASS) is a rare autoimmune disease characterized by the presence of an anti-aminoacyl transfer RNA (tRNA) synthetase (anti-ARS) antibody with clinical features, including arthritis, myositis, interstitial lung disease (ILD), Raynaud's phenomenon (RP), mechanic's hand (MH), and/or fever.[1] The presence of any one of the clinical features satisfies the Connors' criteria for ASS.[2] For Solomen criteria,[3] two major (myositis and ILD) or one major and two minor features (RP, MH, and fever) are required. A total of eight classes of anti-ARS antibodies have been described, of which anti-Jo-1 (anti-histidyl-tRNA synthetase) is the most common and well-described antibody. The non-Jo-1 antibodies include anti-PL-7 (anti-threonyl-tRNA synthetase), anti-PL-12 (anti-alanyl-tRNA synthetase), anti-EJ (anti-glycyl-tRNA synthetase), anti-OJ (anti-isoleucyl-tRNA synthetase), anti-KS (anti-asparaginyl-tRNA synthetase), anti-Ha (anti-tyrosyl-tRNA synthetase), and anti-Zo (anti-phenylalanyl-tRNA synthetase).[1] The prevalence of anti-OJ antibodies in ASS is 2%–5%.[4],[5] Only one case with anti-OJ antibody is reported from India in a cohort of 250 patients with idiopathic inflammatory myositis.[6] Thus, information regarding the clinical presentation and disease evolution in these patients is limited. Here, we report a patient of ASS with anti-OJ antibodies from Southern India.

  Case Report Top

A 45-year-old female presented with 6 months of symmetrical inflammatory polyarthritis and nonprogressive dyspnea of Medical Research Council (MRC) grade 2. She denied a history of rash, myalgia, muscle weakness, RP, or fever. On examination, multiple joints were tender and swollen, and fine crepitations were heard in both the lungs. Evaluation revealed normal erythrocyte sedimentation rate, C-reactive protein, and creatine phosphokinase. Rheumatoid factor was negative, and anti-cyclic citrullinated peptide antibodies were positive (six times above normal limit). High-resolution computed tomography (HRCT) scan of the chest showed diffuse ground-glass opacities in the bilateral upper and lower lobes with interstitial thickening, fibrosis, and cystic changes, suggestive of ILD [Figure 1a and b]. Forced vital capacity on spirometry was 51%. Antinuclear antibody test showed fine speckled nuclear staining. Line immunoassay for myositis-specific and myositis-associated antibodies detected anti-OJ (1+) and anti-Ro-52 (2+) antibodies. Other anti-extractable nuclear antigen antibodies were negative. With a diagnosis of ASS, she received 1 mg/kg oral corticosteroid along with mycophenolate mofetil (MMF) 2 g/day. MMF was later replaced with monthly infusions of intravenous cyclophosphamide in view of gastrointestinal intolerance. After 2 months of therapy, her respiratory symptoms relatively improved and arthritis subsided.

  Discussion Top

Although patients with anti-ARS antibodies share common clinical features, there is a degree of heterogeneity within ASS. It is not uncommon for these patients to present with a single clinical feature, thus receiving a different diagnosis (idiopathic ILD and rheumatoid arthritis) in the beginning. The clinical presentation and disease evolution in these patients depend on the type of anti-ARS antibodies.[1]

PubMed search with terms “anti-OJ,” “anti-isoleucyl-tRNA synthetase,” “myositis AND anti-OJ,” and “antisynthetase syndrome AND anti-OJ” identified 69 patients of ASS with anti-OJ antibodies published in English literature, with adequate clinical data.[4],[5],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19] Clinical characteristics of these patients including our patient are summarized in [Table 1]. Cavagna et al.[4] described the clinical features of 828 ASS patients from the American and European NEtwork of ASS Collaborative Group Cohort. Eighteen patients (2%) had anti-OJ antibodies. Incomplete ASS (one/two triad features: arthritis, myositis, and ILD) was seen in 17/18 patients, and seven of these developed all triad features at a median of 12 months (interquartile range 3–13) after the disease onset. The prevalence of arthritis, myositis, and ILD, at the disease onset, in these 18 patients was 41%, 50%, and 44%, respectively, whereas it was 33%, 42%, and 70%, when derived from the available data of previously reported cases including our patient [Table 1]. One or more additional clinical features (fever [6%]; MH [39%]); and RP [22%]) were seen in 9/18 (50%) patients. The most common presentation pattern was isolated arthritis in anti-Jo-1; isolated ILD in anti-PL-7, anti-PL-12, and anti-EJ; and isolated myositis in anti-OJ patients. In contrast to this, the data from [Table 1] show that isolated ILD (24%) was the most common presentation followed by myositis with ILD (16%), isolated arthritis (15%), and isolated myositis (13%). The subset of patients presenting with isolated ILD are important to recognize, as early diagnosis of ASS-associated ILD is associated with a better prognosis than idiopathic pulmonary fibrosis.[1]
Table 1: Clinical characteristics of the patients with anti-OJ antibodies

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Hamaguchi et al.[5] described 165 patients with anti-ARS antibodies, of which eight (5%) had anti-OJ antibodies. Arthritis was most common in anti-Jo-1 group (58%) and infrequently observed in anti-OJ patients (13%). Patients with anti-Jo-1, anti-PL-7, and anti-EJ had a higher frequency of muscle weakness (78%, 76%, and 55%, respectively) than the anti-OJ group (25%). However, the prevalence of myositis was 100% in 14 patients reported by Nagochi et al.[14] The overall prevalence of arthritis, myositis, and ILD in seventy patients with anti-OJ antibodies was 44%, 77%, and 83%, respectively [Table 1].

The cystic changes on HRCT [Figure 1b] in our patient were unusual for nonspecific interstitial pneumonia pattern and have not been described in ASS patients with anti-OJ antibodies. Such cystic changes usually suggest lymphoid interstitial pneumonia (LIP), a pattern commonly encountered in Sjögren's syndrome (SS).[20] Although SS was ruled out in our patient based on the absence of anti-SSA/SSB antibodies and clinical features, the possibility of LIP cannot be completely excluded without histopathological examination of the lung tissue.

The mechanisms responsible for these differences in clinical features and disease course associated with each of the anti-ARS autoantibodies are largely unknown. Further observations from large number of cases are required to explore the association of anti-OJ antibodies with clinical features of ASS.

Consent for publication

Written informed consent for publication was obtained from the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

McHugh NJ, Tansley SL. Autoantibodies in myositis. Nat Rev Rheumatol 2018;14:290-302.  Back to cited text no. 1
Connors GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: What progress has been made in the past 35 years? Chest 2010;138:1464-74.  Back to cited text no. 2
Solomon J, Swigris JJ, Brown KK. Myositis-related interstitial lung disease and antisynthetase syndrome. J Bras Pneumol 2011;37:100-9.  Back to cited text no. 3
Cavagna L, Trallero-Araguás E, Meloni F, Cavazzana I, Rojas-Serrano J, Feist E, et al. Influence of antisynthetase antibodies specificities on antisynthetase syndrome clinical spectrum time course. J Clin Med 2019;8:2013.  Back to cited text no. 4
Hamaguchi Y, Fujimoto M, Matsushita T, Kaji K, Komura K, Hasegawa M, et al. Common and distinct clinical features in adult patients with anti-aminoacyl-tRNA synthetase antibodies: Heterogeneity within the syndrome. PLoS One 2013;8:e60442.  Back to cited text no. 5
Gupta L, Gaur P, Agarwal V, Aggarwal R, Misra R. SAT0323 AT0323 Myositis specific and myositis-associated autoantibodies in aIndian cohort of inflammatory myositis reveal novel clinico-phenotypic patterns. Ann Rheum Dis 2020;79:1107.  Back to cited text no. 6
Targoff IN, Trieu EP, Miller FW. Reaction of anti-OJ autoantibodies with components of the multi-enzyme complex of aminoacyl-tRNA synthetases in addition to isoleucyl-tRNA synthetase. J Clin Invest 1993;91:2556-64.  Back to cited text no. 7
Sato S, Kuwana M, Hirakata M. Clinical characteristics of Japanese patients with anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibodies. Rheumatology (Oxford) 2007;46:842-5.  Back to cited text no. 8
Friedman AW, Targoff IN, Arnett FC. Interstitial lung disease with autoantibodies against aminoacyl-tRNA synthetases in the absence of clinically apparent myositis. Semin Arthritis Rheum 1996;26:459-67.  Back to cited text no. 9
Kunimasa K, Arita M, Nakazawa T, Tanaka M, Tsubouchi K, Konishi S, et al. The clinical characteristics of two anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibody-positive interstitial lung disease patients with polymyositis/dermatomyositis. Intern Med 2012;51:3405-10.  Back to cited text no. 10
Johnson C, Connors GR, Oaks J, Han S, Truong A, Richardson B, et al. Clinical and pathologic differences in interstitial lung disease based on antisynthetase antibody type. Respir Med 2014;108:1542-8.  Back to cited text no. 11
Noda S, Asano Y, Tamaki Z, Hirabayashi M, Yamamoto M, Takekoshi T, et al. Dermatomyositis with anti-OJ antibody. Rheumatol Int 2011;31:1673-5.  Back to cited text no. 12
Hamada M, Tanaka I, Sakurai Y, Hosono Y, Mimori T. Juvenile polymyositis associated with anti-OJ (anti-isoleucyl-tRNA synthetase) autoantibody in a 13-year-old girl. Mod Rheumatol 2017;27:541-4.  Back to cited text no. 13
Koreeda Y, Higashimoto I, Yamamoto M, Takahashi M, Kaji K, Fujimoto M, et al. Clinical and pathological findings of interstitial lung disease patients with anti-aminoacyl-tRNA synthetase autoantibodies. Intern Med 2010;49:361-9.  Back to cited text no. 14
Hervier B, Lambert M, Hachulla E, Musset L, Benveniste O, Piette JC, et al. Anti-synthetase syndrome positive for anti-isoleucyl-tRNA synthetase antibodies: An unusual case overlapping with systemic sclerosis and Sjogren's syndrome. Rheumatology (Oxford) 2011;50:1175-6.  Back to cited text no. 15
Ohosone Y, Ishida M, Takahashi Y, Matsumura M, Hirakata M, Kawahara Y, et al. Spectrum and clinical significance of autoantibodies against transfer RNA. Arthritis Rheum 1998;41:1625-31.  Back to cited text no. 16
Kapoor A, Vaidyan P, Jalil B, Upaluri C. Novel case of anti-synthetase syndrome. Eur J Rheumatol 2018;5:275-7.  Back to cited text no. 17
Gelpí C, Kanterewicz E, Gratacos J, Targoff IN, Rodríguez-Sánchez JL. Coexistence of two antisynthetases in a patient with the antisynthetase syndrome. Arthritis Rheum 1996;39:692-7.  Back to cited text no. 18
Noguchi E, Uruha A, Suzuki S, Hamanaka K, Ohnuki Y, Tsugawa J, et al. Skeletal muscle involvement in antisynthetase syndrome. JAMA Neurol 2017;74:992-9.  Back to cited text no. 19
Ferguson EC, Berkowitz EA. Lung CT: Part 2, the interstitial pneumonias – Clinical, histologic, and CT manifestations. AJR Am J Roentgenol 2012;199:W464-76.  Back to cited text no. 20


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