Anti–Jo-1 Syndrome Often Misdiagnosed as Rheumatoid Arthritis (for Many Years)

Kumar, Rajiv; Jha, Saket; Dhooria, Aadhaar; Naidu, Gsrsnk; Minz, Ranzana W; Kumar, Susheel; Sharma, Shefali; Sharma, Aman; Jain, Sanjay; Dhir, Varun

doi:10.1097/rhu.0000000000001234

Cited by 15 publications

(22 citation statements)

References 21 publications

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“…The prevalence of ILD in most cohorts, including ours, was ∼75% [ 10 , 11 , 16 ]. Non-specific interstitial pneumonia, as in other studies, was the commonest pattern present on high-resolution CT in our study [ 9 , 13 , 16 , 17 ]. Although the two RP-ILD cases in our cohort were anti-Jo-1 positive, Shi et al [ 9 ] reported a significant association with anti-PL-7 antibodies and mortality of ∼9%.…”

Section: Discussionsupporting

confidence: 88%

“…Rojas-Serrano et al [ 12 ] reported 43 Mexican ASS patients, and Shi et al [ 9 ] described 124 Chinese patients with 18 and 22 years of follow-up, respectively. The cohorts that focused on anti-Jo-1-positive patients were the European American AENEAS group (58 patients), the cohort described by Kumar et al from India (27 patients), and the Spanish group by Trallero-Araguás et al (148 patients) [ 1 , 13 , 14 ]. The study by Srivastava et al [ 15 ] on the prevalence of myositis-specific and -associated antibodies in a North Indian population reported 29 ASS patients, of whom 14 were Jo-1 positive.…”

Section: Discussionmentioning

confidence: 99%

“…Antisynthetase syndrome may present as arthritis [ 13 , 18 ]. We reported a very high incidence of isolated arthritis at presentation (42%) compared with three previous reports (21, 25 and 21%) [ 10 , 12 , 18 ].…”

Section: Discussionmentioning

confidence: 99%

“…A very high percentage of seronegative arthritis was reported by Lefevre et al [ 18 ], the AENEAS cohort [ 19 ], and our study (87, 73 and 86%, respectively). Kumar et al [ 13 ], however, found a much higher (71.4%) seropositivity, probably because they had focused on patients misdiagnosed as RA. The distal subluxating arthropathy of the hand seen in one of our patients had been described in case reports of ASS [ 20 , 21 ].…”

Section: Discussionmentioning

confidence: 99%

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Clinical profile and treatment outcomes in antisynthetase syndrome: a tertiary centre experience

Sreevilasan¹,

Devarasetti²,

Narahari

et al. 2021

Rheumatology Advances in Practice

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Objectives The aim was to describe the clinical profile and outcomes in patients with antisynthetase syndrome (ASS) from a tertiary care centre. Methods The clinical data and investigations of all patients classified as ASS by Connors criteria over 5 years were recorded, and they were followed up prospectively. The median (interquartile range) was used for descriptive statistics. Clinical variables between the Jo-1 and non-Jo-1 groups and between patients with and without anti-Ro52 antibodies were compared using the χ2 test. Survival analysis was done using the log rank test. Results The 28 patients (23 females) had a median age of 42.5 (34.8–52.3) years, with a disease duration of 1.75 (0.6–3.8) years at diagnosis, and had a follow-up of 2 (0.25–4.25) years. Seronegative arthritis was seen in 23 of 28 patients. Non-specific interstitial pneumonia was seen in 19 patients with interstitial lung disease (ILD). Antibodies to Jo-1 (n = 17) were more frequent than non-Jo-1 antibodies (n = 11; five anti-PL-12, four anti-PL-7 and two anti-EJ). There was no significant difference in the prevalence of myositis (P = 0.07) or ILD (P = 0.11) between groups. Anti-Ro52 antibodies were more frequently found in the non-Jo-1 group (P = 0.006, ϕ = 0.51). A partial or complete improvement with treatment was seen in three-quarters of the patients. Five patients succumbed to the illness. Better survival was seen in the Jo-1 group (P = 0.05). Conclusion The most typical presenting manifestation of ASS in our cohort was isolated seronegative arthritis. Non-specific interstitial pneumonia was the commonest ILD pattern. Patients with antibodies to Jo-1 had better survival compared with non-Jo-1. The non-Jo-1 aminoacyl-transfer RNA synthetases had a strong association with anti-Ro52 antibodies.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Clinical profile and treatment outcomes in antisynthetase syndrome: a tertiary centre experience

Sreevilasan¹,

Devarasetti²,

Narahari

et al. 2021

Rheumatology Advances in Practice

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“…Hervier and Lambert et al reported an interesting rare case of overlap syndrome with PL-7 ASS, Sjogren's, systemic lupus erythematosus and RA with evolving symptoms over time. 8,[13][14][15] Anti-synthetase syndrome has often been reported in overlap with polymyositis, dermatomyositis, systemic sclerosis, Sjogren's and RA.…”

Section: Discussionmentioning

confidence: 99%

The anti-synthetase syndrome in a type 1 diabetes

Sajjan¹,

Pahuja²,

Ponvijaya³

et al. 2020

Int J Res Med Sci

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Anti-synthetase syndrome is an autoimmune disease associated with interstitial lung disease (ILD), dermatomyositis and polymyositis. It has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with dermatomyositis. 37-year-old male, known case of type 1 diabetes mellitus, came with complaints of: generalized anasarca, pain in both knees, ankles, wrist and small joints of fingers. He also had dyspnoea on exertion, chronic non-productive cough, and fever off and on, all for 1 month. Initially all involvement was attributed to diabetes. For joint pain an antinuclear antibodies (ANA) was sent. He turned out to be anti-Jo1, antibody positive. Rash on hands was diagnosed by dermatologist as, mechanic’s hand, hence diagnosed as an inflammatory myopathy with dermatomyositis anti-synthetase syndrome. Patient was successfully treated with immunosuppressants and supportive treatment and responded to tablet Prednisolone and Mycophenolate mofetil. The patient had one major and 2 minor criteria-ILD, arthritis and Mechanic’s hand and anti-Jo 1 antibody positive. Thus, diagnosed as anti-synthetase syndrome with type 1 diabetes mellitus.

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Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

Zhang,

Wang,

Zhou

et al. 2023

Immunity Inflam & Disease

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ObjectivesTo identify the differences of clinical characteristics, laboratory findings, and the long‐term outcomes in patients with anti‐synthetase syndrome (ASS) of different anti‐aminoacyl‐transfer RNA synthetase antibodies.MethodsWe retrospectively enrolled 119 patients with ASS, and the clinical characteristics and laboratory findings were collected. Additionally, multivariate COX regression analysis was performed to estimate the risk factors of prognosis in patients with ASS.ResultsThe frequency of interstitial lung disease (ILD) reached 93.3% in our cohort, of 28 (23.5%) was classified as rapidly progressive (RP)‐ILD. The highest incidence of RP‐ILD was 36.4% in the PL12 group of ASS patients. The ILD group was characterized by an older age, a lower prevalence of V sign, and a higher prevalence of pulmonary symptoms when contrasted with the non‐ILD group. There were statistical differences of clinical significance in arthritis, myositis, mechanic's hands, triad, shawl sign, V sign, and Raynaud's phenomenon among the four subgroups (all p < .05). Additionally, the prevalence rates of arthritis, myositis, mechanic's hands, triad, and V sign in the anti‐Jo1 antibody‐positive group were significantly higher than anti‐Jo1 antibody‐negative patients with ASS (all p < .05). Multivariate Cox regression analysis showed mechanic's hands (odds ratio [OR] = 6.47, p < .001), anti‐nuclear antibodies (ANA) (OR = 2.13, p = .026), ILD (OR = 10.50, p < .001), and V sign (OR = 0.30, p = .007) were independent factors affecting the prognosis of patients with ASS. The incidences of RP‐ILD, arthritis, myositis, triad, mechanic's hands, and shawl sign were more frequent in the anti‐Ro52 antibody‐positive group than the anti‐Ro52 antibody‐negative patients with ASS (all p < .05).ConclusionsPatients with ASS accompanied with ILD are highly prevalent. Mechanic's hands, ANA, and ILD may be a potential biomarker for predicting a poor prognosis in patients with ASS. Additionally, the detection of the anti‐Ro52 antibody provides valuable insights for managing and predicting disease progression and long‐term outcomes.

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Anti–Jo-1 Syndrome Often Misdiagnosed as Rheumatoid Arthritis (for Many Years)

Cited by 15 publications

References 21 publications

Clinical profile and treatment outcomes in antisynthetase syndrome: a tertiary centre experience

Clinical profile and treatment outcomes in antisynthetase syndrome: a tertiary centre experience

The anti-synthetase syndrome in a type 1 diabetes

Clinical characteristics and prognostic analysis of idiopathic inflammatory myopathy with positive anti‐aminoacyl‐tRNA synthetase antibodies: A single center experience

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