THU0614 Interstitial lung disease in patients with antisynthetase syndrome and anti-ro52 antibodies positive

Abstract

BackgroundAntisynthetase syndrome (ASS) is characterised by the presence of myositis, arthritis, interstitial lung disease (ILD), fever, Raynaud’s phenomenon and mechanical’s hand, in the presence of antisynthetase autoantibodies (AA), the most frequent being anti-Jo1, anti-PL7 and anti-PL12. An association between ASS and anti-Ro52 with increased ILD has been described and it is believed that the presence of both antibodies is accompanied by a more severe ILD.ObjectivesTo describe the clinical and analytical characteristics of a cohort of patients with ASS. To analyse the lung involvement in this type of patient and to determine the possible relationship between the different subtypes of ILD and the presence of anti-Ro52.MethodsRetrospective descriptive study of patients treated in our Hospital (2006–2017), with AA and at least 2 clinical characteristics. The data was obtained through the review of medical records.Variables analysed:age, sex, age, smoking, clinical presentation, diagnosis of ASS, associated neoplasia and paraneoplastic syndrome (PS)(neoplasia 3 years before or after the diagnosis of ASS), muscle enzymes (CK and aldolase), autoimmunity, glucocorticoids (GC), immunosuppressants (IS), diagnosis of ILD, HRCT pattern (High Resolution Computed Tomography) and respiratory function tests (RFT) at the beginning of ILD.ResultsWe included 27 patients (20 women), mean age 61±13 years. 7.4% smokers and 18.5% ex-smokers. 88.8% were anti-Jo1,7.4% anti-PL12% and 3.7% anti-PL7. Anti-Ro52 present in 18 patients. The most common clinical presentation:ILD 88% (59% had Ro52), followed by myositis 85% (40% are dermatomyositis), arthritis 81%, mechanic’s hand 51%, fever 37% and Raynaud’s phenomenon 25%. The classic triad (arthritis, myositis, ILD) was present in 16 patients. Three patients presented neoplasia in the course of the disease, being identified as PS. Elevation of CK in 70% and aldolase in 74%. 96% of patients have been treated with GC and IS.The HRCT patterns were:non-specific interstitial pneumonia (NSIP)(66%), usual interstitial pneumonia (UIP) (29%),organised cryptogenic pneumonia (OP)(4%), baseline RFT were performed in 19 patients. Diagnosis of ASS and ILD, both entities appear at the same time in 6 patients, in 3 patients the ILD appears before and in 14 after. In these, the median duration (range) of the ASS until the diagnosis of ILD was 1 year (0–1).There is no relationship between the HRCT and anti-Ro52 patterns (chi-square considering the exact distribution p=0.892), nor between the ILD and anti-Ro52 (Fisher exact test p=0.999).ConclusionsOur results, in general, agree with what is published in the literature. Three patients have an uncommon presentation of ASS, with a diagnosis of ILD prior to myopathy (in most of the published cases, myositis precedes or coincides with the onset of IDL), and it is important to include ASS in the differential diagnosis of ILD. In our cohort, the association between ILD and anti-Ro52 has not been demonstrated, nor among the different subtypes of ILD to Ro52. Ther...

Defining anti-synthetase syndrome: a systematic literature review

“…The remaining 19 studies required one of the following features: myositis, ILD, arthritis, fever, skin rash or mechanic's hands, and RP (12,(76)(77)(78)(79)(80)(81)(82)(83)(84)(85)(86)(87)(88)(89)(90)(91)(92)(93). Among the remaining definitions, 16 studies defined ASSD as the presence of more than one variable associated with anti-ARS positivity (11,12,(94)(95)(96)(97)(98)(99)(100)(101)(102)(103)(104)(105)(106)(107). In two studies, ASSD was defined as having a connective tissue disease (CTD) diagnosis and simultaneous positive anti-ARS (3,108).…”

“…Frequency of variables used to define anti-synthetase syndrome. ,32,84,85,88,94,95,[97][98][99][100]104) and fever (7 studies)(12,85,88,(94)(95)(96)98). TableIIsummarises the frequencies of use of each variable.…”