Clinical spectrum time course of interstitial pneumonia with autoimmune features in patients positive for antisynthetase antibodies.

Abstract

•Classification criteria for antisyntehase syndrome (ASSD) are lacking.•Lung involvement is common in antisynthetase Ab (anti-ARS) positive patients.•Several patients diagnosed with ASSD satisfy IPAF classification criteria.•Clinical spectrum time course heterogeneity is common in anti-ARS positive patients.•The establishment of new classification criteria for ASSD is mandatory. Interstitial pneumonia with autoimmune features Connective tissue disease Antisynthetase antibody Antisynthetase syndrome Rheumatoid arthritis Idiopathic inflammatory myopathy undifferentiated polyarthritis We read with interest the paper by Chartrand et al. [[1]Chartrand S. Swigris J.J. Stanchev L. Lee J.S. Brown K.K. Fischer A. Clinical features and natural history of interstitial pneumonia with autoimmune features: a single center experience.Respir. Med. 2016; 119: 150-154http://dx.doi.org/10.1016/j.rmed.2016.09.002Abstract Full Text Full Text PDF PubMed Scopus (95) Google Scholar], describing a large series of patients affected by interstitial pneumonia with autoimmune features (IPAF). The proportion of IPAF patients developing an established connective tissue disease (CTD) was between the points of discussion suggested by authors. To this purpose, we want to report our clinical experience on antisynthetase antibodies (anti-ARS). These antibodies are markers of the so-called antisynthetase syndrome (ASSD), a CTD characterized by the occurrence of myositis, interstitial lung disease (ILD) and arthritis, but without established classification criteria. Recently, we collected and described the characteristics of a very large cohort of anti-ARS positive patients [2Cavagna L. Nuño L. Scirè C.A. Govoni M. Longo F.J. Franceschini F. et al.Clinical spectrum time course in anti Jo-1 positive antisynthetase syndrome: results from an international retrospective multicenter study.Med. Baltim. 2015; 94: e1144http://dx.doi.org/10.1097/MD.0000000000001144Crossref PubMed Scopus (110) Google Scholar, 3Trallero-Araguás E. Grau-Junyent J.M. Labirua-Iturburu A. García-Hernández F.J. Monteagudo-Jiménez M. Fraile-Rodriguez G. et al.Clinical manifestations and long-term outcome of anti-Jo1 antisynthetase patients in a large cohort of Spanish patients from the GEAS-IIM group.Semin. Arthritis Rheum. 2016; 46: 225-231http://dx.doi.org/10.1016/j.semarthrit.2016.03.011Crossref PubMed Scopus (66) Google Scholar, 4Cavagna L. Nuño L. Scirè C.A. Govoni M. Longo F.J. Franceschini F. et al.Serum Jo-1 autoantibody and isolated arthritis in the antisynthetase syndrome: review of the literature and report of the experience of AENEAS collaborative group.Clin. Rev. Allergy Immunol. 2017; 52: 71-80http://dx.doi.org/10.1007/s12016-016-8528-9Crossref PubMed Scopus (52) Google Scholar, 5Bartoloni E. Gonzalez-Gay M.A. Scirè C. Castaneda S. Gerli R. Lopez-Longo F.J. et al.Clinical follow-up predictors of disease pattern change in anti-Jo1 positive anti-synthetase syndrome: results from a multicenter, international and retrospective study.Autoimmun. Rev. 2017; 16: 253-257http://dx.doi.org/10.1016/j.autrev.2017.01.008Crossref PubMed Scopus (40) Google Scholar]. In our shared casuistry, 146 (21%) out of the 684 included patients would have been classified as IPAF [[5]Bartoloni E. Gonzalez-Gay M.A. Scirè C. Castaneda S. Gerli R. Lopez-Longo F.J. et al.Clinical follow-up predictors of disease pattern change in anti-Jo1 positive anti-synthetase syndrome: results from a multicenter, international and retrospective study.Autoimmun. Rev. 2017; 16: 253-257http://dx.doi.org/10.1016/j.autrev.2017.01.008Crossref PubMed Scopus (40) Google Scholar]. Anti Jo-1 was the most commonly detected anti-ARS antibody (n = 81, 55%), followed by anti PL-12 (n = 33, 23%), PL-7 (n = 23, 16%), EJ (n = 7, 5%), and OJ (n = 2, 1%). ANA test was positive in 79 (54%) patients and anti Ro52 KD in 75 (51%). IPAF would have been the first possible diagnosis in 132 (90%) cases. At the end of the follow-up, 85 (58%) patients would have still been classified as IPAF, whereas the remaining 61 (42%) patients had other diagnosis (Table 1). The median progression time from IPAF to another CTD was 12 months (Interquartile range: 5–19). In these patients, the median follow-up was 58 months (IQR 21–87) whereas in IPAF without subsequent diagnosis, the median follow-up was 41 months (IQR 27.5–89) (p = 0.464, Whelch-test for unequal variances). In case of anti ARS positivity, IPAF is generally a transient condition that may progress into another well-defined rheumatic disease. Our result highlight the need of newly established and shared classification criteria for ASSD.Table 1Diagnosis' evolution in patients that could be classified as IPAF. In bold, the final diagnosis.First diagnosisSecond diagnosisThird diagnosisNumber (%)IPAF75 (51.5)IPAFIIM37 (25.5)IPAFIIMIIM and RA (overlap)1 (1)IPAFRA7 (4.5)IPAFRA and IIM (overlap)8 (5.5)IPAFRARA and IIM (overlap)4 (2.5)UPAIPAF10 (7)UPAIPAFIIM4 (2.5)146 (100) Total patientsIPAF = interstitial pneumonia with autoimmune features, RA = Rheumatoid arthritis, IIM = idiopathic inflammatory myopathy, UPA = undifferentiated polyarthritis. Open table in a new tab IPAF = interstitial pneumonia with autoimmune features, RA = Rheumatoid arthritis, IIM = idiopathic inflammatory myopathy, UPA = undifferentiated polyarthritis. To all members of the AENEAS collaborative group. Clinical features and natural history of interstitial pneumonia with autoimmune features: A single center experienceRespiratory MedicineVol. 119PreviewTo describe the clinical phenotype and natural history of a cohort of patients with interstitial pneumonia with autoimmune features (IPAF). Full-Text PDF Open Archive