Seronegative polyarthritis revealing antisynthetase syndrome: a multicentre study of 40 patients

Lefèvre, Guillaume; Meyer, Alain; Launay, David; Machelart, Irène; Debandt, M.; Michaud, Julien; Tournadre, Anne; Godmer, Pascal; Kahn, Jean‐Emmanuel; Behra-Marsac, A.; Timsit, Marie-Agnès; Schleinitz, N.; Wendling, Daniel; Melac-Ducamp, Sylvie; Boyer, Philippe; Peretz, Anne; Lequerré, Thierry; Richez, Christophe; Stervinou-Wémeau, L.; Morell-Dubois, Sandrine; Lambert, Marc; Dubucquoi, Sylvain; Wallaert, B.; Benveniste, Olivier; Flipo, René‐Marc; Hatron, Pierre‐Yves; Sibilia, Jean; Hachulla, É.; Hervier, B.

doi:10.1093/rheumatology/keu404

Cited by 42 publications

(38 citation statements)

References 19 publications

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“…The positivity of IgM-RF and ACPA was associated with the occurrence of typical RA erosions, confirming previous observations from other series of anti Jo-1 ASSD patients 13 , 14 , 23 and in other connective tissue diseases. 16 , 17 This finding is of great relevance because arthritis is frequently the only classic triad finding at disease onset, 6 and, because of that, some patients may be misclassified as having RA. Furthermore, we observed that the rate of clinical picture progression in patients presenting only with arthritis is as high as 90% of cases.…”

Section: Discussionmentioning

confidence: 99%

“… 2 , 5 – 10 In these patients, the clinical picture may evolve during follow-up. 6 Furthermore, ASSD is characterized by a large heterogeneity in the severity of clinical findings, 5 , 11 , 12 in particular, for joint involvement, ranging from simple polyarthralgias, 5 to a symmetrical polyarthritis, 6 and that may be also seropositive, 13 , 14 for both Ig-M rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (ACPA).…”

Section: Introductionmentioning

confidence: 99%

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Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome

et al. 2015

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Anti Jo-1 antibodies are the main markers of the antisynthetase syndrome (ASSD), an autoimmune disease clinically characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). These manifestations usually co-occur (for practical purpose complete forms) in the same patient, but cases with only 1 or 2 of these findings (for practical purpose incomplete forms) have been described. In incomplete forms, the ex novo occurrence of further manifestations is possible, although with frequencies and timing not still defined. The aim of this international, multicenter, retrospective study was to characterize the clinical time course of anti Jo-1 positive ASSD in a large cohort of patients. Included patients should be anti Jo-1 positive and with at least 1 feature between arthritis, myositis, and ILD. We evaluated the differences between complete and incomplete forms, timing of clinical picture appearance and analyzed factors predicting the appearance of further manifestations in incomplete ASSD. Finally, we collected 225 patients (58 males and 167 females) with a median follow-up of 80 months. At the onset, complete ASSD were 44 and incomplete 181. Patients with incomplete ASSD had frequently only 1 of the classic triad findings (110 cases), in particular, isolated arthritis in 54 cases, isolated myositis in 28 cases, and isolated ILD in 28 cases. At the end of follow-up, complete ASSD were 113, incomplete 112. Only 5 patients had an isolated arthritis, only 5 an isolated myositis, and 15 an isolated ILD. During the follow-up, 108 patients with incomplete forms developed further manifestations. Single main feature onset was the main risk factor for the ex novo appearance of further manifestation. ILD was the prevalent ex novo manifestation (74 cases). In conclusion, ASSD is a condition that should be carefully considered in all patients presenting with arthritis, myositis, and ILD, even when isolated. The ex novo appearance of further manifestations in patients with incomplete forms is common, thus indicating the need for an adequate clinical and instrumental follow-up. Furthermore, the study clearly suggested that in ASSD multidisciplinary approach involving Rheumatology, Neurology, Pneumology, and Internal Medicine specialists is mandatory.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome

et al. 2015

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“…In a large series of Japanese patients positive for anti-Jo-1, anti-EJ, anti-PL-7, anti-PL-12, anti-KS or anti-OJ highlighted the degree of heterogeneity within ASS in terms of distribution and onset of myositis, lung disease and skin lesions [33]. Furthermore, non-erosive inflammatory arthritis may be the presenting clinical feature in around one third of ASS patients, with or followed by ILD [34]. It now seems clear that as we will evaluate the degree of myositis and dermatological findings, we need to concentrate on different presentations of interstitial pneumonia and arthropathy to identify patients and thus not delay diagnosis.…”

Section: Anti-synthetase Syndromementioning

confidence: 99%

The Clinical Features of Myositis-Associated Autoantibodies: a Review

Gunawardena

2015

Clinic Rev Allerg Immunol

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The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases traditionally defined by clinical manifestations including skeletal muscle weakness, skin rashes, elevated skeletal muscle enzymes, and neurophysiological and/or histological evidence of muscle inflammation. Patients with myositis overlap can develop other features including parenchymal lung disease, inflammatory arthritis, gastrointestinal manifestations and marked constitutional symptoms. Although patients may be diagnosed as having polymyositis (PM) or dermatomyositis (DM) under the IIM spectrum, it is quite clear that disease course between subgroups of patients is different. For example, interstitial lung disease may predominate in some, whereas cutaneous complications, cancer risk, or severe refractory myopathy may be a significant feature in others. Therefore, tools that facilitate diagnosis and indicate which patients require more detailed investigation for disease complications are invaluable in clinical practice. The expanding field of autoantibodies (autoAbs) associated with connective tissue disease (CTD)-myositis overlap has generated considerable interest over the last few years. Using an immunological diagnostic approach, this group of heterogeneous conditions can be separated into a number of distinct clinical phenotypes. Rather than diagnose a patient as simply having PM, DM or overlap CTD, we can define syndromes to differentiate disease subsets that emphasise clinical outcomes and guide management. There are now over 15 CTD-myositis overlap autoAbs found in patients with a range of clinical manifestations including interstitial pneumonia, cutaneous disease, cancer-associated myositis and autoimmune-mediated necrotising myopathy. This review describes their diagnostic utility, potential role in disease monitoring and response to treatment. In the future, routine use of these autoAb will allow a stratified approach to managing this complex set of conditions.

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“…Some consider the diagnosis of AS only when two consecutive tests for anti-ARS antibody are positive, whereas others diagnose AS even when a single test is positive. [ 3 13 ] The three major clinical criteria for the diagnosis of AS include ILD, myositis and arthritis. Presence of any one major criteria is sufficient to make the diagnosis of AS although inclusion of inflammatory arthritis as a major criteria is debatable, and some consider it as a minor criterion.…”

Section: Discussionmentioning

confidence: 99%

“…These include patients presenting with ILD alone, with arthritis alone or as pyrexia of unknown origin. [ 3 4 ] Most cases of AS are either misdiagnosed as idiopathic ILD or inflammatory myopathy not only due to the lack of awareness of the disease entity but also due to the lack of facilities for detection of anti-ARS antibodies.…”

Section: Introductionmentioning

confidence: 99%

Antisynthetase syndrome: An under-recognized cause of interstitial lung disease

et al. 2016

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Background:Antisynthetase syndrome (AS) is an uncommon and under-recognised connective tissue disease characterized by the presence of antibodies to anti-aminoacyl t-RNA synthetase along with features of interstitial lung disease (ILD), myositis and arthritis. The aim of the current study is to describe our experience with management of AS.Materials and Methods:This was a 2-year (2013-2014) retrospective analysis of patients diagnosed with anti-Jo-1-related AS. The presence of anti-Jo-1 antibody was tested by the immunoblot assay. All patients underwent high-resolution computed tomography of the chest, transthoracic echocardiography and evaluation for inflammatory myositis. Transbronchial lung biopsies and muscle biopsies were obtained when clinically indicated.Results:Nine patients (mean age: 43.8 years) were diagnosed with anti-Jo-1-related AS. The median duration of symptoms before diagnosis of AS was 6 months. All patients were negative for antinuclear antibodies by indirect immunofluorescence. The prevalence of ILD, myositis and arthritis at presentation was 100%, 77.8% and 55.6%, respectively. The most common ILD pattern was non-specific interstitial pneumonia (n = 6) followed by organizing pneumonia (n = 2) and usual interstitial pneumonia (n = 1). ILD was the sole manifestation in two patients and was subclinical in two patients. Six patients had pleuropericardial effusions, three patients had pulmonary artery hypertension and two patients had venous thromboembolism. Eight of the nine patients improved after treatment with steroids and other immunosuppressants.Conclusion:Antisynthetase syndrome is an important and a treatable cause of ILD. Strong clinical suspicion is needed to achieve an early diagnosis.

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Seronegative polyarthritis revealing antisynthetase syndrome: a multicentre study of 40 patients

Abstract: ASS may be revealed by polyarthritis. To decrease the delay in diagnosis of ASS, pulmonary and muscle symptoms and anti-ARS antibodies might usefully be searched for in seronegative polyarthritis patients, especially in those with RP.

Cited by 42 publications

References 19 publications

Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome

Clinical Spectrum Time Course in Anti Jo-1 Positive Antisynthetase Syndrome

The Clinical Features of Myositis-Associated Autoantibodies: a Review

Antisynthetase syndrome: An under-recognized cause of interstitial lung disease

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