Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.


The Clinical Spectrum but Not the Evolution of Antisynthetase Syndrome Is Related to the Antisynthetase Antibody Specificity: A Retrospective Analysis of 142 Patients.

Hervier1,  Baptiste, Devilliers2,  Hervé, Stanciu1,  Raluca, Hachulla3,  Eric, Uzunhan4,  Yurdagul, Wallaert5,  Bruno, Fautrel6,  Bruno

Pitie-Salpetriere Hospital, APHP, UPMC Paris VI, Paris, France
Service de médecine interne, Hôpital Universitaire de Nantes, Nantes, France, Nantes, France
Pitie-Salpetriere Hospital, Paris, France
CHU Dijon, Dijon, France
Internal Medicine, Lille CEDEX, France
Hopital Avicenne, APHP, Bobigny, France
CHRU, Lille CEDEX, France
Université Pierre et Marie Curie - Paris 6 - Pitie Salpetriere University Hospital, Paris, France
CHU, Toulouse, France
CHU Pitié-Salpêtrière, Paris, France
CHU Toulouse Purpan, Toulouse, France

Background/Purpose:

Antisynthetase syndrome (ASS) is characterized by the association of an inflammatory myositis with interstitial lung disease (ILD) and different antisynthetase antibodies (AS-Ab). The clinical spectrum and the evolution of ASS are heterogeneous. This study was conducted to examine whether this heterogeneity would be related to the AS-Ab specificity.

Methods:

Patients (n=142, 47.8+/- 15.3 years at diagnosis) from 7 French tertiary hospitals from 1985–2011 were included. The association of >= 1 symptom with the positivity of 1 AS-Ab (anti-Jo1: n=85, anti-PL12: n=40 and anti-PL7: n=17) defined ASS. At the end of the follow-up (median = 3,4 years, 0–25), a worsening disease was defined either by an uncontrolled myositis or a pulmonary aggravation. The ASS patterns were examined by multiple correspondence & cluster analyses. Differences between worsening & stable/improving patients were tested by the Mann-Whitney (countinuous data) and the Khi-2 (categorical data) tests and defined the prognosis factors. A p value <.05 was significant.

Results:

Patients presented with myositis (n=100, 70%), ILD (n=125, 88%) and other symptoms, including arthralgia (n=79, 56%), Raynaud's phenomenon (n=57, 40%) and mechanic's hands (n=24, 17%). ASS overlapping with Systemic Sclerosis (SSc) and Sjögren Syndrome (SS) occurred in 41, 29% & 51, 36% cases respectively.

At diagnosis, myositis occurred more frequently in patients with anti-Jo1-Ab (85%) rather than anti-PL12/7-Ab (43%/41%, p<0,001). The myositis was more severe as attested by a higher frequency of muscular deficit (66% vs 20%/18%, p<.001) & myogenic electromyography pattern (n=40, p=0,002) and also a higher creatin-kinase (CK) level (5058 ± 5607 IU/L vs 452±576/1129±1529, p<.001). Patients with anti-PL12/7-Ab disclosed more frequently an ILD without myositis (50%/53 vs 11%, p<.001). The other ASS parameters were not correlated with the AS-Ab subtype.

Multiple correspondence analysis showed two different ASS patterns related to the AS-Ab specificity: patients with anti-Jo1-Ab presented a more diffuse disease with myositis, ILD, arthralgia and SS or SSc overlap, whereas patients with anti-PL12/7-Ab were quite similar to each other, disclosing a disease more restricted to the lungs. Moreover, the clustering analysis isolated a pattern of patients defined by the presence of anti-Jo1-Ab, severe myositis, ILD and arthralgia.

During the follow-up period, 36 patients (29%) developed a pulmonary hypertension (defined by a systolic PAP > 37 mmHg by echocardiography) and a total of 42 (30%) patients worsened. The evolution of the disease was not correlated to the AS-Ab specificity (p=.14). However, we identified different factors predictive of a worsening disease, including lung involvement (p<.02), co-occurrence of myositis & ILD at diagnosis (p<.04), severe rhabdomyolysis (p<.002) and development of pulmonary hypertension (p<.05).

Conclusion:

Based on the AS-Ab specificity, two different ASS patterns can be described: anti-Jo1-Ab pattern with severe myositis, ILD and arthralgia vs anti-PL12/7-Ab pattern with ILD but inconstant myositis. Prognosis factors, independent of AS-Ab specificity, were lung involvement, severe myositis and pulmonary hypertension

To cite this abstract, please use the following information:
Hervier, Baptiste, Devilliers, Hervé, Stanciu, Raluca, Hachulla, Eric, Uzunhan, Yurdagul, Wallaert, Bruno, et al; The Clinical Spectrum but Not the Evolution of Antisynthetase Syndrome Is Related to the Antisynthetase Antibody Specificity: A Retrospective Analysis of 142 Patients. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :230
DOI:

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