S. Josse

Comparison of long-term outcome between anti-Jo1- and anti-PL7/PL12 positive patients with antisynthetase syndrome.

The aims of the present study were to: compare the characteristics between antisynthetase syndrome (ASS) patients with anti-Jo1 antibody and those with anti-PL7/PL12 antibody. The medical records of 95 consecutive patients with ASS were reviewed. Seventy-five of these patients had anti-Jo1 antibody; the other patients had anti-PL7 (n=15) or anti-PL12 (n=5) antibody. At ASS diagnosis, the prevalence of myalgia (p=0.007) and muscle weakness (p=0.02) was significantly lower in the group of anti-PL7/PL12-positive patients than in those with anti-Jo1 antibody; median value of CK (p=0.00003) was also lower in anti-PL7/PL12 patients. Anti-Jo1 positive patients developed more rarely myositis resolution (21.3% vs. 46.2%); in addition, the overall recurrence rate of myositis was higher in anti-Jo1 positive patients than in patients with anti-PL7/PL12 antibody (65.9% vs. 19.4%). Anti-Jo1-positive patients, compared with those with anti-PL7/PL12 antibody, more often experienced: joint involvement (63.3%vs. 40%) and cancer (13.3% vs. 5%). By contrast, anti-PL7/PL12 positive patients, compared with those with anti-Jo1 antibody, more commonly exhibited: ILD (90% vs. 68%); in anti-PL7/PL12 positive patients, ILD was more often symptomatic at diagnosis, and led more rarely to resolution of lung manifestations (5.6% vs. 29.4%). Finally, the group of anti-PL7/PL12 positive patients more commonly experienced gastrointestinal manifestations related to ASS (p=0.02). Taken together, although anti-Jo1 positive patients with ASS share some features with those with anti-PL7/PL12 antibody, they exhibit many differences regarding clinical phenotype and long-term outcome. Our study underscores that the presence of anti-Jo1 antibody results in more severe myositis, joint impairment and increased risk of cancer. On the other hand, the presence of anti-PL7/PL12 antibody is markedly associated with: early and severe ILD, and gastrointestinal complications. Thus, our study interestingly indicates that the finding for anti-Jo1 and anti-PL7/PL12 antibodies impacts both the long-term outcome and prognosis of patients with ASS.

Interstitial Lung Disease in Anti–Jo‐1 Patients With Antisynthetase Syndrome

To assess the outcome of interstitial lung disease (ILD) in anti–Jo‐1 patients with antisynthetase syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti–Jo‐1 patients with and without ILD.

Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome.

BACKGROUND The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS). METHODS The medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. RESULTS Anti-PL7 patients exhibited polymyositis (n=14) and dermatomyositis (n=1); extra-pulmonary manifestations of ASS included: Raynauds phenomenon (40%), mechanics hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n=5), was identified concomitantly with ASS (n=8) and occurred after ASS diagnosis (n=1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n=1), progressive onset of lung signs (n=11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n=2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO<45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP). CONCLUSION Our series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO<45% at ILD diagnosis and UIP pattern on HRCT-scan.

Sustained response to infliximab in a patient with relapsing polychondritis with aortic involvement

Sustained response to infliximab in a patient with relapsing polychondritis with aortic involvement SIR, Relapsing polychondritis (RP) is a systemic disorder, which is characterized by recurrent inflammation and destruction of cartilage structure [1]. Auricular, nasal, ocular, tracheobronchial and articular impairment are well-recognized manifestations of RP [1]. Aortic involvement is considered to be rare, and usually occurs during the course of the disease [2–3]. We recently observed a new case, which is of particular interest as the patient developed aortic involvement, revealing a recurrence of refractory RP, with favourable outcome after initiation of infliximab. A 38-year-old woman presented in July 2005 with a 2-month history of asthenia and abdominal pain. She was diagnosed as having RP in March 2004 because of the following manifestations: bilateral auricle chondritis, nasal chondritis, hoarseness of the voice and ocular inflammation. The patient was given prednisone (at an initial dose of 1 mg/kg daily), resulting in improvement of clinical symptoms of RP. In May 2004, the patient received MTX (20 mg weekly); she developed hepatic cytolysis, resulting in MTX discontinuation. In June 2004, the patient further received AZA (150 mg daily). When steroid regimen was reduced (7.5 mg/day), the patient relapsed in July 2005. Indeed, she experienced bilateral auricle chondritis, nasal chondritis, hoarseness of the voice and ocular inflammation; she also complained concomitantly of abdominal pain. At admission, the patient had no fever; physical examination also revealed tender abdomen on palpation. 9 /l; findings of renal and liver tests as well as blood electrophoresis were normal. CT scan of both lungs and abdomen showed circumferential thickening of the abdominal aortic wall. Blood cultures and bacterial serologies (especially Treponema pallidum) were negative; autoantibody screening was also negative for RFs, ANAs and anti-cytoplasmic antibodies. The diagnosis of RP aortic involvement was conducted. The patient was given combined therapy of prednisone (1 mg/kg daily) and pulses of cyclophosphamide (0.7 g/m 2 , monthly). Four months later, the patient still exhibited severe ocular inflammation and abdominal pain. CT scan demonstrated aortic aneurysm located on the abdominal aorta and circumfer-ential thickening of the abdominal aortic wall (Figs 1 and 2). Because RP was refractory to steroid/immunosuppressive therapy, the patient was given anti-TNF-: infliximab (5 mg/kg) at weeks 0, 2, 6 and then 8 weekly. Infliximab therapy resulted in resolution of ocular inflammation; repeated CT scan showed improvement of aortic impairment. At 3-year follow-up, the patient is symptom free, receiving prednisone (3 …