Objective. 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. Methods. Ninety-one anti-Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans. Results. Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n ‫؍‬ 12), progressive onset of lung signs (n ‫؍‬ 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n ‫؍‬ 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age >55 years. Furthermore, anti-Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels. Conclusion. Our findings confirm that ILD is a frequent complication in anti-Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti-Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.

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Our findings suggest that rituximab may be a helpful therapy for refractory interstitial lung disease in patients with ASS.

We conducted this retrospective study to determine the prevalence of giant cell arteritis (GCA) in patients exhibiting nonatherosclerotic upper and/or lower extremity arterial involvement and to evaluate the clinical features and long-term outcome of those patients.From January 1997 to March 2008, 36 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of symptomatic upper/lower extremity vasculitis related to GCA. In the 36 patients, upper/lower extremity vasculitis preceded the initial GCA diagnosis in 7 patients (19.4%), it was identified in association with GCA in 13 patients (36.1%), and it developed after the onset of GCA in the remaining 16 patients (44.4%). GCA clinical manifestations were severe resulting in ischemic complications of the extremities in 10 patients (27.8%). GCA-related large-vessel involvement was located in the upper extremity alone in 21 patients (58.3%), the lower extremity alone in 7 patients (19.4%), and both the upper and lower extremities in 8 patients (22.2%).Arterial involvement in GCA patients with upper extremity vasculitis was distributed in the subclavian (55.6%), axillary (47.2%), and brachial (22.2%) arteries. In patients with lower extremity vasculitis, involvement included the internal iliac artery (11.1%), common femoral artery (13.9%), superficial femoral artery (33.3%), deep femoral artery (5.6%), and popliteal and anterior tibial arteries (5.6%). Aortic localizations were common in GCA patients with upper/lower extremity vasculitis (68.9% of cases).All patients were given steroid therapy at a median daily dose of 1 mg/kg initially. Reconstructive study was performed in 10 patients (27.8%): venous bypass graft (n = 6), angioplasty (n = 1), thromboendarteriectomy (n = 2), or thrombectomy (n = 1); 2 other patients with extremity ischemia underwent amputation. The median observation time was 32 months; the outcome of upper/lower extremity vasculitis was disappearance of clinical symptoms (44.4%), improvement of clinical manifestations (44.4%), and deterioration of clinical manifestations (11.1%). At last follow-up, the median daily dose of prednisone was 6 mg. Steroid therapy could be discontinued in 12 patients (33.3%).We found that upper/lower extremity vasculitis is not uncommon in patients with GCA, and may be present in the early acute phase of GCA. Nevertheless, because upper/lower extremity vasculitis occurs during the course of GCA, yearly clinical vascular examinations may be adequate to screen for upper/lower extremity vasculitis at an early stage in GCA patients. Early diagnosis of GCA-related upper/lower extremity vasculitis is crucial, and can result in decreased severe ischemic complications. Because aortic localizations were common, GCA patients with upper/lower extremity vasculitis should undergo routine investigations for underlying life-threatening aortic complications (aortic ectasia/aneurysm). We also suggest that patients exhibiting aortic complications should undergo routine clinic...