Background:Rheumatoid arthritis-related interstitial lung disease (RA-ILD) is the most common type of lung involvement in rheumatoid arthritis (RA). The existence of RA-ILD is associated with worse survival. There is no mainstay treatment for RA-ILD. However, in recent studies, rituximab (RTX) seems to be effective in RA-ILD.Objectives:To determine the effects of RTX in patients with RA-ILD from a single-centre.Methods:In our biological treatment outpatient clinic, a retrospective study was conducted in patients with RA who were treated with RTX. Among them, patients with RA-ILD were analysed. For lung response to RTX, progression was defined as a decline of 10% ≥ in forced vital capacity (FVC) and/or a decline of 15% ≥ in diffusion capacity of carbon monoxide (DLCO). Computed tomography of the chest (chest-CT) were integrated to lung response so as to constitute missing data of pulmonary function tests (PFTs).Results:A total of 165 patients who are followed-up in our biological treatment outpatient clinic have been using RTX for their RA diagnosis. Among 165 patients, 26 (15.8%) patients with RA-ILD were initiated RTX. Five patients were diagnosed with RA-ILD while using RTX (incidence rate 3%). Patients‘ characteristics were demonstrated in table 1. Of 26 patients, the most commonly used concomitant disease-modifying antirheumatic drugs was leflunomide (46.2%) followed by mycophenolate mofetil (11.5), methotrexate (11.5%) and azathioprine (3.8%). Twenty-four (92.3%) patients used steroids. For the evaluation of lung response, 20 patients who had follow-up PFTs and/or chest-CT were compared. Median DLCO values for pre- and post RTX were 71.0% (60.0-77.0) and 63.0% (47.0-74.0), respectively (p= 0.061). Median pre and post-RTX FVC were 74.0% (61.0-99.0) and 84.0% (63.0-100.0), respectively (p= 0.28). After a median of 2.7 years, 11 (55.0%) patients had stable disease, 2 (10.0%) patients had regression of RA-ILD and 7 (35.0%) patients had progressive disease. Sex, age, seropositivity and radiographic patterns were not associated with progression. In total, 23 adverse events were detected in 11 (42.3%) patients. Five of them were serious infections requiring hospitalization, mostly pneumonia. Other adverse events were simple infections and surgeries such as arthroplasty. RTX was stopped in 4 (15.4%) patients due to infections secondary to hypogammaglobulinemia in 2, malignancy in 1 and allergic reaction in 1.Conclusion:RTX seems to achieve stabilization/improvement of RA-ILD with fewer adverse events. However, given that RA-ILD develops post-RTX, it is not a panacea; therefore, we need more effective treatment modalities in the case of RA-ILD.Table 1.Characteristics of 26 patients with RA-ILD treated with rituximabAge at first RTX infusion, median (IQR), years61.2 (57.1-65.1)RA disease duration at first RTX, median (IQR), years10.1 (4.3-29.7)ILD disease duration at first RTX, median (IQR), years1.9 (0.1-4.5)Age at RA diagnosis, median (IQR), years51.1 (40.0-60.6)Age at ILD diagnosis, median (IQR), years58.3 (53.9-63.3)Male patient, n (%)16 (61.5)RF positivity, n (%)25 (96.2)ACPA positivity, n (%)20 (90.9)CRP levels,mg/L, mean (SD)40.3 (58.9)ESR levels, mean (SD)37.7 (18.2)Smoking status, n (%)16 (64.0)Prior TNF inhibitor treatment, n (%)5 (19.2)DAS28 at first RTX infusion, mean (SD)5.3 (1.5)Radiographic pattern, n (%)UIP7 (26.9)NSIP8 (30.8)OP2 (7.7)Indeterminate8 (30.8)Others1 (3.8)Follow-up duration, median (IQR), years2.5 (1.2-3.7)RA-ILD= Rheumatoid arthritis-related interstitial lung disease, RTX= rituximab, RA= rheumatoid arthritis, RF= rheumatoid factor, ACPA= anti-citrullinated protein antibody, TNF= tumor necrosis factor, CRP= C-reactive protein, ESR= erythrocyte sedimentation rate, UIP= usual interstitial pneumonia, NSIP= non-specific interstitial pneumonia, OP= organizing pneumoniaDisclosure of Interests:None declared
Background:Behçet’s Syndrome (BS) is a vasculitis of unknown origin. Vascular involvement, so-called vascular Behçet’s syndrome (VBS), may involve blood vessels of all sizes belonging to both venous and arterial system, with pulmonary artery involvement (PAI) being the most frequent form of arterial involvement. PAI in BS occurs in the form of aneurysms or thrombosis, and results in significant mortality.Objectives:To report the clinical characteristics of PAI in patients with BS and to define the predictors of relapses.Methods:We performed a retrospective analysis of BS patients with PAI who fulfilled international study group criteria. Among 460 patients with VBS, 66 were diagnosed with PAI. For final analyses, 61 patients with PAI, who had at least 2 follow-up visits (72.1% male, mean age at BS diagnosis 29.34 (SD 10.1) years), were included. The data of the patients were recorded. Relapse was defined as the reoccurrence of vascular event at any vascular structure. Factors associated with relapse were assessed by logistic regression analysis.Results:There were not any differences considering demographic and clinical features of the patients with and without PAI in VBS group except for that intracardiac thrombosis was more common in the patients with PAI than the patients without (19.7% vs 0.3%, respectively) (Figure 1). Among 61 patients with PAI, 50 (82.0%) had isolated pulmonary artery thrombosis (PAT), whereas 11 (18.0%) had pulmonary artery aneurysm (PAA) with or without PAT. The characteristics of 61 patients with PAI were shown in Table 1. A total of 37 relapses occurred in 24 (39.3%) patients during follow-up a median of 65.9 (IQR 20.1-109.0) months. To define the factors associated with relapses, the patients with isolated PAT were analysed. In multivariable logistic regression analysis, older age at BS diagnosis and anticoagulation usage seemed to be protective, even though they could not reach statistical significance (OR: 0.92 95% CI 0.86-1.02, OR: 0.34 95% CI 0.09-1.33, respectively).Table 1.Characteristics of the patients with pulmonary artery involvementAlln= 61Isolated PATn= 50PAAn= 11Sex (male), n (%)44 (72.1)35 (70.0)9 (81.8)Age at diagnosis of BS, years (SD)29.3 (10.1)28.8 (10.4)31.9 (8.0)Age at diagnosis of PAI, years (SD)36.3 (12.83)36.7 (13.8)34.7 (6.7)Symptoms, n (%)Dyspnea28 (45.9)23 (46.0)5 (45.5)Hemoptysis26 (42.6)19 (38.0)7 (63.6)Vascular event before PAI, n (%)21 (34.4)20 (40.0)1 (9.1)Other vascular events at PAI, n (%)33 (54.1)28 (56.0)5 (45.5)Thrombophlebitis, n (%)7 (21.2)6 (21.4)1 (20.0)Upper and/or lower extremity DVT, n (%)20 (60.6)16 (57.1)4 (80.0)SVCS, n (%)1 (3.0)1 (3.6)0 (0)IVCS, n (%)3 (9.1)1 (3.6)2 (40.0)Intracranial thrombosis, n (%)4 (12.1)4 (14.3)0 (0)Intracardiac thrombosis, n (%)8 (24.2)7 (25.0)1 (20.0)Arterial event other than PAI, n (%)4 (12.1)3 (10.7)1 (20.0)Relapse, n (%)24 (39.3)20 (40.0)4 (36.4)Time to the first relapse, median (IQR), month49.8 (13.2-102.6)54.1 (24.4-117.9)3.9 (1.7-67.9)Death, n (%)2 (3.3)0 (0)2 (18.2)BS= Behçet Syndrome, PAI= pulmonary artery involvement, PAT= pulmonary artery thrombosis, PAA= pulmonary artery aneurysm, DVT= deep vein thrombosis, SVCS= superior vena cava syndrome, IVCS= inferior vena cava syndromeFigure 1.A) Axial CT image shows thrombus formation in the left upper lobe pulmonary artery branch. B) Giant thrombus is seen in the right ventricle (black arrow). Note the thrombus formation in the left lower lobe pulmonary artery branches (white arrows).Conclusion:Our results indicate that there is a higher frequency of intracardiac thrombosis in BS patients with PAI and an increasing current trend of PAT in patients with PAI. In addition, there might be a possible efficacy of anticoagulation usage in preventing relapses, which needs confirmation with further studies.Disclosure of Interests:None declared
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