IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.

BackgroundIgG4-related disease (IgG4-RD) is a relapsing-remitting fibro-inflammatory disorder that dramatically responds to corticosteroids but oftentimes relapses while tapering these medications. Corticosteroid refractoriness appears to be more common in patients with extrapancreatic disease. Experience with disease-modifying antirheumatic drugs as steroid-sparing agents is lacking.ObjectivesWe aimed to assess the efficacy of methotrexate (MTX) in maintaining steroid-induced remission of IgG4-RD with systemic involvement.MethodsThe present study included 10 subjects with biopsy proven IgG4-RD followed between January 2008 and May 2014. At IgG4-RD relapse MTX (up to 20 mg/week) was added to conventional steroid therapy. Efficacy of MTX in maintaining steroid induced remission was assessed at 6 and 12 months by (i) IgG4-RD Responder Index (IgG4-RD RI), (ii) patient's ability to taper or stop prednisone, (iii) total IgG and IgG subclasses, and (iv) radiological imaging. An improvement of the IgG4-RD RI of more than 2 points and an overall IgG4-RD RI <3 after therapy defined partial (PR) and complete (CR) remission, respectively. Paired t-test was used to compare variables at disease relapse, 6 and 12 months after MTX introduction. A p value <0.05 was considered statistically significant.ResultsPatients' cohort included 5 males and 5 females, with a mean age of 65 years at the time of diagnosis (range 51 – 75 years). Organ involvement included pancreas, biliary tree, aorta, salivary glands (submandibular and parotid), lacrimal glands, lymph nodes, pachymeninges and retroperitoneum. IgG4-RD relapse occurred on average 7 months (range 2-10 months) after the diagnosis, at a mean steroid daily dosage of 9.6 mg (range 0-20 mg). The mean IgG4-RD RI and serum IgG4 concentration at disease relapse were 8.5 (range 3-15) and 666 mg/dL (range 46-2500 mg/dL), respectively. Oral prednisone at a starting dose of 1mg/kg for 3 weeks induced CR in 4 patients and PR in 6. After 3 weeks of prednisone, the mean IgG4-RD RI, serum IgG4 level, and daily dosage of prednisone were 2.75 (range 1-6), 513 mg/dL (19-1500 mg/dL) and 5 mg (range 0-10 mg), respectively (p value <0.05 for all variables when compared to their values at disease relapse). MTX was introduced on average 5 weeks (range 1-16 weeks) after initiation of corticosteroid therapy and escalated to a dosage of 20 mg/week. The mean daily dosage of prednisone at the time of MTX introduction was 20.75 mg (range 10-50 mg). Six months after MTX introduction 3 patients in CR were able to stop glucocorticoid treatment. Twelve months after MTX initiation, 5 patients were in CR without prednisone; 5 were in PR on prednisone 5 mg/day. No relapses occurred under MTX.ConclusionsTreatment with methotrexate represents a promising strategy for maintaining steroid-induced remission in patients with IgG4-RD.ReferencesKhosroshahi A, Stone JH. Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol 2011;23(1):67-71.AcknowledgementsThe authors thanks Prof John H Stone from the Mas...

Background:Exposure to severe or chronic life stressors may alter immune function and high levels of subsequent distress have been implicated in autoimmune disease pathogenesis. Post-traumatic stress disorder (PTSD) is a debilitating psychiatric condition affecting 1-12% of the general population1, occurring in response to traumatic events. Growing evidence supports an association between trauma exposure and PTSD with systemic lupus erythematosus (SLE) onset2.Objectives:To cross-sectionally assess PTSD prevalence in a cohort of patients with SLE and to examine its correlation with quality of life.Methods:A 189-item anonymous questionnaire including demographics, disease features, the 9-domain Trauma and Loss Spectrum – Self Report (TALS-SR) and the 8-domain Lupus Quality of Life (Lupus QoL) was administered via web to a cohort of patients with SLE. Patients were classified as PTSD cases based on TALS-SR items corresponding to the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) criteria for PTSD.Results:Ninety-nine (95% female and 5% male) patients with a median follow-up of 16.5 years completed the questionnaire. Self-reported fatigue prevalence was 75%. Fifteen patients (15%) reportedly were on psychological and/or psychiatric support. Thirty-one patients (31%) met the DSM-5 criteria for PTSD. The average LupusQoL interdomain score was 80/100. PTSD cases reported significantly lower scores compared to non-cases in three LupusQoL domains: planning (83 vs. 100, p=0.035), body image (85 vs. 95, p=0.031), and fatigue (67 vs. 92, p=0.001). An inverse correlation between TALS-SR scores and Lupus QoL subscales was found (Table1). In particular, the degree of stress secondary to losses or upsetting events was strongly correlated to fatigue intensity (rho= -0.458, p<0.001).Conclusion:PTSD prevalence might be higher in SLE than in the general population and have a detrimental influence on quality of life. Fatigue perception might be more significantly affected by PTSD. Intervention studies are needed to assess the therapeutical effects of psychological support in patients with SLE.References:[1]Shalev A et al. Post-Traumatic Stress Disorder. New England Journal of Medicine, June 2017.[2]Roberts AL et al. Association of Trauma and Posttraumatic Stress Disorder With Incident Systemic Lupus Erythematosus in a Longitudinal Cohort of Women. Arthritis & Rheumatology, November 2017.Table 1.Spearman rho coefficients outlining correlation across Lupus QoL and TALS-SR domains. The highest negative correlation has been found between fatigue and reaction to traumatic events. Significant correlations boxes are coloured in yellow (weak, rho 0.20-0.39) and red (moderate, rho 0.40-0.59). * p<0.05, ** p<0.01.LupusQoL DomainsLupus QoL Total ScorePhysical healthPainPlanningIntimate relationshipsBurden to othersEmotional healthBody imageFatigueTALS-SR DomainsLoss events-.217-.217-.096-.031.047-.022.076-.009-.061Grief reactions-.145-.104-.041-.018-.124-.172-.192-.149-.107Potentially traumatic events.039-.082-.169-.167-.207-.185-.046-.229-.096Reaction to losses or upsetting events-.221-.256*-.289*-.218-.290*-.369**-.371**-.458**-.341**Re-experiencing-.139-.215-.245*-.228-.320**-.275*-.287*-.342**-.274*Avoidance and numbing-.176-.246*-.279*-.257*-.337**-.405**-.413**-.406**-.338**Maladaptive coping-.190-.238-.294*-.282*-.324**-.340**-.358**-.405**-.327**Arousal-.134-.177-.263*-.320**-.283*-.279*-.321**-.397**-.282*Personal characteristics / risk factors-.044-.115-.266*-.189-.409**-.231-.197-.253*-.199Disclosure of Interests:Luca Moroni: None declared, Martina Mazzetti: None declared, Giuseppe Alvise Ramirez: None declared, Simone Zuffada: None declared, Nicola Farina: None declared, Enrica Bozzolo: None declared, Valentina Di Mattei: None declared, Lorenzo Dagna Consultant of: Abbvie, Amgen, Biogen, BristolMyers Squibb, Celltrion, Galapagos, GlaxoSmithKline, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI, Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, GlaxoSmithKline,Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi Genzyme, and SOBI

Background:LN is still a severe manifestation of Systemic lupus erythematosus (SLE) and multitarget therapy is needed to control the disease especially in refractory cases.Objectives:To evaluate renal response in SLE patients with glomerulonephritis (GN) treated with Belimumab in real-life setting.Methods:Patients with proteinuria >0.5 g/24 h and/or active sediment at baseline enrolled in a multicentre Italian cohort of SLE patients (BeRLiSS study), treated with monthly iv Belimumab 10 mg/kg plus standard of care were considered in this study. Complete renal response (CRR) was defined as proteinuria <0.5 g/24 h, estimated glomerular filtration rate (eGFR)≥90ml/min/1.73m2 and no rescue therapy. Primary efficacy renal response (PERR) was defined as proteinuria ≤0.7 g/24 h, eGFR ≥60ml/min/1.73m2 and no rescue therapy. Prevalence and predictive factors of CRR and PERR at 12 and 24 months after Belimumab initiation were analyzed by multivariate logistic regression analysis.Results:A total of 91 patients were considered in this study, 79 female, mean age 40.51±9.03 years, mean disease duration 12.18±8.15 years, median follow-up time after Belimumab initiation 22 months. Twenty patients had baseline proteinuria ≥0.5 <1 g/day, 17 ≥1 <2 g/day, 13 ≥2 g/day. Belimumab was started at GN onset in 20 (22%) patients and at the time of a renal flare in all other cases. Seventy-five patients underwent a renal biopsy: 1 class I, 4 class II, 14 class III, 47 class IV and 9 class V. Baseline serum creatinine was 82.44±29.26 umol/L; 15 patients showed eGFR<60ml/min/1.73m2 at baseline. Immunosuppresants were taken by 70 (76.9%) patients: 47 micofenolate, 15 azathioprine and 5 ciclosporine. Sixty patients (65.9%) were on antimalarials. During follow-up 34 (37.4%) patients achieved CRR. Among them 5 (14.7%) patients relapsed and 29 (85.3%) patients maintained remission. Mean time to achieved CRR was 9.71±5.91 months.High levels of baseline proteinuria were a negative independent predictor of CRR and PERR at 6 months (OR 0.044 CI95% 0.006-0.320 p=0.002 and OR 0.232 CI95% 0.091-0.596 p=0.002) and 12 months (OR 0.029 CI95% 0.002-0.556 p=0.019 and OR 0.056 CI95% 0.009-0.327 p=0.001). High levels of baseline creatinine were a negative independent predictor of renal response. Renal response at 6 months was a strong predictive factor of renal response at 12 and 24 months.Conclusion:Belimumab is an effective add-on therapy in the treatment of GN in real-life practice setting.Disclosure of Interests:None declared